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1. Erlic Z, Neumann HP: Familial pheochromocytoma. Hormones (Athens); 2009 Jan-Mar;8(1):29-38
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  • [Title] Familial pheochromocytoma.
  • While the World Health Organization (WHO) applies pheochromocytoma exclusively to adrenal tumours, many clinicians use the term pheochromocytoma also for extra-adrenal abdominal and thoracic tumours, since by tradition pheochromocytoma is a vasoactive tumour.
  • In contrast, head and neck paraganglioma is mostly only a space-occupying mass.
  • The diagnosis is confirmed by both biochemical testing and radiological imaging.
  • All pheochromocytoma patients with NF 1 also show cutaneous lesions.
  • About 50% of MEN2 patients harbour pheochromocytoma.
  • The dominant lesion in this entity is Medullary Thyroid Carcinoma (MTC) occurring in up to 100% of patients.
  • Von Hippel-Lindau disease (VHL)is found in about 20% of patients in association with pheochromocytoma.
  • VHL is classified as type 1 predominantly without and type 2 predominantly with pheochromocytoma.
  • Paraganglioma syndromes include predisposition to paraganglial tumours in any location, whereas PGL 3 patients mostly show only head and neck paragangliomas.
  • Familial paraganglial tumours are characterized by younger age at diagnosis and more frequently multifocal and extra-adrenal abdominal pheochromocytomas.
  • Patients with PGL 4 and less frequently VHL, are particularly predisposed to malignant pheochromocytoma.
  • Endoscopic surgery is the primary treatment for pheochromocytoma.
  • For malignant cases, chemotherapeutic as well as radionuclear approaches are available.
  • Thus, early diagnosis and regular follow-up are the only means for a better outcome.
  • [MeSH-major] Paraganglioma / genetics. Pheochromocytoma / genetics
  • [MeSH-minor] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / genetics. Carcinoma, Medullary / genetics. Humans. Multiple Endocrine Neoplasia Type 2a / genetics. Neurofibromatosis 1 / genetics. Succinate Dehydrogenase / genetics. Thyroid Neoplasms / diagnosis. Thyroid Neoplasms / genetics. von Hippel-Lindau Disease / genetics

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  • (PMID = 19269919.001).
  • [ISSN] 1109-3099
  • [Journal-full-title] Hormones (Athens, Greece)
  • [ISO-abbreviation] Hormones (Athens)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] EC 1.3.99.1 / Succinate Dehydrogenase
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2. Sandgren J, Andersson R, Rada-Iglesias A, Enroth S, Akerstrom G, Dumanski JP, Komorowski J, Westin G, Wadelius C: Integrative epigenomic and genomic analysis of malignant pheochromocytoma. Exp Mol Med; 2010 Jul 31;42(7):484-502
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  • [Title] Integrative epigenomic and genomic analysis of malignant pheochromocytoma.
  • Epigenomic and genomic changes affect gene expression and contribute to tumor development.
  • Pheochromocytoma is a rare endocrine tumor of the adrenal gland that mostly occurs sporadic with unknown epigenetic/genetic cause.
  • Here we aimed to combine the genome-wide profiling of H3K4me3 and H3K27me3, obtained by the ChIP-chip methodology, and DNA copy number data with global gene expression examination in a malignant pheochromocytoma sample.
  • The integrated analysis of the tumor expression levels, in relation to normal adrenal medulla, indicated that either histone modifications or chromosomal alterations, or both, have great impact on the expression of a substantial fraction of the genes in the investigated sample.
  • Candidate tumor suppressor genes identified with decreased expression, a H3K27me3 mark and/or in regions of deletion were for instance TGIF1, DSC3, TNFRSF10B, RASSF2, HOXA9, PTPRE and CDH11.
  • Our approach to associate histone methylations and DNA copy number changes to gene expression revealed apparent impact on global gene transcription, and enabled the identification of candidate tumor genes for further exploration.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / pathology. Epigenesis, Genetic. Genome, Human / genetics. Genomics. Pheochromocytoma / genetics. Pheochromocytoma / pathology
  • [MeSH-minor] Female. Gene Dosage / genetics. Gene Expression Regulation, Neoplastic. Gene Regulatory Networks / genetics. Histones / metabolism. Humans. Lysine / metabolism. Methylation. Protein Processing, Post-Translational. Tumor Suppressor Proteins / genetics. Tumor Suppressor Proteins / metabolism

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  • (PMID = 20534969.001).
  • [ISSN] 2092-6413
  • [Journal-full-title] Experimental & molecular medicine
  • [ISO-abbreviation] Exp. Mol. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Histones; 0 / Tumor Suppressor Proteins; K3Z4F929H6 / Lysine
  • [Other-IDs] NLM/ PMC2912476
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3. Segawa N, Osafune T: Malignant pheochromocytoma of the urinary bladder. Hinyokika Kiyo; 2005 Apr;51(4):291-6
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  • [Title] Malignant pheochromocytoma of the urinary bladder.
  • A case of malignant pheochromocytoma of the urinary bladder is presented.
  • Cystoscopy revealed a broad-based, small fingertip-sized, nonpapillary tumor on the posterior wall of the urinary bladder.
  • Transurethral resection (TUR) was performed without suspicion of pheochromocytoma because of her well-controlled blood pressure and lack of characteristic symptoms.
  • Transient elevation of systolic blood pressure to 240 mmHg occurred during resection of the tumor.
  • Pathologic examination revealed a malignant pheochromocytoma.
  • On January 30, 2003, partial cystectomy of the residual tumor and pelvic lymphadenectomy were performed.
  • [MeSH-major] Pheochromocytoma / surgery. Urinary Bladder Neoplasms / surgery

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  • (PMID = 15912793.001).
  • [ISSN] 0018-1994
  • [Journal-full-title] Hinyokika kiyo. Acta urologica Japonica
  • [ISO-abbreviation] Hinyokika Kiyo
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 11
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4. Waldmann J, Fendrich V, Holler J, Buchholz M, Heinmöller E, Langer P, Ramaswamy A, Samans B, Walz MK, Rothmund M, Bartsch DK, Slater EP: Microarray analysis reveals differential expression of benign and malignant pheochromocytoma. Endocr Relat Cancer; 2010 Sep;17(3):743-56
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Microarray analysis reveals differential expression of benign and malignant pheochromocytoma.
  • The diagnosis of a malignant pheochromocytoma (PC) can only be established by the presence of distant metastases, but a subset of apparently benign PCs develop metastases.
  • We have employed a microarray analysis to identify a typical gene expression profile which distinguishes malignant from benign PC.
  • Total RNA was isolated from fresh-frozen tissue of five benign and five malignant PCs.
  • The reference consisted of laser microdissected tissue from normal adrenal medulla.
  • The analysis revealed a more than twofold difference in expression between benign and malignant PCs in 132 genes: 19 were up-regulated and 113 were down-regulated.
  • IHC for calsequestrin revealed an overexpression in malignant PCs (7/10 vs 1/10, P=0.03).
  • Comparative analysis by microarray of all ten PCs (benign/malignant) versus normal adrenal medulla revealed a more than twofold expression difference in 455/539 and 491/671 genes respectively.
  • Several of these genes are known to participate on adrenal tumorigenesis, potential tumor suppressor genes, and oncogenes.
  • Comprehensive gene expression analysis of malignant and benign PCs revealed different gene profiles, which could be used to discriminate between malignant and benign PCs.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Adrenal Medulla / metabolism. Biomarkers, Tumor / metabolism. Gene Expression Profiling. Pheochromocytoma / metabolism
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Case-Control Studies. Humans. Immunoenzyme Techniques. Middle Aged. Neoplasm Metastasis. Oligonucleotide Array Sequence Analysis. Prognosis. RNA, Messenger / genetics. Reverse Transcriptase Polymerase Chain Reaction. Young Adult


5. Niaz WA, Alvi S: Metastatic malignant pheochromocytoma of adrenal gland. J Coll Physicians Surg Pak; 2008 May;18(5):305-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metastatic malignant pheochromocytoma of adrenal gland.
  • Malignant pheochromocytoma is a rare disease with a high mortality.
  • A case of metastatic malignant pheochromocytoma of the right adrenal gland is presented who had fluctuating blood pressure with episodic headache and raised urinary VMA levels.
  • [MeSH-major] Adrenal Gland Neoplasms / secondary. Pheochromocytoma / secondary
  • [MeSH-minor] Adrenalectomy / methods. Adult. Follow-Up Studies. Humans. Male. Neoplasm Metastasis. Severity of Illness Index. Tomography, X-Ray Computed. Ultrasonography, Doppler, Color

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  • (PMID = 18541088.001).
  • [ISSN] 1022-386X
  • [Journal-full-title] Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
  • [ISO-abbreviation] J Coll Physicians Surg Pak
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Pakistan
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6. Havekes B, Lai EW, Corssmit EP, Romijn JA, Timmers HJ, Pacak K: Detection and treatment of pheochromocytomas and paragangliomas: current standing of MIBG scintigraphy and future role of PET imaging. Q J Nucl Med Mol Imaging; 2008 Dec;52(4):419-29
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Pheochromocytomas are rare tumors arising from chromaffin cells of adrenal medullary or extra-adrenal paraganglionic tissue.
  • Similar to the sympathetic nervous system, pheochromocytomas express cellular norepinephrine transporters (NET) through which catecholamines can enter pheochromocytoma cells to be stored in vesicles.
  • Metaiodobenzylguanidine (MIBG) resemblance to norepinephrine and its good affinity and uptake by NET resulted in its use in pheochromocytoma diagnosis from 1981.
  • Importantly, with this distinction of clinical phenotypes, MIBG was found to have a different performance in subsets of pheochromocytoma patients.
  • Reduced sensitivity of MIBG scintigraphy in some familial paraganglioma syndromes, malignant disease and extra-adrenal paragangliomas has been found.
  • Therefore, newer compounds, especially for positron emission tomography (PET), such as [(11)C]hydroxyephedrine ([(11)C]HED), [(18)F]fluoro-2-deoxy-D-glucose ([(18)F]FDG), [(18)F]fluoro-dihydroxyphenylalanine ([(18)F] FDOPA) and [(18)F]fluorodopamine ([(18)F]FDA) have emerged and were found to be superior to MIBG in the localization of certain types of pheochromocytoma and paragangliomas.
  • Finally, using [(131)I]MIBG represents an important treatment option in patients with malignant pheochromocytoma, but the development of newer treatment modalities is expected.
  • In this review, we provide the reader with an overview of the current standing of [(123)I]- and [(131)I]MIBG in diagnosis and treatment of pheochromocytoma amongst the newer PET imaging agents.
  • [MeSH-major] 3-Iodobenzylguanidine / therapeutic use. Paraganglioma / radionuclide imaging. Paraganglioma / radiotherapy. Pheochromocytoma / radionuclide imaging. Pheochromocytoma / radiotherapy

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  • (PMID = 19088695.001).
  • [ISSN] 1824-4785
  • [Journal-full-title] The quarterly journal of nuclear medicine and molecular imaging : official publication of the Italian Association of Nuclear Medicine (AIMN) [and] the International Association of Radiopharmacology (IAR), [and] Section of the Society of Radiopharmaceutical Chemistry and Biology
  • [ISO-abbreviation] Q J Nucl Med Mol Imaging
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Iodine Radioisotopes; 35MRW7B4AD / 3-Iodobenzylguanidine
  • [Number-of-references] 98
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7. Sisson JC, Shulkin BL, Esfandiari NH: Courses of malignant pheochromocytoma: implications for therapy. Ann N Y Acad Sci; 2006 Aug;1073:505-11
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Courses of malignant pheochromocytoma: implications for therapy.
  • Survival of patients with metastatic pheochromocytoma that have exceeded 30 years without therapy to reduce tumors have been reported.
  • We reviewed the records of 38 patients with malignant pheochromocytoma who had received 131I-metaiodiobenzylguanidine (131I-MIBG) treatments between 1981 and 1996 to evaluate longevity.
  • Survival from diagnosis to last follow-up exceeded 5 years in 21 of 38 (55%) and >or=10 years in 50%.
  • In 17 of 21, the interval from diagnosis to 131I-MIBG therapy was greater than 5 years.
  • Objective responses to 131I-MIBG therapy were seen in about 30% and were usually of a few years, duration, but one individual exhibited marked reductions in volume and function of tumors that have persisted for 21 years.
  • In summary, many patients with malignant pheochromocytoma will follow a course extending over many years.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology

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  • (PMID = 17102118.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Grant] United States / NCRR NIH HHS / RR / M01-RR00042
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
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8. Ohta S, Lai EW, Pang AL, Brouwers FM, Chan WY, Eisenhofer G, de Krijger R, Ksinantova L, Breza J, Blazicek P, Kvetnansky R, Wesley RA, Pacak K: Downregulation of metastasis suppressor genes in malignant pheochromocytoma. Int J Cancer; 2005 Mar 10;114(1):139-43
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Downregulation of metastasis suppressor genes in malignant pheochromocytoma.
  • There is no reliable method currently available to predict malignant potential of pheochromocytoma based on conventional histology or genetic, molecular or immunohistochemical markers.
  • Metastasis suppressor genes affect the spread of several cancers and, therefore, may provide promise as prognostic markers or therapeutic targets for malignant pheochromocytoma.
  • We hypothesized that the downregulation of metastasis suppressor genes in malignant pheochromocytoma may play a role in malignant behavior.
  • The study included 15 benign and 10 malignant pheochromocytomas.
  • Six metastasis suppressor genes (nm23-H1, TIMP-4, BRMS-1, TXNIP, CRSP-3 and E-Cad) were downregulated significantly in malignant compared to benign pheochromocytoma (p < 0.05, Mann-Whitney U-test).
  • We applied a non-linear rule using median malignant value (MMV) as a threshold to use metastasis suppressor genes to distinguish malignant from benign samples.
  • After cross-validation, the non-linear rule produced no errors in 10 malignant samples and 3 errors in the 15 benign samples, with an overall error rate of 12%.
  • These results suggest that downregulation of metastasis suppressor genes reflect malignant pheochromocytoma with a high degree of sensitivity.
  • Thus, we conclude that altered function of these metastasis suppressor gene pathways may play an important role in the malignant behavior of pheochromocytoma.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Genes, Tumor Suppressor. Pheochromocytoma / metabolism
  • [MeSH-minor] Adrenal Gland Neoplasms / metabolism. Adult. Aged. Cadherins / metabolism. Down-Regulation. Female. Humans. Male. Mediator Complex. Middle Aged. NM23 Nucleoside Diphosphate Kinases. Nucleoside-Diphosphate Kinase / metabolism. Polymerase Chain Reaction. Prognosis. Tissue Inhibitor of Metalloproteinases / metabolism. Trans-Activators / metabolism

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  • (PMID = 15523699.001).
  • [ISSN] 0020-7136
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Cadherins; 0 / MED23 protein, human; 0 / Mediator Complex; 0 / NM23 Nucleoside Diphosphate Kinases; 0 / Tissue Inhibitor of Metalloproteinases; 0 / Trans-Activators; 0 / tissue inhibitor of metalloproteinase-4; EC 2.7.4.6 / NME1 protein, human; EC 2.7.4.6 / Nucleoside-Diphosphate Kinase
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9. Gimm O, Krause U, Brauckhoff M, Hoang-Vu C, Dralle H: Distinct expression of galectin-3 in pheochromocytomas. Ann N Y Acad Sci; 2006 Aug;1073:571-7
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  • Unless distant metastases or local invasion are present, the diagnosis of malignant pheochromocytoma is challenging.
  • Four malignant and 24 benign (10 sporadic, 14 hereditary) pheochromocytomas were analyzed for the expression of galectin-3.
  • One malignant pheochromocytoma with distant metastases showed strong and one malignant undifferentiated pheochromocytoma with local invasion showed partly strong cytoplasmic staining.
  • One benign sporadic pheochromocytoma had moderate cytoplasmic staining.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Galectin 3 / genetics. Pheochromocytoma / genetics

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  • (PMID = 17102125.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Galectin 3
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10. Mori H, Nagata M, Nishijima N, Nagura K, Igarashi H, Hamazaki M, Ozono S, Sugimura H: Malignant pheochromocytoma in a young adult forming the structure simulating Homer Wright rosette: differentiation from neuroblastoma on repeating fluorescence in situ hybridization. Pathol Int; 2008 Aug;58(8):518-23
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  • [Title] Malignant pheochromocytoma in a young adult forming the structure simulating Homer Wright rosette: differentiation from neuroblastoma on repeating fluorescence in situ hybridization.
  • A peculiar adrenal tumor was analyzed using immunohistochemistry, electron microscopy, and fluorescence in situ hybridization (FISH) with multiple bacterial artificial chromosome (BAC) probes.
  • Her serum and urine dopamine level were elevated, and a diagnosis of malignant pheochromocytoma was made.
  • On post-mortem an adrenal tumor composed of small round cells forming Homer Wright rosette-like structures, a feature rarely observed in pheochromocytoma, was found.
  • Because these results did not rule out a diagnosis of neuroblastoma, the tumor was further characterized on FISH with multiple BAC probes for loci known to be altered in neuroblastoma or pheochromocytoma, according to information in the literature that was for the most part obtained using comparative genomic hybridization.
  • FISH demonstrated loss of heterozygosity at 11p, and gains at 16p, 19p, and 19q, a profile that favored a diagnosis of malignant pheochromocytoma over neuroblastoma.
  • This case demonstrates that repeating FISH is useful for differential diagnosis.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. In Situ Hybridization, Fluorescence / methods. Neuroblastoma / diagnosis. Pheochromocytoma / pathology
  • [MeSH-minor] Adult. Chromosomes, Artificial, Bacterial / chemistry. Chromosomes, Artificial, Bacterial / genetics. DNA Probes / chemistry. DNA, Neoplasm / analysis. Diagnosis, Differential. Dopamine / blood. Fatal Outcome. Female. Humans


11. Bahrami A, Truong LD, Shen SS, Krishnan B: Synchronous renal and adrenal masses: an analysis of 80 cases. Ann Diagn Pathol; 2009 Feb;13(1):9-15
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Synchronous renal and adrenal masses: an analysis of 80 cases.
  • Synchronous renal and adrenal masses are uncommon.
  • Although adrenal masses in the context of renal cell carcinoma (RCC) are often suspected as metastasis, other adrenal lesions with different therapeutic and prognostic implications may coexist with RCC.
  • In a retrospective review of 550 radical nephrectomies with ipsilateral adrenalectomy, 80 cases of coexisting renal and adrenal masses were identified.
  • The renal masses included 76 RCCs, 3 oncocytomas, and 1 malignant pheochromocytoma of adrenal gland involving the kidney.
  • Although the gross pathologic impression of adrenal masses in the presence of RCC was metastasis, on histologic examination, 56% of them were found to be benign lesions (mostly adrenal adenoma/hyperplasia), whereas malignant involvement from RCC was seen in 43%.
  • The benign and malignant nature of the adrenal lesions in the context of RCC could not be discriminated based on the size of adrenal mass.
  • Because of the prognostic implication of direct or metastatic involvement of adrenal gland in the setting of RCC and the possibility of finding small metastatic foci, a meticulous gross and microscopic examination of adrenal glands is emphasized.
  • Rare unusual combinations of renal and adrenal lesions such as RCC and adrenal histoplasmosis, RCC and adrenal myelolipoma, renal oncocytoma, and adrenal pheochromocytoma are also described.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Glands / pathology. Carcinoma, Renal Cell / pathology. Kidney / pathology. Kidney Neoplasms / pathology
  • [MeSH-minor] Adenoma, Oxyphilic / pathology. Adenoma, Oxyphilic / surgery. Adrenalectomy. Adult. Aged. Diagnosis, Differential. Female. Humans. Male. Middle Aged. Neoplasm Staging. Nephrectomy. Pheochromocytoma / pathology. Pheochromocytoma / surgery. Retrospective Studies

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  • (PMID = 19118776.001).
  • [ISSN] 1532-8198
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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12. Kasliwal MK, Sharma MS, Vaishya S, Sharma BS: Metachronous pheochromocytoma metastasis to the upper dorsal spine-6-year survival. Spine J; 2008 Sep-Oct;8(5):845-8
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  • [Title] Metachronous pheochromocytoma metastasis to the upper dorsal spine-6-year survival.
  • BACKGROUND CONTEXT: Malignant pheochromocytoma is a rare neoplasm of chromaffin tissue.
  • Very few cases of malignant adrenal pheochromocytoma metastatic to vertebrae exist.
  • PURPOSE: To determine the prognosis of a patient with an excised adrenal pheochromocytoma and a single metachronous metastasis to the upper dorsal spine.
  • STUDY DESIGN: Case report METHODS: The authors report a patient who underwent total excision of an adrenal pheochromocytoma of the left adrenal gland in 2000 who developed a single metastasis to the second dorsal vertebra in 2002 with no evidence of abdominal recurrence.
  • CONCLUSIONS: Patients with adrenal pheochromocytomas must be screened periodically with whole body imaging despite normal abdominal imaging as there is a definite risk of metachronous metastasis.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / secondary. Spinal Neoplasms / secondary

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  • (PMID = 18024223.001).
  • [ISSN] 1529-9430
  • [Journal-full-title] The spine journal : official journal of the North American Spine Society
  • [ISO-abbreviation] Spine J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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13. McNicol AM: Histopathology and immunohistochemistry of adrenal medullary tumors and paragangliomas. Endocr Pathol; 2006;17(4):329-36
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  • [Title] Histopathology and immunohistochemistry of adrenal medullary tumors and paragangliomas.
  • Paragangliomas arise from sympathetic or parasympathetic paraganglia and should now be defined by their site and type.
  • The term pheochromocytoma is reserved for intra-adrenal tumors.
  • This short review discusses the gross and microscopic features, the immunohistochemical profile, the problem of recognizing malignant potential, and the rare instances where a differential diagnosis has to be considered.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Medulla / pathology. Paraganglioma, Extra-Adrenal / pathology. Pheochromocytoma / pathology
  • [MeSH-minor] Biomarkers, Tumor / analysis. Chromaffin Cells / chemistry. Chromaffin Cells / pathology. Humans. Hyperplasia. Immunohistochemistry / methods

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  • (PMID = 17525481.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Number-of-references] 46
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14. Gupta R, Sharma A, Arora R, Vijayaraghavan M: Composite phaeochromocytoma with malignant peripheral nerve sheath tumour and rhabdomyosarcomatous differentiation in a patient without von Recklinghausen disease. J Clin Pathol; 2009 Jul;62(7):659-61
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  • [Title] Composite phaeochromocytoma with malignant peripheral nerve sheath tumour and rhabdomyosarcomatous differentiation in a patient without von Recklinghausen disease.
  • The coexistence of adrenal phaeochromocytoma with non-chromaffin tumours is a rare fascinating occurrence.
  • The coexistence of adrenal phaeochromocytoma with a malignant Triton tumour does not appear to have been described in the available literature so far.
  • A unique case of composite phaeochromocytoma in a 26-year-old male patient, where the non-chromaffin component was a malignant Triton tumour composed of peripheral nerve sheath tumour and skeletal muscle differentiation, is reported.
  • This is the first case of such a phenomenon in a composite phaeochromocytoma.
  • The present case further widens the histomorphological range of composite phaeochromocytoma of the adrenal gland, which the histopathologist should be aware of.
  • Since the prognosis of composite phaeochromocytoma with malignant nerve sheath tumour would be determined by the nerve sheath component, recognition of this tumour is imperative.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Neoplasms, Multiple Primary / pathology. Nerve Sheath Neoplasms / pathology. Pheochromocytoma / pathology. Rhabdomyosarcoma / pathology

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  • (PMID = 19561238.001).
  • [ISSN] 1472-4146
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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15. Marzano LA, Tauchmanova L, Marzano E, Arienzo R, Guarino R, Ciancia G, Porcelli A, Lombardi G, Rossi R: Large idiopathic unilateral adrenal hematoma in a young woman. J Endocrinol Invest; 2007 Jan;30(1):52-8
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  • [Title] Large idiopathic unilateral adrenal hematoma in a young woman.
  • This is a case report on a young woman with a large idiopathic unilateral adrenal hematoma (AH).
  • Magnetic resonance imaging (MRI) confirmed the presence of a 13-cm sized lesion in the right hemi-abdomen; T1 and T2 weighed imaging was compatible with subacute-to-chronic adrenal hematoma.
  • In conclusion, in the absence of known risk factors, differential diagnosis of a large AH may not be easy.
  • The possibility of an underlying pheochromocytoma, malignant adrenal or metastatic tumor must always be considered.
  • In our patient, computed tomography (CT) scan and MRI suggested the presence of a large subacute-to-chronic AH, and PET excluded metabolic activity of the mass.
  • The correct diagnosis, early recognition and treatment of complications including adrenal insufficiency may decrease patient morbidity and mortality.
  • [MeSH-major] Adrenal Gland Diseases / diagnosis. Hematoma / diagnosis

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  • (PMID = 17318023.001).
  • [ISSN] 1720-8386
  • [Journal-full-title] Journal of endocrinological investigation
  • [ISO-abbreviation] J. Endocrinol. Invest.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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16. Whitman HH 3rd, Fishman EK, Oberg K, Wildman JM, Long AL: Catecholamine-secreting metastatic carcinoid as differential diagnosis in pheochromocytoma: clinical, laboratory, and imaging clues in the search for the lurking neuroendocrine tumor (NET). Ann N Y Acad Sci; 2006 Aug;1073:59-78
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  • [Title] Catecholamine-secreting metastatic carcinoid as differential diagnosis in pheochromocytoma: clinical, laboratory, and imaging clues in the search for the lurking neuroendocrine tumor (NET).
  • Catecholamine-secreting metastatic carcinoid should be considered in differential diagnosis of malignant pheochromocytoma.
  • Paroxysmal functioning or hormonally silent gastroenteropancreatic neuroendocrine tumors (GEP NETs) require repeat biochemical measurements and sensitive anatomic and functional imaging studies overlapping those for malignant pheochromocytoma.
  • NIH plasma metanephrines studies and [6]-[18F]Fluorodopamine PET ruled out malignant pheochromocytoma (pheo).
  • In combination, intensive biochemical testing repeated over time, dual-phase 64-slice MDCT with 3D image reconstruction and volume-rendering (VR) technique, and advanced radionuclide imaging are required to detect NETs' sporadic or paroxysmal functioning, rule out extra-adrenal pheochromocytoma, and localize and characterize metastatic carcinoid.
  • If pheochromocytoma is ruled out, yet symptoms and biochemical markers for catecholamine excess are present, then carcinoid and other amine-precursor-uptake decarboxylation (APUD) tumors must remain in the differential diagnosis.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Carcinoid Tumor / diagnosis. Catecholamines / secretion. Pheochromocytoma / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Humans. Neoplasm Metastasis. Positron-Emission Tomography


17. Zarnegar R, Kebebew E, Duh QY, Clark OH: Malignant pheochromocytoma. Surg Oncol Clin N Am; 2006 Jul;15(3):555-71
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  • [Title] Malignant pheochromocytoma.
  • Malignant pheochromocytoma is a rare disease with a high mortality.
  • However, differentiating between benign and malignant pheochromocytoma is impossible in the absence of locoregional invasion or distant metastasis.
  • With emerging molecular markers of malignant disease, optimal extent and approach for surgical treatment and appropriate extent of follow up could be established based on specific tumor behavior and the need for additional systemic therapy.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Pheochromocytoma / surgery

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  • (PMID = 16882497.001).
  • [ISSN] 1055-3207
  • [Journal-full-title] Surgical oncology clinics of North America
  • [ISO-abbreviation] Surg. Oncol. Clin. N. Am.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine
  • [Number-of-references] 79
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18. Powari M, Rajesh L, Joshi K: Clinically unsuspected bilateral malignant pheochromocytoma. Indian J Pathol Microbiol; 2007 Apr;50(2):313-4
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  • [Title] Clinically unsuspected bilateral malignant pheochromocytoma.
  • Pheochromocytoma is an uncommon tumor and occurrence of bilateral malignant tumor is extremely rare.
  • Herein we describe one such case ofbilateral malignant pheochromocytoma, which was clinically silent except for pain and mass in hypochondrium.
  • Histopathology showed features of malignancy along with hyaline globules, which are less described with malignant tumors.
  • Electron microscopy showed neurosecretory granules in tumor cells.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Neoplasms, Multiple Primary / pathology. Pheochromocytoma / pathology

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  • (PMID = 17883053.001).
  • [ISSN] 0377-4929
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
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19. Esfandiari NH, Shulkin BL, Bui C, Jaffe CA: Multimodality imaging of malignant pheochromocytoma. Clin Nucl Med; 2006 Dec;31(12):822-5
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  • [Title] Multimodality imaging of malignant pheochromocytoma.
  • We present a patient with malignant pheochromocytoma who underwent multitracer imaging to characterize the tumor and probe its pathophysiology to direct a therapeutic approach.
  • This case underscores the inherent difficulties in the diagnosis and localization of malignant pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Fluorodeoxyglucose F18. Indium Radioisotopes. Pheochromocytoma / diagnosis. Positron-Emission Tomography / methods. Tomography, X-Ray Computed / methods

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  • (PMID = 17117086.001).
  • [ISSN] 0363-9762
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / 5R25 CA23944; United States / NCRR NIH HHS / RR / M-01-RR00042; United States / NCI NIH HHS / CA / R01 CA54217
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Indium Radioisotopes; 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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20. Shigemura K, Tanaka K, Arakawa S, Hara I, Kawabata G, Fujisawa M: Malignant pheochromocytoma with IVC thrombus. Int Urol Nephrol; 2007;39(1):103-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant pheochromocytoma with IVC thrombus.
  • We diagnosed right pheochromocytoma with inferior vena cava (IVC) thrombus by some plasma and urine catecholamine and their metabolites data and imaging findings.
  • Pathological finding showed potentially malignant pheochromocytoma.
  • It is very important to get preoperative information of the exact location of tumor thrombus for the safe surgical management of pheochromocytoma with IVC thrombus.
  • Magnetic resonance imaging (MRI) and ultrasound imaging more useful for diagnosis of the exact location of IVC thrombus.
  • [MeSH-major] Pheochromocytoma / complications. Vena Cava, Inferior / pathology. Venous Thrombosis / complications

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  • [Cites] Hypertension. 1985 May-Jun;7(3 Pt 2):I18-24 [3997232.001]
  • [Cites] Int Urol Nephrol. 2002;34(2):241-3 [12775104.001]
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  • (PMID = 17268909.001).
  • [ISSN] 0301-1623
  • [Journal-full-title] International urology and nephrology
  • [ISO-abbreviation] Int Urol Nephrol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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21. Wakasugi S, Kinouchi T, Taniguchi H, Yokoyama K, Fukuchi K, Noguchi A, Takeshita M, Hashizume T: A case of malignant pheochromocytoma with early intense uptake and immediate rapid washout of 99mTc-tetrofosmin characterizing the overexpression of anti-apoptotic Bcl-2. Ann Nucl Med; 2006 May;20(4):325-8
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  • [Title] A case of malignant pheochromocytoma with early intense uptake and immediate rapid washout of 99mTc-tetrofosmin characterizing the overexpression of anti-apoptotic Bcl-2.
  • Defective apoptotic program due to the overexpression of the anti-apoptotic Bcl-2 protein of the outer mitochondrial membrane may be a cause of the poor response of malignant pheochromocytoma to 131I-MIBG therapy.
  • We report a case of malignant pheochromocytoma which showed early intense uptake and immediate rapid washout of 99mTc-tetrofosmin characterizing the overexpression of anti-apoptotic Bcl-2 and which was refractory to 131I-MIBG therapy.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Adrenal Gland Neoplasms / radionuclide imaging. Apoptosis Regulatory Proteins / metabolism. Organophosphorus Compounds / pharmacokinetics. Organotechnetium Compounds / pharmacokinetics. Pheochromocytoma / metabolism. Pheochromocytoma / radionuclide imaging. Proto-Oncogene Proteins c-bcl-2 / metabolism

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  • (PMID = 16856578.001).
  • [ISSN] 0914-7187
  • [Journal-full-title] Annals of nuclear medicine
  • [ISO-abbreviation] Ann Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Apoptosis Regulatory Proteins; 0 / Organophosphorus Compounds; 0 / Organotechnetium Compounds; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / Radiopharmaceuticals; 0 / technetium Tc 99m 1,2-bis(bis(2-ethoxyethyl)phosphino)ethane
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22. Kasperlik-Zaluska AA, Roslonowska E, Slowinska-Srzednicka J, Otto M, Cichocki A, Cwikla J, Slapa R, Eisenhofer G: 1,111 patients with adrenal incidentalomas observed at a single endocrinological center: incidence of chromaffin tumors. Ann N Y Acad Sci; 2006 Aug;1073:38-46
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  • [Title] 1,111 patients with adrenal incidentalomas observed at a single endocrinological center: incidence of chromaffin tumors.
  • A majority of incidentally found adrenal tumors derive from the adrenal cortex.
  • The aim of our study was evaluation of the incidence of chromaffin tumors in a group of 1,111 patients with adrenal incidentalomas.
  • Chromaffin tumors were detected in 43 patients, 33 women, and 10 men aged 20-75 years: pheochromocytoma in 36 (malignant in 3); chromaffin cells hyperplasia in 2; paraganglioma in 3; ganglioneuroblastoma in 1; ganglioneuroma in 1; and schwannoma in 2.
  • Chromaffin tumors were detected in 4% (pheochromocytomas in 3%) of 1,111 patients with adrenal incidentalomas.
  • Malignancy was present in 9% of 43 patients with chromaffin tumors.
  • High density in CT was a very important diagnostic finding in the incidentally found medullary tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Chromaffin Cells / pathology

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  • (PMID = 17102070.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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23. Zwahlen D, Fishman PN, Honey J, Milosevic M, Tannock I: Malignant pheochromocytoma of the urinary bladder. Can J Urol; 2007 Feb;14(1):3455-7
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  • [Title] Malignant pheochromocytoma of the urinary bladder.
  • This report describes an illustrative case of malignant pheochromocytoma of the urinary bladder in a 28-year old man.
  • [MeSH-major] Pheochromocytoma. Urinary Bladder Neoplasms

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  • (PMID = 17324326.001).
  • [ISSN] 1195-9479
  • [Journal-full-title] The Canadian journal of urology
  • [ISO-abbreviation] Can J Urol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Canada
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24. Scholz T, Schulz C, Klose S, Lehnert H: Diagnostic management of benign and malignant pheochromocytoma. Exp Clin Endocrinol Diabetes; 2007 Mar;115(3):155-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Diagnostic management of benign and malignant pheochromocytoma.
  • However, a comparable improvement in the prognosis of malignant chromaffin cell tumors, which occur in approximately 10-15% of all cases, has not yet been achieved.
  • Here, telomerase catalytic subunit (hTERT) activity and heat shock protein 90 expression could serve both as molecular markers allowing an earlier diagnosis of malignancy and as therapeutic targets.
  • Familial syndromes should be considered both in benign and malignant pheochromocytoma, and should be tested for prior to surgery in selected patient groups.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Genetic Techniques. Humans. Sensitivity and Specificity

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  • (PMID = 17427102.001).
  • [ISSN] 0947-7349
  • [Journal-full-title] Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
  • [ISO-abbreviation] Exp. Clin. Endocrinol. Diabetes
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 48
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25. Lam MG, Lips CJ, Jager PL, Dullaart RP, Lentjes EG, van Rijk PP, de Klerk JM: Repeated [131I]metaiodobenzylguanidine therapy in two patients with malignant pheochromocytoma. J Clin Endocrinol Metab; 2005 Oct;90(10):5888-95
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  • [Title] Repeated [131I]metaiodobenzylguanidine therapy in two patients with malignant pheochromocytoma.
  • CONTEXT: Approximately 10% of pheochromocytomas are malignant with a 5-yr survival rate of less than 40%.
  • Promising results have been published on single high-dosage [131I]metaiodobenzylguanidine ([131I]MIBG) treatment for malignant pheochromocytoma.
  • SETTING AND PATIENTS: The study took place at University Medical Centers and included two patients (one male, 36 yr of age, and one female, 43 yr of age) with widely spread metastatic pheochromocytoma and bad prognosis because of liver and lung metastases.
  • INTERVENTIONS: Instead of a single high dosage, these two patients were treated with multiple intermediate dosages of [131I]MIBG.
  • RESULTS: Both patients had a complete symptomatic response and a partial tumor volume response.
  • The survival (so far) of these patients was 5 yr (clinical case 1) and 16 yr (clinical case 2) after initial diagnosis.
  • CONCLUSIONS: Repeated intermediate-dosage [131I]MIBG treatment appears to be a reliable and well-tolerated radionuclide therapy and might be a useful adjunct in patients with malignant pheochromocytoma, providing longstanding palliation and prolonged survival.
  • [MeSH-major] 3-Iodobenzylguanidine / therapeutic use. Adrenal Gland Neoplasms / radiotherapy. Pheochromocytoma / radiotherapy. Radiopharmaceuticals / therapeutic use

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  • (PMID = 16046591.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Catecholamines; 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine
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26. Scholz T, Eisenhofer G, Pacak K, Dralle H, Lehnert H: Clinical review: Current treatment of malignant pheochromocytoma. J Clin Endocrinol Metab; 2007 Apr;92(4):1217-25
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical review: Current treatment of malignant pheochromocytoma.
  • CONTEXT: Pheochromocytomas are rare tumors of predominantly adrenal origin that often produce and secrete catecholamines.
  • Malignancy occurs in a variable percentage of cases depending on genetic background and tumor location.
  • Definitive diagnosis relies on the detection of distant metastases.
  • Treatments for malignant pheochromocytoma include surgical debulking, pharmacological control of hormone-mediated symptoms, targeted methods such as external irradiation, and systemic antineoplastic therapy.
  • EVIDENCE ACQUISITION: Literature on antineoplastic therapies for malignant pheochromocytoma was identified by searching the PubMed database with restriction to articles published in English during the past 30 yr.
  • EVIDENCE SYNTHESIS: Because of the rarity of the condition, no randomized clinical trials concerning the treatment of malignant pheochromocytoma have been performed.
  • [MeSH-major] Adrenal Gland Neoplasms / drug therapy. Adrenal Gland Neoplasms / radiotherapy. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Pheochromocytoma / drug therapy. Pheochromocytoma / radiotherapy

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  • (PMID = 17284633.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Iodine Radioisotopes; 35MRW7B4AD / 3-Iodobenzylguanidine; 5J49Q6B70F / Vincristine; 7GR28W0FJI / Dacarbazine; 8N3DW7272P / Cyclophosphamide; CVD protocol
  • [Number-of-references] 117
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27. Dossett LA, Rudzinski ER, Blevins LS, Chambers EP Jr: Malignant pheochromocytoma of the organ of Zuckerkandl requiring aortic and vena caval reconstruction. Endocr Pract; 2007 Sep;13(5):493-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant pheochromocytoma of the organ of Zuckerkandl requiring aortic and vena caval reconstruction.
  • OBJECTIVE: To describe a case of a malignant pheochromocytoma located in the organ of Zuckerkandl that required aortic and vena caval resection and reconstruction.
  • RESULTS: A 46-year-old man was referred for evaluation of a 1.4-cm left adrenal mass incidentally discovered on an abdominopelvic computed tomography (CT) scan.
  • Imaging characteristics of the left adrenal mass were consistent with a benign adenoma, but CT also demonstrated a hypervascular para-aortic mass.
  • 123I-metaiodo-benzylguanidine scanning with fusion CT imaging demonstrated increased radiopharmaceutical uptake within the para-aortic mass consistent with a paraganglioma in the organ of Zuckerkandl.
  • The remaining right ureter and kidney were removed to avoid the possibility of a urine leak from an ureteroureterostomy.
  • Final pathologic and operative findings confirmed a malignant pheochromocytoma of the organ of Zuckerkandl with invasion into the wall of the inferior vena cava and tumor thrombus extending into the lumen.
  • CONCLUSION: Malignant pheochromocytoma of the organ of Zuckerkandl involving the aorta and inferior vena cava is exceedingly rare, and although surgical resection and reconstruction can be radical and aggressive, this treatment offers the only chance for cure.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / surgery. Aorta, Abdominal / surgery. Para-Aortic Bodies / pathology. Pheochromocytoma / pathology. Pheochromocytoma / surgery. Vena Cava, Inferior / surgery

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  • (PMID = 17872352.001).
  • [ISSN] 1934-2403
  • [Journal-full-title] Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists
  • [ISO-abbreviation] Endocr Pract
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Iodine Radioisotopes
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28. Ezuddin S, Fragkaki C: MIBG and FDG PET findings in a patient with malignant pheochromocytoma: a significant discrepancy. Clin Nucl Med; 2005 Aug;30(8):579-81
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] MIBG and FDG PET findings in a patient with malignant pheochromocytoma: a significant discrepancy.
  • However, recent studies with positron emission tomography (PET) using 2-[fluorine-18]fluoro-2-deoxy-D-glucose (FDG) in pheochromocytomas have shown that FDG PET imaging can be useful in those pheochromocytomas (usually malignant) that fail to accumulate MIBG.
  • The therapeutic plan of malignant pheochromocytoma can include chemotherapy and/or a high dose of I-131 MIBG, so precise staging and characterization is mandatory for correct management and treatment.
  • [MeSH-major] 3-Iodobenzylguanidine. Adrenal Gland Neoplasms / radionuclide imaging. Fluorodeoxyglucose F18. Pheochromocytoma / radionuclide imaging. Positron-Emission Tomography. Radiopharmaceuticals
  • [MeSH-minor] Adult. Follow-Up Studies. Humans. Male. Neoplasm Metastasis. Neoplasm Recurrence, Local / radionuclide imaging. Neoplasm Staging. Patient Care Planning

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  • (PMID = 16024963.001).
  • [ISSN] 0363-9762
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18; 35MRW7B4AD / 3-Iodobenzylguanidine
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29. Ahlman H: Malignant pheochromocytoma: state of the field with future projections. Ann N Y Acad Sci; 2006 Aug;1073:449-64
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  • [Title] Malignant pheochromocytoma: state of the field with future projections.
  • The prevalence of malignant pheochromocytoma is about 10%, and is somewhat higher for paraganglioma.
  • At the first international symposium on pheochromocytoma in Bethesda (2005) experts from different disciplines and patients shared their experiences, and the present knowledge of this rare disease was updated.
  • The discussion related to future strategies for better clinical/histopathologic diagnosis and understanding of different geno- and phenotypes.
  • The main therapeutic goal is therefore often tumor reduction and control of hypertension.
  • Certain patients may benefit from 131I-MIBG combined with radiotherapy via somatostatin receptors expressed by the tumor, or the combination with chemotherapy.
  • [MeSH-major] Adrenal Gland Neoplasms / epidemiology. Pheochromocytoma / epidemiology


30. Takano A, Oriuchi N, Tsushima Y, Taketomi-Takahashi A, Nakajima T, Arisaka Y, Higuchi T, Amanuma M, Endo K: Detection of metastatic lesions from malignant pheochromocytoma and paraganglioma with diffusion-weighted magnetic resonance imaging: comparison with 18F-FDG positron emission tomography and 123I-MIBG scintigraphy. Ann Nucl Med; 2008 Jun;22(5):395-401
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  • [Title] Detection of metastatic lesions from malignant pheochromocytoma and paraganglioma with diffusion-weighted magnetic resonance imaging: comparison with 18F-FDG positron emission tomography and 123I-MIBG scintigraphy.
  • OBJECTIVE: To investigate the diagnostic features of whole-body diffusion-weighted magnetic resonance imaging (DWI) as compared with 2-[(18)F]-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) and (123)I-meta-iodo-benzyl guanidine scintigraphy (MIBG) on metastatic lesions of patients with malignant pheochromocytoma or paraganglioma.
  • METHODS: We prospectively studied 11 patients with histologically confirmed pheochromocytoma/paraganglioma and possible metastatic lesions.
  • [MeSH-major] 3-Iodobenzylguanidine. Diffusion Magnetic Resonance Imaging / methods. Fluorodeoxyglucose F18. Paraganglioma / diagnosis. Paraganglioma / secondary. Pheochromocytoma / diagnosis. Pheochromocytoma / secondary. Positron-Emission Tomography / methods

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  • (PMID = 18600417.001).
  • [ISSN] 0914-7187
  • [Journal-full-title] Annals of nuclear medicine
  • [ISO-abbreviation] Ann Nucl Med
  • [Language] eng
  • [Publication-type] Comparative Study; Evaluation Studies; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0Z5B2CJX4D / Fluorodeoxyglucose F18; 35MRW7B4AD / 3-Iodobenzylguanidine
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31. Wan WH, Tan KY, Ng C, Tay KH, Mancer K, Tay MH, Chia WK, Soo KC, Ooi LL: Metastatic malignant phaeochromocytoma: A rare entity that underlies a therapeutic quandary. Asian J Surg; 2006 Oct;29(4):294-302
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  • [Title] Metastatic malignant phaeochromocytoma: A rare entity that underlies a therapeutic quandary.
  • Phaeochromocytoma is a rare condition that provides a diagnostic challenge as a result of its variable presentation.
  • Treatment of metastatic malignant phaeochromocytoma is also not well defined owing to its rarity.
  • The database of the Singapore Cancer Registry was used to trace all cases of metastatic malignant phaeochromocytoma from 1984 to 2004, and the case records were then reviewed retrospectively.
  • There were four patients with metastatic malignant phaeochromocytoma seen in Singapore in the last 20 years.
  • We further discuss the difficulties in diagnosis, and the dilemma in appropriate management of such cases.
  • Phaeochromocytoma remains a commonly missed diagnosis unless a high index of suspicion is maintained.
  • Malignant phaeochromocytoma has a variable clinical course.

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  • (PMID = 17098666.001).
  • [ISSN] 1015-9584
  • [Journal-full-title] Asian journal of surgery
  • [ISO-abbreviation] Asian J Surg
  • [Language] ENG
  • [Publication-type] Case Reports; Comparative Study; Journal Article
  • [Publication-country] China
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32. Brouwers FM, Elkahloun AG, Munson PJ, Eisenhofer G, Barb J, Linehan WM, Lenders JW, De Krijger R, Mannelli M, Udelsman R, Ocal IT, Shulkin BL, Bornstein SR, Breza J, Ksinantova L, Pacak K: Gene expression profiling of benign and malignant pheochromocytoma. Ann N Y Acad Sci; 2006 Aug;1073:541-56
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  • [Title] Gene expression profiling of benign and malignant pheochromocytoma.
  • There are currently no reliable diagnostic and prognostic markers or effective treatments for malignant pheochromocytoma.
  • This study used oligonucleotide microarrays to examine gene expression profiles in pheochromocytomas from 90 patients, including 20 with malignant tumors, the latter including metastases and primary tumors from which metastases developed.
  • Other subgroups of tumors included those defined by tissue norepinephrine compared to epinephrine contents (i.e., noradrenergic versus adrenergic phenotypes), adrenal versus extra-adrenal locations, and presence of germline mutations of genes predisposing to the tumor.
  • Correcting for the confounding influence of noradrenergic versus adrenergic catecholamine phenotype by the analysis of variance revealed a larger and more accurate number of genes that discriminated benign from malignant pheochromocytomas than when the confounding influence of catecholamine phenotype was not considered.
  • Seventy percent of these genes were underexpressed in malignant compared to benign tumors.
  • Similarly, 89% of genes were underexpressed in malignant primary tumors compared to benign tumors, suggesting that malignant potential is largely characterized by a less-differentiated pattern of gene expression.
  • The present database of differentially expressed genes provides a unique resource for mapping the pathways leading to malignancy and for establishing new targets for treatment and diagnostic and prognostic markers of malignant disease.
  • Further progress on the basis of this database can be made from follow-up confirmatory studies, application of bioinformatics approaches for data mining and pathway analyses, testing in pheochromocytoma cell culture and animal model systems, and retrospective and prospective studies of diagnostic markers.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Gene Expression Profiling. Pheochromocytoma / genetics

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  • (PMID = 17102123.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z99 CT999999; United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
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33. Park KS, Lee JL, Ahn H, Koh JM, Park I, Choi JS, Kim YR, Park TS, Ahn JH, Lee DH, Kim TW, Lee JS: Sunitinib, a novel therapy for anthracycline- and cisplatin-refractory malignant pheochromocytoma. Jpn J Clin Oncol; 2009 May;39(5):327-31
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  • [Title] Sunitinib, a novel therapy for anthracycline- and cisplatin-refractory malignant pheochromocytoma.
  • We report a case of malignant pheochromocytoma recurred after debulking surgery.
  • Level of urinary catecholamine and its metabolite increased above normal values and abdominal computed tomography showed a huge right adrenal mass.
  • The tumor showed very good metabolic response to the therapy.
  • In patient with malignant pheochromocytoma, sunitinib might be one therapeutic strategy for malignant pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / drug therapy. Antineoplastic Agents / therapeutic use. Indoles / therapeutic use. Pheochromocytoma / drug therapy. Pyrroles / therapeutic use
  • [MeSH-minor] Adolescent. Anthracyclines / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cisplatin / administration & dosage. Drug Resistance, Neoplasm. Humans. Liver Neoplasms / secondary. Male. Mediastinal Neoplasms / secondary

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  • (PMID = 19264767.001).
  • [ISSN] 1465-3621
  • [Journal-full-title] Japanese journal of clinical oncology
  • [ISO-abbreviation] Jpn. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Anthracyclines; 0 / Antineoplastic Agents; 0 / Indoles; 0 / Pyrroles; 0 / sunitinib; Q20Q21Q62J / Cisplatin
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34. Watanabe D, Tanabe A, Naruse M, Tsuiki M, Torii N, Noshiro T, Takano K: Transcatheter arterial embolization for the treatment of liver metastases in a patient with malignant pheochromocytoma. Endocr J; 2006 Feb;53(1):59-66
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  • [Title] Transcatheter arterial embolization for the treatment of liver metastases in a patient with malignant pheochromocytoma.
  • A 63-year-old male patient was admitted for the treatment of malignant pheochromocytoma with multiple liver metastases.
  • Plasma CgA level is a useful marker in the treatment of malignant pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Embolization, Therapeutic / methods. Liver Neoplasms / secondary. Liver Neoplasms / therapy. Pheochromocytoma / secondary. Pheochromocytoma / therapy

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  • (PMID = 16543673.001).
  • [ISSN] 0918-8959
  • [Journal-full-title] Endocrine journal
  • [ISO-abbreviation] Endocr. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Adrenergic alpha-Antagonists; 0 / Antibiotics, Antineoplastic; 0 / Catecholamines; 0 / Chromogranin A; 0 / Chromogranins; 50SG953SK6 / Mitomycin
  • [Number-of-references] 24
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35. Gullu S, Gursoy A, Erdogan MF, Dizbaysak S, Erdogan G, Kamel N: Multiple endocrine neoplasia type 2A/localized cutaneous lichen amyloidosis associated with malignant pheochromocytoma and ganglioneuroma. J Endocrinol Invest; 2005 Sep;28(8):734-7
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  • [Title] Multiple endocrine neoplasia type 2A/localized cutaneous lichen amyloidosis associated with malignant pheochromocytoma and ganglioneuroma.
  • We hereby present a rare variant of multiple endocrine neoplasia type 2A (MEN2A) associated with a rare skin disease primary cutaneous lichen amyloidosis and discrete malignant pheochromocytoma in both adrenal glands and pancreatic tail, and interestingly accompanied ganglioneuroma located in retroperitoneum in a 34-yr-old female.
  • The presence of composite tumor of pheochromocytoma and ganglioneuroma arising in the adrenal glands has been described previously in MEN2A and in sporadic cases.
  • Biochemical screening proved pheochromocytoma.
  • Computed tomography revealed multiple mass lesions in both adrenal glands.
  • It also showed a large heterogeneous mass that clearly discriminated from right adrenal gland in retroperitoneal location.
  • After surgical exploration, both adrenal glands and the suspicious mass in pancreatic tail were removed successfully together with subtotal resection of the retroperitoneal tumor.
  • Histopathologic examinations confirmed the presence of pheochromocytoma in both adrenal glands as well as pancreatic lesion.
  • Symptomatic and biochemical evidence of pheochromocytoma subsided after the operation.
  • Further evaluation for medullary thyroid carcinoma and primary hyperparathyroidism confirmed MEN2A.
  • Mutation analysis of the ret proto-oncogene revealed a missense point mutation at position 634 in exon 11, which gives rise to the substitution of a cysteine codon with a tyrosine residue.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Ganglioneuroma / pathology. Multiple Endocrine Neoplasia Type 2a / pathology. Pheochromocytoma / secondary. Retroperitoneal Neoplasms / pathology


36. Shilkrut M, Bar-Deroma R, Bar-Sela G, Berniger A, Kuten A: Low-dose iodine-131 metaiodobenzylguanidine therapy for patients with malignant pheochromocytoma and paraganglioma: single center experience. Am J Clin Oncol; 2010 Feb;33(1):79-82
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  • [Title] Low-dose iodine-131 metaiodobenzylguanidine therapy for patients with malignant pheochromocytoma and paraganglioma: single center experience.
  • The following is a report on the clinical experience of an Israeli referral center for iodine-131 metaiodobenzylguanidine (131-MIBG) therapy for malignant pheochromocytoma (MPCC) and malignant paraganglioma (MPGG).
  • Response to 131-MIBG therapy was evaluated by tumor, hormone, and symptomatic relief criteria.
  • Three patients (30%) had partial tumor response, 5 (50%) had stable disease, and 2 (20%) progressed after therapy.
  • [MeSH-major] 3-Iodobenzylguanidine / administration & dosage. Adrenal Gland Neoplasms / drug therapy. Antineoplastic Agents / administration & dosage. Paraganglioma / drug therapy. Pheochromocytoma / drug therapy
  • [MeSH-minor] Adult. Aged. Dose-Response Relationship, Radiation. Female. Follow-Up Studies. Humans. Iodine Radioisotopes. Male. Middle Aged. Neoplasm Staging. Prognosis. Survival Rate. Treatment Outcome. Young Adult

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  • (PMID = 19704370.001).
  • [ISSN] 1537-453X
  • [Journal-full-title] American journal of clinical oncology
  • [ISO-abbreviation] Am. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Iodine Radioisotopes; 35MRW7B4AD / 3-Iodobenzylguanidine
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37. Druce MR, Kaltsas GA, Fraenkel M, Gross DJ, Grossman AB: Novel and evolving therapies in the treatment of malignant phaeochromocytoma: experience with the mTOR inhibitor everolimus (RAD001). Horm Metab Res; 2009 Sep;41(9):697-702
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  • [Title] Novel and evolving therapies in the treatment of malignant phaeochromocytoma: experience with the mTOR inhibitor everolimus (RAD001).
  • Phaeochromocytoma and paraganglioma are rare neuroendocrine tumours (NETS).
  • They may be benign or malignant but the pathological distinction is mainly made when metastases are present.
  • This short review outlines the main molecular and histological features of malignant phaeochromocytoma and the difficulties in differentiating between benign and malignant disease.
  • We list current therapies used for malignant pheochromocytoma; however, these generally achieve relatively low success rates.
  • In vitro studies have implicated the PI3/Akt/mTOR pathway in the pathogenesis of malignant NETS, including phaeochromocytoma.
  • We have used RAD001 in four patients with progressive malignant paraganglioma/phaeochromocytoma in addition to other therapies (with institutional approval for compassionate use), and evaluated the effects of this treatment.
  • [MeSH-major] Adrenal Gland Neoplasms / drug therapy. Pheochromocytoma / drug therapy. Protein Kinase Inhibitors / therapeutic use. Sirolimus / analogs & derivatives

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  • (PMID = 19424940.001).
  • [ISSN] 1439-4286
  • [Journal-full-title] Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et métabolisme
  • [ISO-abbreviation] Horm. Metab. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Protein Kinase Inhibitors; 9HW64Q8G6G / Everolimus; EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; W36ZG6FT64 / Sirolimus
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38. Yoshihara A, Tanabe A, Saito H, Hizuka N, Ishizawa A, Horikawa R, Takano K: A case of malignant pheochromocytoma with Holt-Oram syndrome. Endocr J; 2008 Mar;55(1):153-9
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  • [Title] A case of malignant pheochromocytoma with Holt-Oram syndrome.
  • A 23-year-old female patient with malignant pheochromocytoma was admitted to the Tokyo Women's Medical University.
  • Since she had complex congenital heart disease, chronic heart failure, and severe hypoxia, the risk surrounding surgery to remove the primary tumor was predicted to be very high, and subsequently, chemotherapy was performed.
  • To our knowledge, this is the first report of a patient with the combination of malignant pheochromocytoma and Holt-Oram syndrome.
  • A correlation between chronic hypoxia and pheochromocytoma has been reported.
  • This instructive case reminds us to consider the possibility of pheochromocytoma with congenital heart disease when these types of unexpected or unusual symptoms are encountered.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Adrenal Gland Neoplasms / diagnosis. Heart Diseases / complications. Heart Diseases / congenital. Pheochromocytoma / complications. Pheochromocytoma / diagnosis


39. Hidaka S, Hiraoka A, Ochi H, Uehara T, Ninomiya T, Miyamoto Y, Hasebe A, Tanihira T, Tanabe A, Ichiryu M, Nakahara H, Tazuya N, Ninomiya I, Michitaka K: Malignant pheochromocytoma with liver metastasis treated by transcatheter arterial chemo-embolization (TACE). Intern Med; 2010;49(7):645-51
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  • [Title] Malignant pheochromocytoma with liver metastasis treated by transcatheter arterial chemo-embolization (TACE).
  • Multiple liver metastatic lesions were shown by computed tomography and scintigraphy with 123I- metaiodobenzylguanidine (MIBG) in a 76-year-old woman 2 years after resection of a pheochromocytoma of the right adrenal gland.
  • TACE with strict blood pressure control was an effective and safe therapy for multiple liver metastatic lesions related to malignant pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / therapy. Antineoplastic Agents / administration & dosage. Chemoembolization, Therapeutic / methods. Liver Neoplasms / therapy. Pheochromocytoma / therapy

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  • (PMID = 20371953.001).
  • [ISSN] 1349-7235
  • [Journal-full-title] Internal medicine (Tokyo, Japan)
  • [ISO-abbreviation] Intern. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 18
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40. Dural C, Bilge O, Toker A, Erbil Y, Salmaslioglu A, Ozbey N, Aral F: Malignant phaeochromocytoma with cavoatrial extension: transcaval removal of tumor without cardiopulmonary bypass A case report. Minerva Chir; 2010 Aug;65(4):485-8
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  • [Title] Malignant phaeochromocytoma with cavoatrial extension: transcaval removal of tumor without cardiopulmonary bypass A case report.
  • Adrenal carcinoma is a rare tumor and with metastasis usually in lungs, lymph nodes, liver, and bones.
  • The surgical approach to adrenal tumor extending into the vena cava is challenging.
  • The optimal surgical approach of tumor with inferior vena cava extension depends on the level of vena cava involvement.
  • This article reports a case of malignant pheochromocytoma extending into the cavoatrial junction in a young man.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Heart Atria. Heart Neoplasms / secondary. Pheochromocytoma / pathology. Vena Cava, Inferior
  • [MeSH-minor] Adolescent. Adrenalectomy. Cardiopulmonary Bypass / methods. Humans. Male. Neoplasm Invasiveness. Treatment Outcome

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  • (PMID = 20802436.001).
  • [ISSN] 0026-4733
  • [Journal-full-title] Minerva chirurgica
  • [ISO-abbreviation] Minerva Chir
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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41. Nomura K, Kimura H, Shimizu S, Kodama H, Okamoto T, Obara T, Takano K: Survival of patients with metastatic malignant pheochromocytoma and efficacy of combined cyclophosphamide, vincristine, and dacarbazine chemotherapy. J Clin Endocrinol Metab; 2009 Aug;94(8):2850-6
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  • [Title] Survival of patients with metastatic malignant pheochromocytoma and efficacy of combined cyclophosphamide, vincristine, and dacarbazine chemotherapy.
  • CONTEXT: About 10% of pheochromocytomas are malignant.
  • OBJECTIVE: The aim of this study was to analyze the survival curves and survival times of patients with malignant pheochromocytoma and to determine the efficacy of chemotherapy on prolongation of life.
  • PATIENTS AND OUTCOME MEASURES: Thirty-two patients with metastasized malignant pheochromocytoma were analyzed for survival.
  • RESULTS: The survival curve of the 32 patients declined continuously and linearly to at least 20 yr after the diagnosis of pheochromocytoma.
  • In the 25 patients whose primary tumor was excised, patients who already had metastases at the time of pheochromocytoma diagnosis had better survival than those whose metastases were found later.
  • The survival rate after diagnosis of metastasis was worse in the CVD group than in controls.
  • When the effects of CVD were examined after stratifying several factors, female gender and adrenal origin of tumor were found to be negative prognostic factors for CVD chemotherapy.
  • CVD chemotherapy was not shown to extend survival, especially for women and patients with adrenal gland-derived primary tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / mortality. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Pheochromocytoma / mortality
  • [MeSH-minor] Adult. Aged. Cyclophosphamide / administration & dosage. Dacarbazine / administration & dosage. Female. Humans. Male. Middle Aged. Neoplasm Metastasis. Survival Rate. Vincristine / administration & dosage

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  • (PMID = 19470630.001).
  • [ISSN] 1945-7197
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7GR28W0FJI / Dacarbazine; 8N3DW7272P / Cyclophosphamide
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42. Holwitt D, Neifeld J, Massey G, Lanning D: Case report of an 11-year-old child with a nonfunctional malignant pheochromocytoma. J Pediatr Surg; 2007 Nov;42(11):E13-5
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  • [Title] Case report of an 11-year-old child with a nonfunctional malignant pheochromocytoma.
  • Pheochromocytoma is an unusual cause of surgical hypertension and is extremely rare in the pediatric population.
  • We present a case of a hypertension-producing malignant pheochromocytoma in an 11-year-old, which was initially unresectable.
  • The tumor responded partially to aggressive chemotherapy and was completely resected.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / therapy. Pheochromocytoma / pathology. Pheochromocytoma / therapy
  • [MeSH-minor] Adrenalectomy / methods. Biopsy, Needle. Blood Pressure Determination. Chemotherapy, Adjuvant. Child. Female. Follow-Up Studies. Humans. Immunohistochemistry. Laparotomy / methods. Magnetic Resonance Angiography. Magnetic Resonance Imaging. Neoplasm Staging. Risk Assessment. Severity of Illness Index. Treatment Outcome

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  • (PMID = 18022420.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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43. Kopetschke R, Slisko M, Kilisli A, Tuschy U, Wallaschofski H, Fassnacht M, Ventz M, Beuschlein F, Reincke M, Reisch N, Quinkler M: Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma. Eur J Endocrinol; 2009 Aug;161(2):355-61
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  • [Title] Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma.
  • CONTEXT: Adrenal and extra-adrenal phaeochromocytoma are chromaffin cell-derived tumours that are discovered due to classical symptom triad with headache, sweating and palpitations combined with persistent or paroxysmal hypertension.
  • However, an increasing proportion of phaeochromocytoma seems to be discovered incidentally upon abdominal imaging.
  • OBJECTIVE: To specify the exact circumstances of discovery of adrenal and extra-adrenal phaeochromocytoma.
  • MEDICAL: records of 201 patients with adrenal and extra-adrenal phaeochromocytoma who were diagnosed between 1973 and 2007 were analyzed.
  • In 24 patients (12.2%), phaeochromocytoma was malignant.
  • Patients with incidentally detected phaeochromocytoma were significantly older (53.1+/-1.9 vs 47.0+/-1.3 years; P<0.05) and often had less blood pressure peaks (37.0 vs 70.7%; P<0.001) than patients in whom the diagnosis was suspected on clinical grounds.
  • Of phaeochromocytomas 94.4% were intra-adrenal tumours, of which 12.9% were bilateral.
  • Bilateral tumours were significantly smaller than unilateral tumours (36.6+/-14.7 vs 52.5+/-34.3 mm; P<0.05), whereas extra-adrenal tumours had a mean diameter of 52.6+/-28.7 mm.
  • CONCLUSIONS: Owing to better availability and accessibility of imaging procedures, the number of incidentally discovered phaeochromocytoma is increasing and reaches nearly 30% in our study population.
  • Every adrenal incidentaloma should be investigated for the presence of phaeochromocytoma.

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  • (PMID = 19497985.001).
  • [ISSN] 1479-683X
  • [Journal-full-title] European journal of endocrinology
  • [ISO-abbreviation] Eur. J. Endocrinol.
  • [Language] ENG
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] England
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44. Park JW, Namkoong S, Chung J, Jung KE, Oh SA, Cinn YW, Kim MH: A case of eccrine spiradenoma in a patient with neurofibromatosis. Ann Dermatol; 2010 May;22(2):191-3

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  • Some benign or malignant tumors such as juvenile xanthogranuloma, pheochromocytoma, and malignant melanoma can accompany neurofibromatosis.
  • Eccrine spiradenoma is a benign uncommon neoplasm of skin adnexa.

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  • [Cites] Arch Dermatol. 1990 Aug;126(8):1051-6 [2166484.001]
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  • (PMID = 20548911.001).
  • [ISSN] 2005-3894
  • [Journal-full-title] Annals of dermatology
  • [ISO-abbreviation] Ann Dermatol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC2883423
  • [Keywords] NOTNLM ; Eccrine spiradenoma / Neurofibromatosis
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45. Tavangar SM, Shojaee A, Moradi Tabriz H, Haghpanah V, Larijani B, Heshmat R, Lashkari A, Azimi S: Immunohistochemical expression of Ki67, c-erbB-2, and c-kit antigens in benign and malignant pheochromocytoma. Pathol Res Pract; 2010 May 15;206(5):305-9
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  • [Title] Immunohistochemical expression of Ki67, c-erbB-2, and c-kit antigens in benign and malignant pheochromocytoma.
  • In this study, we evaluated the immunohistochemical expression and the possible advantages of Ki67 antigen, c-erbB-2, and c-kit proto-oncogenes in the differentiation between benign and malignant pheochromocytomas.
  • Paraffin-embedded tissue blocks from 44 benign (35 adrenal and 9 extra-adrenal) and 11 malignant (9 adrenal and 2 extra-adrenal) cases of pheochromocytoma were selected for immunohistochemical staining using antibodies against Ki67, c-erbB-2, and c-kit antigens.
  • We investigated the relationship between the expression of these antigens and age, gender, tumor size, histologic patterns and necrosis, as well as tumor behavior.
  • The risk of malignancy was higher when tumor size was increased.
  • There was a statistically significant relationship between Ki67- and c-erbB-2-positive staining of the tumor cells and the malignant behavior of the pheochromocytomas (p-value=0.000), while cytoplasmic c-kit staining did not show any correlation with tumor malignancy (p-value=0.087).
  • We concluded that tumor size and the histomorphologic patterns (spindle cell and small round cell) are significantly associated with tumor behavior.
  • In addition, Ki67 positivity and c-erbB-2 expression can be used as immunohistochemical markers for predicting the malignant behavior of pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Ki-67 Antigen / metabolism. Pheochromocytoma / metabolism. Proto-Oncogene Proteins c-kit / metabolism. Receptor, ErbB-2 / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Biomarkers, Tumor / metabolism. Child. Female. Humans. Immunohistochemistry. Male. Middle Aged

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  • [Copyright] (c) 2010 Elsevier GmbH. All rights reserved.
  • (PMID = 20189725.001).
  • [ISSN] 1618-0631
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; EC 2.7.10.1 / Receptor, ErbB-2
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46. Kizu H, Takayama T, Tsushima H, Noguchi A, Nakajima K, Onoguchi M, Kinuya S: Localization of metastases from malignant pheochromocytoma in patients undergoing 131I-MIBG therapy with manually fused 123I-MIBG SPECT and CT images. J Nucl Med Technol; 2008 Dec;36(4):186-8
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  • [Title] Localization of metastases from malignant pheochromocytoma in patients undergoing 131I-MIBG therapy with manually fused 123I-MIBG SPECT and CT images.
  • (131)I-metaiodobenzylguanidine (MIBG) has been used as a therapeutic agent for pheochromocytoma.
  • Tumor localization and precise staging are essential for therapy with high-dose (131)I-MIBG.
  • [MeSH-major] 3-Iodobenzylguanidine / therapeutic use. Adrenal Gland Neoplasms / diagnosis. Pheochromocytoma / diagnosis. Pheochromocytoma / secondary. Subtraction Technique. Tomography, Emission-Computed, Single-Photon / methods. Tomography, X-Ray Computed / methods

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  • (PMID = 19008288.001).
  • [ISSN] 0091-4916
  • [Journal-full-title] Journal of nuclear medicine technology
  • [ISO-abbreviation] J Nucl Med Technol
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Validation Studies
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine
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47. Rufini V, Calcagni ML, Baum RP: Imaging of neuroendocrine tumors. Semin Nucl Med; 2006 Jul;36(3):228-47
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  • Radiolabeled metaiodobenzylguanidine (MIBG) was the first radiopharmaceutical used to specifically depict and localize catecholamine-secreting tumors (pheochromocytomas, paragangliomas, and neuroblastomas) and is still regarded as a first-choice imaging technique for diagnosis and follow-up; in patients with malignant disease, MIBG scintigraphy is an essential step to select patients for (131)I-MIBG therapy.
  • Scintigraphy with (111)In- or (99m)Tc-labeled somatostatin analogs has become the main imaging technique for NETs, particularly those expressing a high density of somatostatin receptors, such as gastroenteropancreatic tumors; this procedure is used routinely for localizing the primary tumor, evaluating disease extension, monitoring the effect of treatment and for selecting patients for radioreceptor therapy.
  • [MeSH-minor] 3-Iodobenzylguanidine / therapeutic use. ACTH Syndrome, Ectopic / etiology. ACTH Syndrome, Ectopic / radionuclide imaging. Adrenal Gland Neoplasms / radionuclide imaging. Adult. Carcinoma, Medullary / radionuclide imaging. Catecholamines / secretion. Child. Gastrointestinal Neoplasms / radionuclide imaging. Humans. Iodine Radioisotopes / therapeutic use. Middle Aged. Neuroblastoma / radionuclide imaging. Neuroblastoma / secondary. Octreotide. Paraganglioma / radionuclide imaging. Pheochromocytoma / radionuclide imaging. Positron-Emission Tomography. Radiopharmaceuticals / therapeutic use. Somatostatin / analogs & derivatives. Thyroid Neoplasms / radionuclide imaging

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  • (PMID = 16762613.001).
  • [ISSN] 0001-2998
  • [Journal-full-title] Seminars in nuclear medicine
  • [ISO-abbreviation] Semin Nucl Med
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Catecholamines; 0 / Iodine Radioisotopes; 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine; 51110-01-1 / Somatostatin; RWM8CCW8GP / Octreotide
  • [Number-of-references] 210
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48. Chrisoulidou A, Kaltsas G, Ilias I, Grossman AB: The diagnosis and management of malignant phaeochromocytoma and paraganglioma. Endocr Relat Cancer; 2007 Sep;14(3):569-85
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  • [Title] The diagnosis and management of malignant phaeochromocytoma and paraganglioma.
  • Malignant phaeochromocytomas are rare tumours accounting for ~10% of all phaeochromocytomas; the prevalence of malignancy among paragangliomas is higher, especially those associated with succinate dehydrogenase subunit B gene mutations.
  • Clinical, biochemical and histological features cannot reliably distinguish malignant from benign tumours.
  • Several imaging modalities have been utilised for the diagnosis and staging of these tumours.
  • Functional imaging using radiolabelled metaiodobenzylguanidine (MIBG) and more recently, (18)F-fluorodopamine and (18)F-fluorodopa positron emission tomography offer substantial sensitivity and specificity to correctly detect metastatic phaeochromocytoma and paraganglioma and helps identify patients suitable for treatment with radiopharmaceuticals.
  • The 5-year mortality rate of patients with malignant phaeochromocytomas and paragangliomas greater than 50% indicates that there is considerable room for the improvement of currently available therapies.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Paraganglioma / diagnosis. Paraganglioma / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy
  • [MeSH-minor] Algorithms. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / analysis. Chromaffin Cells / pathology. Combined Modality Therapy. Endocrine Surgical Procedures. Humans. Radiopharmaceuticals / therapeutic use. Radiotherapy / trends

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  • (PMID = 17914089.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Radiopharmaceuticals
  • [Number-of-references] 159
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49. Yurt A, Arda MN, Vardar E: Metastatic pheochromocytoma of the thoracic spinal extradural space. Case report and review of the literature. Kobe J Med Sci; 2005;51(3-4):49-53
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  • [Title] Metastatic pheochromocytoma of the thoracic spinal extradural space. Case report and review of the literature.
  • In this case report, a thoracic extradural metastatic pheochromocytoma without bony invasion, is presented.
  • The disease which has been identified with its symptoms, bio-chemical features, radiological appearance, histological diagnosis has been discussed in comparison with malignant pheochromocytoma metastases in the literature.
  • The origin of this tumor is the adrenal glands.
  • Our review of the literature shows that tumor has primary metastasis in bony structures of the spine and then demonstrates secondary intraspinal invasion.
  • This is the first case report of an epidural metastasis from malignant pheochromocytoma without a bony invasion.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Epidural Neoplasms / secondary. Pheochromocytoma / secondary

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  • (PMID = 16444096.001).
  • [ISSN] 0023-2513
  • [Journal-full-title] The Kobe journal of medical sciences
  • [ISO-abbreviation] Kobe J Med Sci
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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50. Costa SR, Cabral NM, Abhrão AT, Costa RB, Silva LM, Lupinacci RA: Giant cystic malignant pheochromocytoma invading right hepatic lobe: report on two cases. Sao Paulo Med J; 2008 Jul;126(4):229-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Giant cystic malignant pheochromocytoma invading right hepatic lobe: report on two cases.
  • CONTEXT: Cystic pheochromocytomas are uncommon neuroendocrine tumors that originate from the adrenal medulla.
  • Similarly, radiological diagnosis may also be difficult.
  • Sometimes, there may be a preoperative diagnosis of malignancy.
  • Invasion of organs in this region (i.e. liver or kidney), or even the presence of a large retroperitoneal mass (of uncertain origin) with which multiple organs are involved, may be indicative of malignant origin.
  • CASE REPORT: Two cases of giant cystic pheochromocytoma that invaded the right hepatic lobe are described.
  • Both cases were malignant.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Liver / pathology. Pheochromocytoma / pathology
  • [MeSH-minor] Adult. Female. Hepatectomy. Humans. Immunohistochemistry. Male. Middle Aged. Neoplasm Invasiveness. Nephrectomy. Tomography, X-Ray Computed

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  • (PMID = 18853034.001).
  • [ISSN] 1806-9460
  • [Journal-full-title] São Paulo medical journal = Revista paulista de medicina
  • [ISO-abbreviation] Sao Paulo Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Brazil
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51. Adjallé R, Plouin PF, Pacak K, Lehnert H: Treatment of malignant pheochromocytoma. Horm Metab Res; 2009 Sep;41(9):687-96
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of malignant pheochromocytoma.
  • Pheochromocytoma (PCC) is a rare disease, mainly sporadic, but also associated with some familial disorders, with a malignancy frequency of approximately 10%.
  • Only the presence of distant metastases, derived from large pleomorphic chromaffin cells, is widely accepted as a criterion of malignancy.
  • Since there is no curative treatment for malignant PCC and due to its unfavorable prognosis, assuring quality of life is one of the main therapeutic objectives.
  • In case of a sufficient uptake of (123)I-MIBG treatment with targeted radiation therapy, use of (131)I-MIBG is an option as an adjuvant therapy, following debulking surgery.
  • [MeSH-major] Adrenal Gland Neoplasms / therapy. Pheochromocytoma / therapy

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  • (PMID = 19672813.001).
  • [ISSN] 1439-4286
  • [Journal-full-title] Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et métabolisme
  • [ISO-abbreviation] Horm. Metab. Res.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / ZIA HD008735-09
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 92
  • [Other-IDs] NLM/ NIHMS470438; NLM/ PMC3658628
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52. Wängberg B, Muth A, Khorram-Manesh A, Jansson S, Nilsson O, Forssell-Aronsson E, Tisell L, Ahlman H: Malignant pheochromocytoma in a population-based study: survival and clinical results. Ann N Y Acad Sci; 2006 Aug;1073:512-6
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  • [Title] Malignant pheochromocytoma in a population-based study: survival and clinical results.
  • One hundred fifty-four consecutive patients with pheochromocytoma (PC, n=137) or paraganglioma (PG, n=17) were treated at our unit.
  • Twelve had malignant tumors and were classified according to mode of presentation:.
  • Within 4 years two patients died of tumor progression. (2) Locally advanced disease (n=4), all resected for cure. (3) Malignancy disclosed during follow-up after adrenalectomy with "benign" histopathology (n=4).
  • All patients in groups 2 and 3 developed recurrence 9 (1-17) years after primary surgery; four underwent resection, one remains tumor-free.
  • This series is population-based and may better reflect the natural history of malignant PC/PG than the series from national referral centers.
  • Active surgical treatment and phenoxybenzamine resulted in low tumor-related mortality in groups 2 and 3; five patients died 8-30 years after diagnosis, four of PC/PG (three from group 2 and one from group 3) and one of other causes.
  • We propose tumor uptake studies (MIBG- and octreotide scintigraphy) in patients with nonresectable metastases; to select individual radionuclide therapy data on the expression of CA-transporters/somatostatin receptors may be helpful.
  • To diagnose PC/PG early, screening of adrenal incidentalomas has been suggested.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology

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  • (PMID = 17102119.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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53. Ibuki N, Komura K, Koyama K, Inamoto T, Segawa N, Tanimoto K, Tuji M, Azuma H, Katsuoka Y: [A pheochromocytoma of urinary bladder treated with neoadjuvant chemotherapy]. Hinyokika Kiyo; 2009 Dec;55(12):765-8
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  • [Title] [A pheochromocytoma of urinary bladder treated with neoadjuvant chemotherapy].
  • Cystoscopy revealed a submucosal tumor in the right lateral wall of the bladder.
  • Histologically, pathologic examination revealed a malignant pheochromocytoma.
  • T2-weighted magnetic resonance imaging showed a bladder tumor of high intensity and extra-bladder invasion.
  • Since the tumor size was reduced, she was referred to our hospital for operative indication.
  • Histologically, the tumor was a pheochromocytoma of the urinary bladder.
  • [MeSH-major] Pheochromocytoma / drug therapy. Urinary Bladder Neoplasms / drug therapy

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  • (PMID = 20048562.001).
  • [ISSN] 0018-1994
  • [Journal-full-title] Hinyokika kiyo. Acta urologica Japonica
  • [ISO-abbreviation] Hinyokika Kiyo
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7GR28W0FJI / Dacarbazine; 8N3DW7272P / Cyclophosphamide; CVD protocol
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54. Vaclavikova E, Dvorakova S, Sykorova V, Bilek R, Dvorakova K, Vlcek P, Skaba R, Zelinka T, Bendlova B: RET mutation Tyr791Phe: the genetic cause of different diseases derived from neural crest. Endocrine; 2009 Dec;36(3):419-24
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  • Activating germline RET mutations are presented in patients with familial medullary thyroid carcinoma (FMTC) and multiple endocrine neoplasia (MEN) types 2A and 2B, whereas inactivating germline mutations in patients with Hirschsprung's disease (HSCR).
  • Screening of three groups of patients was performed (276 families with medullary thyroid carcinoma (MTC), 122 families with HSCR, and 29 patients with pheochromocytoma).
  • We found this mutation in 3 families with apparently sporadic MTC, 3 families with FMTC/MEN2, 1 patient with pheochromocytoma, and 3 families with HSCR.
  • A rare case of malignant pheochromocytoma in a patient with the Tyr791Phe mutation is presented.
  • [MeSH-minor] Adolescent. Adult. Aged. Amino Acid Substitution / genetics. Carcinoma, Medullary / genetics. Child. Family. Female. Genetic Association Studies. Germ-Line Mutation. Hirschsprung Disease / genetics. Humans. Male. Middle Aged. Multiple Endocrine Neoplasia Type 2a / genetics. Phenylalanine / genetics. Polymorphism, Single Nucleotide / physiology. Thyroid Neoplasms / genetics. Tyrosine / genetics. Young Adult

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  • (PMID = 19826964.001).
  • [ISSN] 1559-0100
  • [Journal-full-title] Endocrine
  • [ISO-abbreviation] Endocrine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 42HK56048U / Tyrosine; 47E5O17Y3R / Phenylalanine; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret; EC 2.7.10.1 / RET protein, human
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55. Xiao ZJ, Li CL: [Diagnosis and treatment of malignant bladder non-epithelial tumors]. Zhonghua Yi Xue Za Zhi; 2008 Nov 4;88(40):2845-7
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  • [Title] [Diagnosis and treatment of malignant bladder non-epithelial tumors].
  • OBJECTIVE: To study the clinical diagnosis, treatment, and prognosis of malignant bladder non-epithelial tumors.
  • METHODS: The clinical data of 17 cases with malignant bladder non-epithelial tumor, 15 males and 3 females, aged 28 (3-73), were analyzed.
  • RESULTS: Ten of the 17 cases were diagnosed as with rhabdomyosarcoma, 2 with malignant lymphoma, and 2 with malignant pheochromocytoma, 2 with leiomyosarcoma, and 1 with carcinosarcoma.
  • Two patients with malignant lymphoma underwent partial cystectomy and adjuvant radiotherapy or chemotherapy, and survived for more than 3 years.
  • Of the 2 cases with malignant pheochromocytoma, one underwent lymphadenectomy and adjuvant chemotherapy and survived for more than 5 years; and another case died 2 years after operation and chemotherapy.
  • CONCLUSION: Malignant bladder non-epithelial tumors are rare clinically and most of them occur in children.
  • The prognosis of malignant bladder non-epithelial tumors, different in pathological types, is relatively worse in adults.
  • [MeSH-major] Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / therapy. Urinary Bladder Neoplasms / diagnosis. Urinary Bladder Neoplasms / therapy

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  • (PMID = 19080495.001).
  • [ISSN] 0376-2491
  • [Journal-full-title] Zhonghua yi xue za zhi
  • [ISO-abbreviation] Zhonghua Yi Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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56. Hasse-Lazar K, Krajewska J, Paliczka-Cieślik E, Jurecka-Lubieniecka B, Michalik B, Handkiewicz-Junak D, Roskosz J, Jarzab B: [131I-MIBG therapy in the treatment of pheochromocytoma in children--own experiences]. Endokrynol Pol; 2008 May-Jun;59(3):235-40
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  • [Title] [131I-MIBG therapy in the treatment of pheochromocytoma in children--own experiences].
  • Three cases of pheochromocytoma in children/adolescents or young adults treated by 131I-MIBG are presented in this study.
  • In one patient 131I-MIBG was administrated after ineffective surgical treatment and chemotherapy of a benign retroperitoneal tumor, whereas in two other patients 131I-MIBG therapy was carried out because of malignant pheochromocytoma dissemination.
  • In a child with retroperitoneal paraganglioma decrease of tumor size and its fibrosis after 131I-MIBG therapy allowed radical surgery and complete recovery.
  • The presented data confirm effectiveness and acceptable tolerance of 131I-MIBG treatment in pheochromocytoma, what is very important in pediatric patients.
  • [MeSH-major] 3-Iodobenzylguanidine / therapeutic use. Adrenal Gland Neoplasms / radiotherapy. Antineoplastic Agents / therapeutic use. Iodine Radioisotopes / therapeutic use. Pheochromocytoma / radiotherapy

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  • (PMID = 18615399.001).
  • [ISSN] 0423-104X
  • [Journal-full-title] Endokrynologia Polska
  • [ISO-abbreviation] Endokrynol Pol
  • [Language] pol
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Iodine Radioisotopes; 35MRW7B4AD / 3-Iodobenzylguanidine
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57. Kiss NB, Geli J, Lundberg F, Avci C, Velazquez-Fernandez D, Hashemi J, Weber G, Höög A, Ekström TJ, Bäckdahl M, Larsson C: Methylation of the p16INK4A promoter is associated with malignant behavior in abdominal extra-adrenal paragangliomas but not pheochromocytomas. Endocr Relat Cancer; 2008 Jun;15(2):609-21
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  • [Title] Methylation of the p16INK4A promoter is associated with malignant behavior in abdominal extra-adrenal paragangliomas but not pheochromocytomas.
  • Pheochromocytomas and abdominal extra-adrenal paragangliomas are related to endocrine tumors of the sympathetic nervous system.
  • Studies in animal models have shown that inactivation of the products of the cyclin dependent kinase inhibitor 2A (CDKN2A) gene locus, p16INK4A and p14ARF, promotes the development of pheochromocytoma, especially in malignant form.
  • The present study evaluated the involvement of CDKN2A in human pheochromocytomas and abdominal extra-adrenal paragangliomas from 55 patients.
  • For p16, western blot analysis and sequencing were also performed. succinate dehydrogenase complex subunit B (SDHB) sequencing analysis included extra-adrenal paragangliomas, all tumors classified as malignant, and cases diagnosed at 30 years or younger.
  • p16INK4A mRNA expression showed moderate suppression in malignant cases (P<0.05).
  • [MeSH-major] DNA Methylation. Gene Expression Regulation, Neoplastic. Genes, p16 / physiology. Paraganglioma, Extra-Adrenal / genetics. Peripheral Nervous System Neoplasms / genetics. Pheochromocytoma / genetics
  • [MeSH-minor] Breast Neoplasms. Cell Line, Tumor. Genetic Variation. Humans. Mutation, Missense. Osteosarcoma. Polymorphism, Single Nucleotide. Promoter Regions, Genetic / physiology. Reverse Transcriptase Polymerase Chain Reaction. Succinate Dehydrogenase / genetics. Suppression, Genetic / genetics. Sympathetic Nervous System / pathology. Tumor Suppressor Protein p14ARF / genetics

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  • (PMID = 18509008.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Tumor Suppressor Protein p14ARF; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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58. Kajor M, Ziaja J, Lange D, Król R, Ciupińska-Kajor M, Turska-d'Amico M, Maka B, Cierpka L: [Analysis of morphology of adrenal pheochromocytoma as regards their potential malignancy]. Endokrynol Pol; 2005 Nov-Dec;56(6):911-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Analysis of morphology of adrenal pheochromocytoma as regards their potential malignancy].
  • BACKGROUND: Adrenal pheochromocytoma are diagnosed the most often in patients with arterial hypertension or with thyroid medullar cancer and suspicion of MEN II syndromes.
  • The aim of the study is to analyse the morphology of pheochromocytomas on the basis of Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) in order to estimate their potential malignancy.
  • The diagnosis of pheochromocytoma was establish before surgery in 87.5%.
  • In 1 cases malignant form of pheochromocytoma was diagnosed on the basis of lymph nodes metastases.
  • Among 35 patients operated on more than 12 months ago 2 patients died: 1 patient with malignant pheochromocytoma (PASS=8 points) and 1 patient with MEN IIA syndrome (due to disseminated thyroid medullar cancer).
  • CONCLUSION: Analysis of pheochromocytoma in PASS is only of rough character and does not allow for clear-cut histological diagnosis of benign and malignant tumours.
  • The only unquestionable criterion of pheochromocytoma's malignancy remain metastases.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology. Pheochromocytoma / secondary
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Diagnosis, Differential. Female. Humans. Lymphatic Metastasis. Male. Middle Aged. Multiple Endocrine Neoplasia Type 2a / pathology. Neoplasm Staging. Thyroid Neoplasms / pathology

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  • (PMID = 16821210.001).
  • [ISSN] 0423-104X
  • [Journal-full-title] Endokrynologia Polska
  • [ISO-abbreviation] Endokrynol Pol
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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59. Guller U, Turek J, Eubanks S, Delong ER, Oertli D, Feldman JM: Detecting pheochromocytoma: defining the most sensitive test. Ann Surg; 2006 Jan;243(1):102-7
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  • [Title] Detecting pheochromocytoma: defining the most sensitive test.
  • OBJECTIVE: To define the most sensitive biochemical test to establish the diagnosis of pheochromocytoma and also to assess the potential role of iodine 131-labeled metaiodobenzylguanidine scintigraphy (I-MIBG) in the diagnosis of this tumor.
  • SUMMARY BACKGROUND DATA: Pheochromocytoma is a rare, catecholamine-producing tumor with preferential localization in the adrenal gland.
  • Despite its importance, the most sensitive test to establish the diagnosis remains to be defined.
  • METHODS: Prospective data collection was done on patients with pheochromocytoma treated at the Duke University Medical Center and the Durham Veterans Affairs Medical Center, Durham, NC.
  • Spells (defined as profuse sweating, tachycardia, and headache) and hypertension at diagnosis were present in 51.4% and 66.6%, respectively.
  • Bilateral disease was found in 12.5%, malignant pheochromocytoma in 29.6%, and hereditary forms in 23.0%.
  • CONCLUSIONS: The tests of choice to establish the diagnosis of pheochromocytoma are urinary normetanephrine and platelet norepinephrine.
  • We thus advocate performing an MIBG scan if the diagnosis of pheochromocytoma is clinically suspected and catecholamine measurements are within the normal range.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Pheochromocytoma / diagnosis. Radionuclide Imaging / methods

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  • (PMID = 16371743.001).
  • [ISSN] 0003-4932
  • [Journal-full-title] Annals of surgery
  • [ISO-abbreviation] Ann. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine; X4W3ENH1CV / Norepinephrine
  • [Other-IDs] NLM/ PMC1449983
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60. Krzysztof K, Wiktor B, Tadeusz Ł, Waldemar B, Magdalena K, Janusz D: Neuroendocrine tumours--analysis of own material--a nine--year retrospective study. Hepatogastroenterology; 2010 Mar-Apr;57(98):236-41

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The aim of this study was to present the author's observations of the histological tumor types, occurrence and its surgical treatment.
  • Ultrasonography, scintigraphy, computed tomography or magnetic resonance imaging of abdominal cavity, pelvis, thorax or neck--depend on the tumor localization--were done in every individual.
  • In the study we have also 11 cases of medullary thyroid carcinomas.
  • In adrenal glands we observed 10 benign and 1 malignant pheochromocytoma (one bilateral female case with Multiple Endocrine Neoplasia type 2A).
  • The clinical manifestations of some neuroendocrine tumours are not specific, so it causes a lot of difficulties in early diagnosis and treatment.

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  • (PMID = 20583420.001).
  • [ISSN] 0172-6390
  • [Journal-full-title] Hepato-gastroenterology
  • [ISO-abbreviation] Hepatogastroenterology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
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61. Matsubayashi H, Uesaka K, Kanemoto H, Sugiura T, Mizuno T, Sasaki K, Ono H, Hruban R: Multiple endocrine neoplasms and serous cysts of the pancreas in a patient with von Hippel-Lindau disease. J Gastrointest Cancer; 2010 Sep;41(3):197-202
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  • CASE REPORT: A 40-year-old female with a past history of bilateral adrenal pheochromocytomas, whose brother had a history of a malignant pheochromocytoma, was referred to our institution with symptoms of adrenal crisis including general fatigue and insomnia in spite of increasing predonisolone therapy.
  • [MeSH-minor] Adrenal Gland Neoplasms / surgery. Adrenalectomy. Adult. Female. Humans. Pheochromocytoma / surgery

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  • (PMID = 20140653.001).
  • [ISSN] 1941-6636
  • [Journal-full-title] Journal of gastrointestinal cancer
  • [ISO-abbreviation] J Gastrointest Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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62. Babińska A, Sworczak K, Siekierska-Hellmann M, Lewczuk A, Błaut K, Obołoiczyk L, Wiśniewski P, Zielonko J, Kaska L, Lachiński A: [Incidentally discovered adrenal masses in the Department of Internal Medicine, Endocrinology and Hemostatic Disorders, Medical University of Gdansk]. Wiad Lek; 2006;59(11-12):744-50
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  • [Title] [Incidentally discovered adrenal masses in the Department of Internal Medicine, Endocrinology and Hemostatic Disorders, Medical University of Gdansk].
  • Clinically silent adrenal masses (incidentaloma) are incidentally discovered lesions when noninvasive imaging methods (ultrasonography--USG, computer tomography--CT, magnetic resonance imaging--MRI) are performed for the reason other than known or suspected adrenal disease.
  • Most of studies report the prevalence of adrenal incidentaloma range between 1 and 10% in radiological series.
  • MATERIAL AND METHODS: Between 1993 and 03.2004 we observed 198 patients with incidentalomas of adrenal glands (144 females--72.7% and 54 males--27.3%).
  • Adrenocortical adenoma was diagnosed in 54.9%, adrenal hyperplasia in 8.5%, adrenal carcinoma in 6.7%, pheochromocytoma in 12.9% (in 3.1% of all cases malignant pheochromocytoma was diagnosed), in 4.9% others malignant tumors (primary or metastatic), in 4.9% adrenal cysts and in 7.1% other rare adrenal pathologies were found.
  • In tumors with diameter over 6 cm malignant cases were found in 70.8%.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenocortical Carcinoma / diagnosis. Incidental Findings. Pheochromocytoma / diagnosis
  • [MeSH-minor] Adrenal Gland Diseases / diagnosis. Adrenal Gland Diseases / epidemiology. Adrenal Gland Diseases / pathology. Adrenal Gland Diseases / surgery. Adrenalectomy. Adult. Aged. Aged, 80 and over. Biopsy, Needle. Cysts / diagnosis. Cysts / epidemiology. Cysts / pathology. Cysts / surgery. Female. Hospitals, University / statistics & numerical data. Humans. Laparoscopy / methods. Magnetic Resonance Imaging. Male. Middle Aged. Poland / epidemiology. Prevalence. Radiography, Abdominal. Retrospective Studies. Risk Factors. Sex Distribution. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 17427485.001).
  • [ISSN] 0043-5147
  • [Journal-full-title] Wiadomości lekarskie (Warsaw, Poland : 1960)
  • [ISO-abbreviation] Wiad. Lek.
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
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63. Grossrubatscher E, Dalino P, Vignati F, Gambacorta M, Pugliese R, Boniardi M, Rossetti O, Marocchi A, Bertuzzi M, Loli P: The role of chromogranin A in the management of patients with phaeochromocytoma. Clin Endocrinol (Oxf); 2006 Sep;65(3):287-93
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  • [Title] The role of chromogranin A in the management of patients with phaeochromocytoma.
  • Supranormal CgA concentrations have been recorded in patients with tumours of neuroectodermal origin such as phaeochromocytoma and paraganglioma.
  • DESIGN: The present study was performed to assess the role of CgA determination in the management of patients with phaeochromocytoma, in comparison with urinary catecholamines and their metabolites.
  • PATIENTS: The patients studied included 22 cases with phaeochromocytoma at initial presentation or at relapse some years after surgical cure or during follow-up of a malignant phaeochromocytoma.
  • Seventeen patients were evaluated before and after surgical removal of phaeochromocytoma.
  • To assess the specificity of the hormonal parameters, 20 subjects were enrolled as controls; they were from a group of patients referred to our observation for possible phaeochromocytoma and who were subsequently proven not to have the disease.
  • The combination of urinary metanephrine and normetanephrine had a sensitivity of 100% in identifying a phaeochromocytoma.
  • Combining the results of CgA and urinary catecholamines (epinephrine and norepinephrine), the sensitivity for diagnosis of phaeochromocytoma was 100%.
  • Urinary catecholamines, metabolites (metanephrine and normetanephrine) and CgA levels in patients with malignant phaeochromocytoma did not differ significantly from those of patients with benign lesions.
  • CONCLUSIONS: Our results indicate that CgA is a good marker of phaeochromocytoma; measurement of CgA could have a role in the follow-up of patients operated on for phaeochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / blood. Biomarkers, Tumor / blood. Chromogranin A / blood. Pheochromocytoma / blood

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  • (PMID = 16918946.001).
  • [ISSN] 0300-0664
  • [Journal-full-title] Clinical endocrinology
  • [ISO-abbreviation] Clin. Endocrinol. (Oxf)
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Catecholamines; 0 / Chromogranin A; 0J45DE6B88 / Normetanephrine; 5001-33-2 / Metanephrine
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64. Castellani MR, Seghezzi S, Chiesa C, Aliberti GL, Maccauro M, Seregni E, Orunesu E, Luksch R, Bombardieri E: (131)I-MIBG treatment of pheochromocytoma: low versus intermediate activity regimens of therapy. Q J Nucl Med Mol Imaging; 2010 Feb;54(1):100-13
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  • [Title] (131)I-MIBG treatment of pheochromocytoma: low versus intermediate activity regimens of therapy.
  • AIM: Since the second half of the 1980s, (131)I-MIBG has been widely used for treatment of patients with malignant pheochromocytoma.
  • In 1991, at the International Meeting in Rome, it was agreed that (131)I-MIBG therapy induces significant tumor responses in about 30-50% of cases, long-term stabilization of disease in several cases and significant reduction of cathecolamine-related symptoms in almost all patients.
  • Nevertheless, more than 20 years later, its therapeutic use in malignant phaeochromocytoma has not yet been standardized.
  • Consequently, the increase of (131)I-MIBG activity, without reaching myeloablative levels, can be recommended for standard treatment of pheochromocytoma and paraganglioma patients.
  • [MeSH-major] 3-Iodobenzylguanidine / therapeutic use. Adrenal Gland Neoplasms / radiotherapy. Pheochromocytoma / radiotherapy. Radiation Dosage

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  • (PMID = 20168292.001).
  • [ISSN] 1824-4785
  • [Journal-full-title] The quarterly journal of nuclear medicine and molecular imaging : official publication of the Italian Association of Nuclear Medicine (AIMN) [and] the International Association of Radiopharmacology (IAR), [and] Section of the Society of Radiopharmaceutical Chemistry and Biology
  • [ISO-abbreviation] Q J Nucl Med Mol Imaging
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Iodine Radioisotopes; 35MRW7B4AD / 3-Iodobenzylguanidine
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65. Gockel I, Heintz A, Domeyer M, Trinh TT, Dünschede F, Junginger T: [Indications for conventional adrenalectomy]. Zentralbl Chir; 2008 Jun;133(3):255-9
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  • RESULTS: Indications for conventional adrenalectomy were - as compared with the minimally invasive procedure - significantly more frequent adrenocortical carcinomas (ACC), especially in the context of multivisceral resections, as well as adrenal metastases (synchronous and metachronous).
  • In contrast, adrenal Cushing's disease (including 19 patients with bilateral tumours), pheochromocytoma, incidentaloma and Conn's syndrome constituted a more frequent indication for minimally invasive adrenalectomy.
  • Conventionally operated adrenal pathologies with on average 6.0 (range: 1.2-19.0) cm diameter were significantly larger than the endoscopically removed tumours with on average 3.3 (range: 0.2-9.2) cm diameter (p < 0.0001).
  • The side localisation and the frequency of bilateral adrenal tumours did not differ significantly in the two groups.
  • CONCLUSION: Since the establishment of the minimally invasive technique in 1994, conventional adrenalectomy has been selected for 26 % of all resected adrenal pathologies at our clinic and, therefore, still plays an important role even in the era of laparoscopic surgery.
  • The benefit of the laparoscopic procedure in the case of malignant pheochromocytoma, adrenocortical carcinoma, and isolated adrenal metastases at a locally confined stage is still unclear and requires prospective, randomised studies.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy / utilization. Minimally Invasive Surgical Procedures / utilization
  • [MeSH-minor] Adrenal Cortex Neoplasms / pathology. Adrenal Cortex Neoplasms / surgery. Adrenal Glands / pathology. Adult. Aged. Aged, 80 and over. Cushing Syndrome / pathology. Cushing Syndrome / surgery. Female. Humans. Hyperaldosteronism / pathology. Hyperaldosteronism / surgery. Male. Mathematical Computing. Middle Aged. Pheochromocytoma / pathology. Pheochromocytoma / surgery. Utilization Review / statistics & numerical data

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  • (PMID = 18563692.001).
  • [ISSN] 0044-409X
  • [Journal-full-title] Zentralblatt für Chirurgie
  • [ISO-abbreviation] Zentralbl Chir
  • [Language] ger
  • [Publication-type] Comparative Study; English Abstract; Journal Article
  • [Publication-country] Germany
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66. Bissada MA, Safwat AS, Seyam RM, Al Sobhi S, Hanash KA, Bissada NK: Familial pheochromocytoma. Urol Oncol; 2008 Jul-Aug;26(4):361-3
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  • [Title] Familial pheochromocytoma.
  • OBJECTIVES: To identify disease characteristics, operative outcome, and prognosis in patients with familial pheochromocytoma.
  • METHODS: Records of 29 patients with familial pheochromocytoma were retrospectively analyzed.
  • Of the 29 patients, 26 had adrenal tumors, 2 had extra-adrenal tumors, and 1 had a combined adrenal and extra-adrenal tumor.
  • Two patients had malignant pheochromocytoma.
  • The two patients with malignant pheochromocytoma had MEN II.
  • All patients were managed by either open or laparoscopic adrenalectomy or excision of extra-adrenal tumors.
  • Presence of other manifestations of familial pheochromocytoma significantly influenced the clinical course and prognosis.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Pheochromocytoma / genetics


67. Zelinka T, Timmers HJ, Kozupa A, Chen CC, Carrasquillo JA, Reynolds JC, Ling A, Eisenhofer G, Lazúrová I, Adams KT, Whatley MA, Widimsky J Jr, Pacak K: Role of positron emission tomography and bone scintigraphy in the evaluation of bone involvement in metastatic pheochromocytoma and paraganglioma: specific implications for succinate dehydrogenase enzyme subunit B gene mutations. Endocr Relat Cancer; 2008 Mar;15(1):311-23
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  • [Title] Role of positron emission tomography and bone scintigraphy in the evaluation of bone involvement in metastatic pheochromocytoma and paraganglioma: specific implications for succinate dehydrogenase enzyme subunit B gene mutations.
  • We performed a retrospective analysis of 71 subjects with metastatic pheochromocytoma and paraganglioma (30 subjects with mutation of succinate dehydrogenase enzyme subunit B (SDHB) gene and 41 subjects without SDHB mutation).
  • In conclusion, bone scintigraphy should be used in the staging of patients with malignant pheochromocytoma and paraganglioma, particularly in patients with SDHB mutations.
  • [MeSH-major] Adrenal Gland Neoplasms / radionuclide imaging. Bone Neoplasms / radionuclide imaging. Iron-Sulfur Proteins / genetics. Mutation / genetics. Paraganglioma / radionuclide imaging. Pheochromocytoma / radionuclide imaging. Positron-Emission Tomography. Radionuclide Imaging. Succinate Dehydrogenase / genetics

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  • (PMID = 18310297.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Iodine Radioisotopes; 0 / Iron-Sulfur Proteins; 0Z5B2CJX4D / Fluorodeoxyglucose F18; 35MRW7B4AD / 3-Iodobenzylguanidine; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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68. Birrenbach T, Stanga Z, Cottagnoud P, Stucki A: Unexpected metastatic pheochromocytoma - an unusual presentation. Eur J Intern Med; 2008 Jan;19(1):60-2
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  • [Title] Unexpected metastatic pheochromocytoma - an unusual presentation.
  • The classic triad of pheochromocytoma consists of episodic headache, sweating, and tachycardia.
  • We recently observed a noteworthy case of malignant pheochromocytoma where there was a lack of specific symptoms despite an advanced tumor stage.
  • Reliable diagnosis of malignancy depends upon evidence of local invasion, distant metastases, or recurrence.
  • As in our case, new scintigraphic methods, such as 111-In-pentetreotide scintigraphy (Octreoscan), may occasionally reveal 123-I-metaiodobenzylguanidine-negative distant metastases and help to establish an early diagnosis of malignancy.
  • Tumor size, and perhaps even biochemical profile, may be factors increasing the likelihood of a malignant process and may contribute to early identification of patients at risk.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Head and Neck Neoplasms / radionuclide imaging. Head and Neck Neoplasms / secondary. Pheochromocytoma / diagnosis. Pheochromocytoma / secondary


69. Tömböl Z, Eder K, Kovács A, Szabó PM, Kulka J, Likó I, Zalatnai A, Rácz G, Tóth M, Patócs A, Falus A, Rácz K, Igaz P: MicroRNA expression profiling in benign (sporadic and hereditary) and recurring adrenal pheochromocytomas. Mod Pathol; 2010 Dec;23(12):1583-95
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  • [Title] MicroRNA expression profiling in benign (sporadic and hereditary) and recurring adrenal pheochromocytomas.
  • Furthermore, microRNA expression profiles of a malignant pheochromocytoma and a pair of primary and recurrent tumors were studied by TaqMan Human MicroRNA Cards.
  • Pathway analysis revealed the possible involvement of Notch- and G-protein-coupled receptor signaling in tumor recurrence.
  • MicroRNA expression profiles in the primary recurrent and recurring malignant comparisons have been similar.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Gene Expression Profiling. MicroRNAs / genetics. Pheochromocytoma / genetics

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  • (PMID = 20818339.001).
  • [ISSN] 1530-0285
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / MicroRNAs
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70. Fujiwara M, Imachi H, Murao K, Muraoka T, Ohyama T, Miyai Y, Kushida Y, Haba R, Kakehi Y, Ishida T: Improvement in renal dysfunction and symptoms after laparoscopic adrenalectomy in a patient with pheochromocytoma complicated by renal dysfunction. Endocrine; 2009 Feb;35(1):57-62
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  • [Title] Improvement in renal dysfunction and symptoms after laparoscopic adrenalectomy in a patient with pheochromocytoma complicated by renal dysfunction.
  • A 70-year-old patient who was undergoing treatment for diabetes mellitus and chronic hepatitis was admitted to our hospital for evaluation of a tumor in the left adrenal gland (50 x 45 mm) and renal failure.
  • On the basis of the patient's increased serum concentrations of catecholamines and other metabolites and the results of positron emission tomography (PET), the patient was diagnosed with a pheochromocytoma; iodinated metaiodobenzylguanidine ([(131)I]MIBG) scintigraphy was insufficient to establish this diagnosis.
  • Subsequently, he underwent surgery for tumor resection.
  • Histological examination suggested the tumor to be a malignant pheochromocytoma.
  • Our patient exhibited a rare condition of pheochromocytoma complicated by renal failure, which was successfully treated with laparoscopic surgery.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Laparoscopy. Pheochromocytoma / surgery. Renal Insufficiency / surgery

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  • (PMID = 18989793.001).
  • [ISSN] 1355-008X
  • [Journal-full-title] Endocrine
  • [ISO-abbreviation] Endocrine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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71. Ohta S, Lai EW, Taniguchi S, Tischler AS, Alesci S, Pacak K: Animal models of pheochromocytoma including NIH initial experience. Ann N Y Acad Sci; 2006 Aug;1073:300-5
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  • [Title] Animal models of pheochromocytoma including NIH initial experience.
  • Mouse models have been used to study the mechanisms underlying the carcinogenesis of a wide variety of human cancer.
  • Some of these models resemble hereditary syndrome-related pheochromocytoma in humans and some may serve as a new starting point for human pheochromocytoma research.
  • Recently, we generated a model of catecholamine-producing metastatic pheochromocytoma in athymic nude mice using tail-vein injection of mouse pheochromocytoma cells (MPCs).
  • This and alternative animal models of metastatic pheochromocytoma are promising avenues in preclinical studies to evaluate new therapeutic approaches for malignant pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Disease Models, Animal. Pheochromocytoma / pathology

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  • (PMID = 17102099.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Grant] United States / NINDS NIH HHS / NS / P01 NS 37685; United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural
  • [Publication-country] United States
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72. Nagashima A, Ikemoto I, Furuta N, Egawa S: [A case of pheochromocytoma associated with incidental multiple renal cell carcinoma originating from acquired cystic disease of the kidney in hemodialysis]. Hinyokika Kiyo; 2006 Jul;52(7):557-60
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  • [Title] [A case of pheochromocytoma associated with incidental multiple renal cell carcinoma originating from acquired cystic disease of the kidney in hemodialysis].
  • Computed tomography and magnetic resonance imaging demonstrated the presence of right adrenal tumor(1.5 x 1.5 cm) and bilateral multiple renal cysts.
  • Right adrenal pheochromocytoma was confirmed by elevated serum level of catecholamines and diagnosed by 131I-MIBG scintigram.
  • Histological examination confirmed malignant pheochromocytoma and small multiple renal cell carcinomas.
  • To our knowledge, this is the fifth case of adrenal pheochromocytoma and renal cell carcinoma associated ACDK in a hemodialysis patient in the Japanese literature.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Carcinoma, Renal Cell / diagnosis. Kidney Diseases, Cystic. Kidney Neoplasms / diagnosis. Pheochromocytoma / complications. Renal Dialysis


73. Lombardi CP, Raffaelli M, De Crea C, Bellantone R: Role of laparoscopy in the management of adrenal malignancies. J Surg Oncol; 2006 Aug 1;94(2):128-31
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  • [Title] Role of laparoscopy in the management of adrenal malignancies.
  • BACKGROUND AND OBJECTIVES: The role of laparoscopic approach for the treatment of malignant adrenal diseases is still controversial.
  • The aim of this study was to verify the results of laparoscopic adrenalectomy (LA) in the management of adrenal malignancies.
  • METHODS: The medical records of all the patients who underwent laparoscopic procedures for adrenal diseases and in whom malignancy was demonstrated at final histology were reviewed.
  • RESULTS: Nine patients were included (three malignant pheochromocytomas, four adrenocortical carcinomas, and two adrenal metastases).
  • One patient operated on for a malignant pheochromocytoma developed multiple intra-abdominal recurrences.
  • CONCLUSIONS: The results of this study demonstrate that LA can have a role also in case of adrenal malignancies.
  • A preliminary laparoscopic exploration can be planned in case of suspected malignant lesions to confirm the diagnosis and to evaluate their operability.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Adrenocortical Carcinoma / surgery. Laparoscopy. Pheochromocytoma / surgery
  • [MeSH-minor] Abdominal Neoplasms / secondary. Adrenal Glands / pathology. Adult. Aged. Biopsy. Female. Humans. Male. Middle Aged. Pelvic Neoplasms / secondary. Registries

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  • [Copyright] Copyright (c) 2006 Wiley-Liss, Inc.
  • (PMID = 16847903.001).
  • [ISSN] 0022-4790
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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74. Gross DJ, Munter G, Bitan M, Siegal T, Gabizon A, Weitzen R, Merimsky O, Ackerstein A, Salmon A, Sella A, Slavin S, Israel Glivec in Solid Tumors Study Group: The role of imatinib mesylate (Glivec) for treatment of patients with malignant endocrine tumors positive for c-kit or PDGF-R. Endocr Relat Cancer; 2006 Jun;13(2):535-40
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  • [Title] The role of imatinib mesylate (Glivec) for treatment of patients with malignant endocrine tumors positive for c-kit or PDGF-R.
  • Since both PDGF-R and c-kit both having a putative role in tumorigenesis, we investigated the efficacy and safety of the use of IM in patients with endocrine tumors unresponsive to conventional therapies that expressed c-kit and/or PDGF-R (within the framework of a comprehensive phase II multi-center study of IM in patients with solid tumors).
  • Between September 2002 and July 2003, 15 adult patients with disseminated endocrine tumors were recruited as follows: medullary thyroid carcinoma (MTC, n = 6); adrenocortical carcinoma (ACC, n = 4); malignant pheochromocytoma (pheo, n = 2); carcinoid (non-secreting, n = 2), neuroendocrine tumor (NET, n = 1).
  • IM does not appear to be useful for treatment of malignant endocrine tumors, also causing significant toxicity in this patient population.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Endocrine Gland Neoplasms / drug therapy. Piperazines / therapeutic use. Protein Kinase Inhibitors / therapeutic use. Pyrimidines / therapeutic use

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  • (PMID = 16728580.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Protein Kinase Inhibitors; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; EC 2.7.10.1 / Receptors, Platelet-Derived Growth Factor
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75. Patel M, Santos P, Jong I, Nandurkar D, McKay J: Malignant pheochromocytoma metastasis to the breast shown on I-123 MIBG scan. Clin Nucl Med; 2010 Oct;35(10):816-7
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  • [Title] Malignant pheochromocytoma metastasis to the breast shown on I-123 MIBG scan.
  • A 36-year-old woman was referred to our department for I-123 metaiodobenzylguanidine (MIBG)-SPECT/CT scan 3 years after surgical excision of pheochromocytoma.
  • Fine-needle aspiration revealed cytologic changes consistent with pheochromocytoma.
  • There has only been 1 previous reported case of pheochromocytoma metastasizing to the breast.
  • [MeSH-major] 3-Iodobenzylguanidine. Adrenal Gland Neoplasms / pathology. Breast Neoplasms / diagnosis. Breast Neoplasms / secondary. Pheochromocytoma / pathology

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  • (PMID = 20838297.001).
  • [ISSN] 1536-0229
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 35MRW7B4AD / 3-Iodobenzylguanidine
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76. Nagata M, Takayama T, Nishijima N, Mugiya S, Ushiyama T, Ozono S: [Clinical course of patient with malignant pheochromocytoma who was treated with CVD chemotherapy and alpha-methyl-p-tyrosine]. Nihon Hinyokika Gakkai Zasshi; 2010 May;101(4):615-8
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  • [Title] [Clinical course of patient with malignant pheochromocytoma who was treated with CVD chemotherapy and alpha-methyl-p-tyrosine].
  • The CT scan and bone scintigraphy showed right adrenal tumor, along with liver metastasis, lymph nodes swelling around aorta and multiple bone metastases.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Enzyme Inhibitors / administration & dosage. Pheochromocytoma / drug therapy. alpha-Methyltyrosine / administration & dosage

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  • (PMID = 20535990.001).
  • [ISSN] 0021-5287
  • [Journal-full-title] Nihon Hinyōkika Gakkai zasshi. The japanese journal of urology
  • [ISO-abbreviation] Nippon Hinyokika Gakkai Zasshi
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Enzyme Inhibitors; 5J49Q6B70F / Vincristine; 658-48-0 / alpha-Methyltyrosine; 7GR28W0FJI / Dacarbazine; 8N3DW7272P / Cyclophosphamide; CVD protocol
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77. Lai EW, Joshi BH, Martiniova L, Dogra R, Fujisawa T, Leland P, de Krijger RR, Lubensky IA, Elkahloun AG, Morris JC, Puri RK, Pacak K: Overexpression of interleukin-13 receptor-alpha2 in neuroendocrine malignant pheochromocytoma: a novel target for receptor directed anti-cancer therapy. J Clin Endocrinol Metab; 2009 Aug;94(8):2952-7
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  • [Title] Overexpression of interleukin-13 receptor-alpha2 in neuroendocrine malignant pheochromocytoma: a novel target for receptor directed anti-cancer therapy.
  • CONTEXT: Pheochromocytomas and paragangliomas are rare catecholamine-secreting neuroendocrine tumors arising from the adrenal medulla and sympathetic tissues.
  • When complete surgical resection is not an option, the treatment of pheochromocytoma is limited.
  • OBJECTIVE: The objective of the study was to identify and characterize overexpression of IL-13 receptor-alpha2 (IL-13Ralpha2) gene expression in human and murine tumors and verify xenograft mouse pheochromocytoma cell (MPC)-derived tumor's response to a selective cytotoxin.
  • DESIGN/SETTING/PATIENTS: Expression of IL-13Ralpha2 was evaluated in a panel of 25 human pheochromocytoma clinical samples by RT-PCR and eight MPC tumors by indirect immunofluorescence assay and RT-PCR.
  • INTERVENTION: The function of IL-13Ralpha2 in these tumor cells was examined by evaluating tumor sensitivity to a recombinant IL-13-Pseudomonas exotoxin (IL-13PE).
  • MAIN OUTCOME MEASURES: IC(50) and tumor size were measured.
  • Our results showed a statistically significant decrease in tumor size as early as 3 d after initial treatment and further suppressed growth of MPC tumors.
  • CONCLUSIONS: Human and murine neuroendocrine pheochromocytoma overexpress the IL-13Ralpha2 chain, and an IL-13PE-based receptor-directed anticancer approach may prove useful in treatment for metastatic pheochromocytoma patients.

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  • [Cites] Mol Med. 2002 Aug;8(8):487-94 [12435859.001]
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  • (PMID = 19491224.001).
  • [ISSN] 1945-7197
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] ENG
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Bacterial Proteins; 0 / Immunotoxins; 0 / Interleukin-13 Receptor alpha2 Subunit; 0 / pseudomonas exoprotein A protein, Pseudomonas aeruginosa
  • [Other-IDs] NLM/ PMC2730867
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78. Titov VN: [Phylogenetic, pathogenetic fundamentals and a role of clinical biochemistry in classification of arterial hypertension]. Klin Lab Diagn; 2009 Oct;(10):3-13
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  • The first option can be related to AH occurring in pheochromoblastoma, glucocorticoid hyperproduction and psychoemotional stress.
  • Elevation of BP in erythemia, under the effect of leukocyte colony-stimulating factor and after injection of a recombinant erythropoietin can also be regarded within the frames of the third option.

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  • (PMID = 20050074.001).
  • [ISSN] 0869-2084
  • [Journal-full-title] Klinicheskaia laboratornaia diagnostika
  • [ISO-abbreviation] Klin. Lab. Diagn.
  • [Language] rus
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
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79. National Toxicology Program: NTP toxicology and carcinogenesis studies of decalin (CAS No. 91-17-8) in F344/N rats and B6C3F(1) mice and a toxicology study of decalin in male NBR rats (inhalation studies). Natl Toxicol Program Tech Rep Ser; 2005 Jan;(513):1-316
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  • Incidences of renal tubule adenoma and adenoma or carcinoma (combined) and of benign or malignant pheochromocytoma (combined) of the adrenal medulla in 100 and 400 ppm males were significantly increased.
  • There was a significant association between nephropathy severity and adrenal pheochromocytoma incidence.
  • The increased incidences of benign or malignant pheochromocytoma (combined) of the adrenal medulla in male rats were also considered to be exposure related.

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  • (PMID = 15891779.001).
  • [ISSN] 0888-8051
  • [Journal-full-title] National Toxicology Program technical report series
  • [ISO-abbreviation] Natl Toxicol Program Tech Rep Ser
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Mutagens; 0 / Naphthalenes; 88451Q4XYF / decalin
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80. Ilias I, Pacak K: Diagnosis, localization and treatment of pheochromocytoma in MEN 2 syndrome. Endocr Regul; 2009 Apr;43(2):89-93
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  • [Title] Diagnosis, localization and treatment of pheochromocytoma in MEN 2 syndrome.
  • Pheochromocytomas/paragangliomas (PHEOs/PGLs) in patients with MEN 2 are usually found in the adrenals after the manifestation of medullary thyroid cancer.
  • Plasma concentrations of free metanephrines (or free metanephrines in urine) are best used for the biochemical diagnosis of PHEO/PGL.
  • If PHEO/PGL-specific modalities turn out to be negative functional imaging should follow with nonspecific modalities (particularly if recurrent, metastatic or malignant disease is suspected).
  • Overall half of the patients with malignant PHEOs remain alive for 5 years.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Multiple Endocrine Neoplasia Type 2a / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Dopamine / analogs & derivatives. Fluorine Radioisotopes. Humans. Magnetic Resonance Imaging. Paraganglioma / diagnosis. Paraganglioma / pathology. Paraganglioma / therapy. Positron-Emission Tomography. Prognosis. Radiopharmaceuticals. Tomography, X-Ray Computed

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  • (PMID = 19856714.001).
  • [ISSN] 1210-0668
  • [Journal-full-title] Endocrine regulations
  • [ISO-abbreviation] Endocr Regul
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Slovakia
  • [Chemical-registry-number] 0 / Fluorine Radioisotopes; 0 / Radiopharmaceuticals; 59043-70-8 / 6-fluorodopamine; VTD58H1Z2X / Dopamine
  • [Number-of-references] 30
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81. Arcos CT, Luque VR, Luque JA, García PM, Jiménez AB, Muñoz MM: Malignant giant pheochromocytoma: a case report and review of the literature. Can Urol Assoc J; 2009 Dec;3(6):E89-91

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  • [Title] Malignant giant pheochromocytoma: a case report and review of the literature.
  • Malignant pheochromocytoma is a rare disease and surgical resection is the only curative treatment.
  • We report a case of a patient with a giant retroperitoneal tumour, the second largest to be published, which was diagnosed as a malignant pheochromocytoma; it was treated with surgery.
  • The literature is reviewed to evaluate tumour features and criteria to distinguish between benign and malignant pheochromocytomas.

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  • (PMID = 20019963.001).
  • [ISSN] 1920-1214
  • [Journal-full-title] Canadian Urological Association journal = Journal de l'Association des urologues du Canada
  • [ISO-abbreviation] Can Urol Assoc J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Canada
  • [Other-IDs] NLM/ PMC2792417
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82. Bissada NK, Safwat AS, Seyam RM, Al Sobhi S, Hanash KA, Jackson RJ, Sakati N, Bissada MA: Pheochromocytoma in children and adolescents: a clinical spectrum. J Pediatr Surg; 2008 Mar;43(3):540-3
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  • [Title] Pheochromocytoma in children and adolescents: a clinical spectrum.
  • PURPOSE: The purpose of the study was to identify the spectrum of disease characteristics of pheochromocytoma in children.
  • METHODS: Records of 21 consecutive children diagnosed with pheochromocytoma were reviewed.
  • Patients included 17 with adrenal and 4 with extra-adrenal tumors including 1 in the urinary bladder.
  • Seventeen had sporadic and 4 had familial pheochromocytoma.
  • Associated manifestations were the predominant features in 1 of the 4 patients with familial pheochromocytoma.
  • The patient with bladder pheochromocytoma presented with gross hematuria.
  • Two patients (1 sporadic and 1 familial) had malignant pheochromocytoma.
  • One patient with benign pheochromocytoma had multiple recurrences in chromaffin-containing sites.
  • One patient with malignant pheochromocytoma died of the disease, and 1 with recurrent pheochromocytoma had neurologic consequences.
  • CONCLUSIONS: Pheochromocytoma in children has unique characteristics.
  • To our knowledge, this series is one of the largest reports of adrenal pheochromocytoma in children.
  • It also reflects the spectrum of pheochromocytoma in this age group.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery. Pheochromocytoma / diagnosis. Pheochromocytoma / surgery
  • [MeSH-minor] Adolescent. Adrenalectomy / methods. Age Factors. Biopsy, Needle. Child. Child, Preschool. Cohort Studies. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Immunohistochemistry. Male. Neoplasm Staging. Neurosurgery. Phenoxybenzamine / therapeutic use. Retrospective Studies. Risk Assessment. Sex Factors. Sickness Impact Profile. Survival Rate. Treatment Outcome


83. Huang KH, Chung SD, Chen SC, Chueh SC, Pu YS, Lai MK, Lin WC: Clinical and pathological data of 10 malignant pheochromocytomas: long-term follow up in a single institute. Int J Urol; 2007 Mar;14(3):181-5
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  • [Title] Clinical and pathological data of 10 malignant pheochromocytomas: long-term follow up in a single institute.
  • BACKGROUND: Malignant pheochromocytomas are rare.
  • We report the clinical and histological data of long-term follow up in 10 patients with malignant pheochromocytoma.
  • METHODS: The clinical charts of 10 patients with malignant pheochromocytoma from a single institute were reviewed.
  • The diagnosis of pheochromocytoma was confirmed at surgery.
  • All patients had metastases in sites where chromaffin tissue was normally absent.
  • Extra-adrenal tumors occurred in four patients including paraganglioma tumors in three cases and bladder tumor in one case.
  • Histological characteristics were not helpful for diagnosis of malignancy and for prediction of prognosis.
  • All patients achieved long-term survival except for two who died of metastasis 1.5 years and 2 years after diagnosis.
  • Close long-term follow up for more than 10 years after surgery is necessary in patients with pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / therapy. Adrenalectomy. Antineoplastic Agents / therapeutic use. Pheochromocytoma
  • [MeSH-minor] Adolescent. Adult. Aged. Bone Neoplasms / diagnosis. Bone Neoplasms / secondary. Female. Follow-Up Studies. Humans. Liver Neoplasms / diagnosis. Liver Neoplasms / secondary. Lung Neoplasms / diagnosis. Lung Neoplasms / secondary. Lymphatic Metastasis. Male. Middle Aged. Positron-Emission Tomography. Prognosis. Radiotherapy, Adjuvant. Retrospective Studies. Survival Rate. Time Factors. Tomography, X-Ray Computed. Urinary Bladder Neoplasms / diagnosis. Urinary Bladder Neoplasms / secondary

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  • (PMID = 17430251.001).
  • [ISSN] 0919-8172
  • [Journal-full-title] International journal of urology : official journal of the Japanese Urological Association
  • [ISO-abbreviation] Int. J. Urol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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84. Mackenzie IS, Gurnell M, Balan KK, Simpson H, Chatterjee K, Brown MJ: The use of 18-fluoro-dihydroxyphenylalanine and 18-fluorodeoxyglucose positron emission tomography scanning in the assessment of metaiodobenzylguanidine-negative phaeochromocytoma. Eur J Endocrinol; 2007 Oct;157(4):533-7
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  • [Title] The use of 18-fluoro-dihydroxyphenylalanine and 18-fluorodeoxyglucose positron emission tomography scanning in the assessment of metaiodobenzylguanidine-negative phaeochromocytoma.
  • 123I-metaiodobenzylguanidine (123I-MIBG) scintigraphy scanning is commonly used in the imaging of phaeochromocytoma (and paraganglioma) to confirm the site of disease and whether any spread has occurred.
  • However, 123I-MIBG imaging is negative in 15% of cases of benign phaeochromocytoma and around 50% of cases of malignant phaeochromocytoma.
  • In recent years, positron emission tomography (PET) scanning using various different radiotracers has been shown to be a good alternative or supplementary investigation in phaeochromocytoma.
  • We present the cases of four patients with symptoms and signs suggestive of phaeochromocytoma, but who had negative 123I-MIBG scans, and illustrate the usefulness of 18-fluoro-dihydroxyphenylalanine PET scanning in their assessment.
  • In one of the patients, we illustrate how fluorodeoxyglucose PETscanning can provide useful information about the extent of malignant disease.
  • These illustrative cases lend further support for the use of PET scanning in the assessment of phaeochromocytoma and suggest that it may have a particularly important role in the investigation of patients in whom 123I-MIBG scanning is negative.

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  • (PMID = 17893270.001).
  • [ISSN] 1479-683X
  • [Journal-full-title] European journal of endocrinology
  • [ISO-abbreviation] Eur. J. Endocrinol.
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Fluorine Radioisotopes; 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18; 2C598205QX / fluorodopa F 18; 35MRW7B4AD / 3-Iodobenzylguanidine; 63-84-3 / Dihydroxyphenylalanine
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85. Ye L, Santarpia L, Gagel RF: The evolving field of tyrosine kinase inhibitors in the treatment of endocrine tumors. Endocr Rev; 2010 Aug;31(4):578-99
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  • Significant reductions in tumor size have been described in medullary and papillary thyroid carcinoma, although no complete responses have been reported.
  • Case reports have described significant tumor volume reductions of malignant pheochromocytomas and paragangliomas.
  • In addition, these compounds showed an initial tumoricidal or apoptotic response followed by long-term static effects on tumor growth.
  • In this review, we will address the genetic alterations of TKRs, the rationale for utilizing TKIs for endocrine tumors, and current information on tumor and patient responses to specific TKIs.
  • [MeSH-major] Endocrine Gland Neoplasms / drug therapy. Protein Kinase Inhibitors / therapeutic use. Protein-Tyrosine Kinases / antagonists & inhibitors
  • [MeSH-minor] Adrenal Gland Neoplasms / drug therapy. Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / pathology. Carcinoid Tumor / drug therapy. Carcinoid Tumor / pathology. Carcinoma, Islet Cell / drug therapy. Carcinoma, Islet Cell / pathology. Humans. Pancreatic Neoplasms / drug therapy. Pancreatic Neoplasms / pathology. Paraganglioma / drug therapy. Paraganglioma / genetics. Paraganglioma / pathology. Pheochromocytoma / drug therapy. Pheochromocytoma / genetics. Pheochromocytoma / pathology. Phosphorylation / drug effects. Proto-Oncogene Proteins c-ret / physiology. Receptor Protein-Tyrosine Kinases / genetics. Receptor Protein-Tyrosine Kinases / metabolism. Thyroid Neoplasms / drug therapy. Thyroid Neoplasms / pathology

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  • (PMID = 20605972.001).
  • [ISSN] 1945-7189
  • [Journal-full-title] Endocrine reviews
  • [ISO-abbreviation] Endocr. Rev.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Protein Kinase Inhibitors; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases
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86. Habra MA, Núñez R, Chuang H, Ayala-Ramirez M, Rich T, Kyle K, Jimenez C: Fatal hypoglycemia in malignant pheochromocytoma: direct glucose consumption as suggested by (18)F-2-fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography imaging. Endocrine; 2010 Feb;37(1):209-12
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  • [Title] Fatal hypoglycemia in malignant pheochromocytoma: direct glucose consumption as suggested by (18)F-2-fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography imaging.
  • We present a patient with metastatic pheochromocytoma, who developed progressive and fatal hypoglycemia most likely secondary to direct tumor glucose consumption that did not respond to high-dose glucose infusion, corticosteroids, or glucagon therapy.
  • The pattern of glucose uptake on (18)F-2-fluoro-2-deoxy-D-glucose positron emission tomography, with preferential tumor glucose uptake in association with a marked reduction in normal uptake in the heart, muscles, and brain, is highly suggestive of direct consumption of glucose by the tumor rather than insulin-like growth factor-2 mediated hypoglycemia.
  • In patients with large-volume metastatic malignancies, direct tumor glucose consumption should be considered in the differential diagnosis of hypoglycemia.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Adrenal Gland Neoplasms / physiopathology. Glucose / metabolism. Hypoglycemia / etiology. Pheochromocytoma / metabolism. Pheochromocytoma / physiopathology
  • [MeSH-minor] Bone Neoplasms / secondary. Disease Progression. Fatal Outcome. Fluorodeoxyglucose F18. Humans. Liver Neoplasms / secondary. Male. Positron-Emission Tomography. Tomography, Emission-Computed, Single-Photon. Tumor Burden. Young Adult


87. Gedik GK, Hoefnagel CA, Bais E, Olmos RA: 131I-MIBG therapy in metastatic phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging; 2008 Apr;35(4):725-33
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  • [Title] 131I-MIBG therapy in metastatic phaeochromocytoma and paraganglioma.
  • We report our experience for the treatment of malignant phaeochromocytoma and paraganglioma.
  • MATERIALS AND METHODS: The charts of 19 patients with malignant phaeochromocytoma (n = 12) or paraganglioma (n = 7), who were treated with (131)I-MIBG, were retrospectively reviewed.
  • CONCLUSION: Our data support that symptomatic and biochemical response can be reached with (131)I-MIBG therapy in patients with metastatic phaeochromocytoma and paraganglioma.
  • [MeSH-major] 3-Iodobenzylguanidine / therapeutic use. Adrenal Gland Neoplasms / radiotherapy. Antineoplastic Agents / therapeutic use. Paraganglioma / radiotherapy. Pheochromocytoma / radiotherapy

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  • (PMID = 18071700.001).
  • [ISSN] 1619-7070
  • [Journal-full-title] European journal of nuclear medicine and molecular imaging
  • [ISO-abbreviation] Eur. J. Nucl. Med. Mol. Imaging
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 35MRW7B4AD / 3-Iodobenzylguanidine
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88. Win Z, Al-Nahhas A, Towey D, Todd JF, Rubello D, Lewington V, Gishen P: 68Ga-DOTATATE PET in neuroectodermal tumours: first experience. Nucl Med Commun; 2007 May;28(5):359-63
MedlinePlus Health Information. consumer health - Pheochromocytoma.

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  • BACKGROUND AND AIM: Phaeochromocytoma is initially imaged with computed tomography (CT) or magnetic resonance imaging (MRI) but functional imaging is commonly needed to assess disease activity, the presence of metastasis and response to therapy.
  • We aimed to assess the utility of a new somatostatin analogue PET tracer, 68Ga-DOTATATE in the management of phaeochromocytoma.
  • METHODS: We retrospectively reviewed five patients with malignant phaeochromocytoma who underwent imaging with CT and 123I-MIBG and compared the results with those of PET imaging using 68Ga-DOTATATE.
  • CONCLUSION: The findings in our small group of patients demonstrate the value of somatostatin receptor PET imaging in malignant phaeochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / radionuclide imaging. Image Enhancement / methods. Organometallic Compounds. Pheochromocytoma / radionuclide imaging. Positron-Emission Tomography / methods

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  • (PMID = 17414885.001).
  • [ISSN] 0143-3636
  • [Journal-full-title] Nuclear medicine communications
  • [ISO-abbreviation] Nucl Med Commun
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / 68Ga-DOTATATE; 0 / Organometallic Compounds; 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine
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89. Basu S, Nair N: Stable disease and improved health-related quality of life (HRQoL) following fractionated low dose 131I-metaiodobenzylguanidine (MIBG) therapy in metastatic paediatric paraganglioma: observation on false "reverse" discordance during pre-therapy work up and its implication for patient selection for high dose targeted therapy. Br J Radiol; 2006 Aug;79(944):e53-8
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  • [Title] Stable disease and improved health-related quality of life (HRQoL) following fractionated low dose 131I-metaiodobenzylguanidine (MIBG) therapy in metastatic paediatric paraganglioma: observation on false "reverse" discordance during pre-therapy work up and its implication for patient selection for high dose targeted therapy.
  • The incidence of paraganglioma in the paediatric population is exceedingly rare, accounting for < 0.1% of childhood cancers.
  • We report here the response and toxicity profile in a case of malignant paraganglioma which was treated with what is currently perceived as an unconventional and non-standard approach, using three consecutive low doses of 131I-MIBG (a cumulative dose of 11 647.6 MBq).
  • Given the rarity of this condition, the experience gained by this therapeutic approach is intriguing from response and toxicity standpoints and may be extrapolated to malignant pheochromocytoma as well.
  • [MeSH-major] 3-Iodobenzylguanidine / administration & dosage. Abdominal Neoplasms / surgery. Bone Neoplasms / secondary. Paraganglioma, Extra-Adrenal / secondary. Radiopharmaceuticals / administration & dosage

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  • (PMID = 16861319.001).
  • [ISSN] 1748-880X
  • [Journal-full-title] The British journal of radiology
  • [ISO-abbreviation] Br J Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine
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90. Pawlu C, Bausch B, Reisch N, Neumann HP: Genetic testing for pheochromocytoma-associated syndromes. Ann Endocrinol (Paris); 2005 Jun;66(3):178-85
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Genetic testing for pheochromocytoma-associated syndromes.
  • Pheochromocytoma and paraganglioma are tumors of the autonomic nervous system.
  • Various syndromes have been found to be associated with the development of pheochromocytomas and paragangliomas: multiple endocrine neoplasia type 2 (MEN 2, susceptibility gene: RET), von Hippel-Lindau disease (VHL, susceptibility gene: VHL), neurofibromatosis 1 (NF 1), and paraganglioma syndromes type 1, 3, and 4 (susceptibility genes: succinate dehydrogenase gene, SDH, subunits D, C and B, respectively).
  • Mutational analysis of the susceptibility genes of these syndromes in patients presenting with pheochromocytoma or paraganglioma may help to judge the risks of multifocality of the tumor as well as development of malignant pheochromocytoma or of other malignant tumors.
  • Based on tumor characteristics and prevalence data we give recommendations for an efficient genetic testing procedure in patients presenting with pheochromocytomas and paragangliomas.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Multiple Endocrine Neoplasia Type 2a / genetics. Mutation. Pheochromocytoma / genetics
  • [MeSH-minor] Genes, Neurofibromatosis 1. Humans. Neurofibromatoses / genetics. Oncogene Proteins / genetics. Proto-Oncogene Proteins c-ret. Receptor Protein-Tyrosine Kinases / genetics. Thyroid Neoplasms / genetics. Tumor Suppressor Proteins / genetics. Ubiquitin-Protein Ligases / genetics. Von Hippel-Lindau Tumor Suppressor Protein

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  • (PMID = 15988378.001).
  • [ISSN] 0003-4266
  • [Journal-full-title] Annales d'endocrinologie
  • [ISO-abbreviation] Ann. Endocrinol. (Paris)
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Oncogene Proteins; 0 / Tumor Suppressor Proteins; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret; EC 2.7.10.1 / RET protein, human; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 6.3.2.19 / Ubiquitin-Protein Ligases; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
  • [Number-of-references] 43
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91. Iihara M: [Therapeutic strategy for pheochromocytoma]. Gan To Kagaku Ryoho; 2009 Oct;36(10):1623-6
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  • [Title] [Therapeutic strategy for pheochromocytoma].
  • Pheochromocytoma is the most common type of neuroendocrine tumor arising from the adrenal gland.
  • Pheochromocytoma has been termed a 10% tumor because roughly 10% of such tumors are malignant, multifocal, bilateral, and arise in extra-adrenal sites.
  • Adrenal-sparing laparoscopic surgery is a treatment of choice for bilateral pheochromocytomas.
  • Cyclophosphamide, vincristine and dacarbazine combined chemotherapy and (131)I-MIBG therapy are required for the treatment of metastatic or unresectable malignant pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / drug therapy. Adrenal Gland Neoplasms / surgery. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Pheochromocytoma / drug therapy. Pheochromocytoma / surgery

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  • (PMID = 19838020.001).
  • [ISSN] 0385-0684
  • [Journal-full-title] Gan to kagaku ryoho. Cancer & chemotherapy
  • [ISO-abbreviation] Gan To Kagaku Ryoho
  • [Language] jpn
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Japan
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92. Onuki T, Yamagishi T, Teranishi J, Suzuki K, Kondo K, Nakaigawa N, Saito K, Noguchi K, Kubota Y: [Clinical study of 38 cases of pheochromocytoma --correlation between the instability of intraoperative blood pressure and 24-hour urinary vanillylmandelic acid]. Hinyokika Kiyo; 2007 Jul;53(7):449-54
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  • [Title] [Clinical study of 38 cases of pheochromocytoma --correlation between the instability of intraoperative blood pressure and 24-hour urinary vanillylmandelic acid].
  • Pheochromocytoma is a rare tumor of chromaffin tissues most commonly arising from the adrenal medulla.
  • We retrospectively reviewed the records of 38 patients with pheochromocytoma who underwent surgical treatment between 1977 and 2004 at our Yokohama City University Medical Center and Yokohama City University Hospital.
  • One patient had bilateral adrenal tumors and pathological examination revealed malignant pheochromocytoma.
  • Six patients had an extra-adrenal tumor and in 2 patients the tumor occurred in the urinary bladder.
  • The 24-h urinary total metanephrines and vanillylmandelic acid (VMA) were the most sensitive biochemical tests for the diagnosis of pheochromocytoma.
  • Fifteen patients had intraoperative hypertensive reactions in the surgical manipulation or hypotension after tumor resection.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Hypertension / diagnosis. Intraoperative Complications / diagnosis. Pheochromocytoma / diagnosis. Vanilmandelic Acid / urine

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  • (PMID = 17702176.001).
  • [ISSN] 0018-1994
  • [Journal-full-title] Hinyokika kiyo. Acta urologica Japonica
  • [ISO-abbreviation] Hinyokika Kiyo
  • [Language] jpn
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Biomarkers; 5001-33-2 / Metanephrine; 55-10-7 / Vanilmandelic Acid
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93. Martin TP, Irving RM, Maher ER: The genetics of paragangliomas: a review. Clin Otolaryngol; 2007 Feb;32(1):7-11
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  • The genes most frequently implicated: SDHD and SDHB, also predispose to phaeochromocytoma.
  • SDHB mutations are associated with malignant phaeochromocytoma.
  • Patients who present with a family history of paraganglioma or phaeochromocytoma, with multiple tumours, or early onset tumours (<50 years), should be referred for genetic investigation.
  • [MeSH-major] DNA, Neoplasm / genetics. Genetic Predisposition to Disease. Head and Neck Neoplasms / genetics. Iron-Sulfur Proteins / genetics. Mutation. Paraganglioma / genetics. Succinate Dehydrogenase / genetics

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  • [CommentIn] Clin Otolaryngol. 2007 Aug;32(4):299-300; author reply 300-1 [17651279.001]
  • (PMID = 17298303.001).
  • [ISSN] 1749-4478
  • [Journal-full-title] Clinical otolaryngology : official journal of ENT-UK ; official journal of Netherlands Society for Oto-Rhino-Laryngology & Cervico-Facial Surgery
  • [ISO-abbreviation] Clin Otolaryngol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Iron-Sulfur Proteins; 0 / SDHD protein, human; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
  • [Number-of-references] 24
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94. Hasani-Ranjbar S, Amoli MM, Ebrahim-Habibi A, Haghpanah V, Hejazi M, Soltani A, Larijani B: Mutation screening of VHL gene in a family with malignant bilateral pheochromocytoma: from isolated familial pheochromocytoma to von Hippel-Lindau disease. Fam Cancer; 2009;8(4):465-71
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  • [Title] Mutation screening of VHL gene in a family with malignant bilateral pheochromocytoma: from isolated familial pheochromocytoma to von Hippel-Lindau disease.
  • von Hippel-Lindau (vHL) disease is an inherited, autosomal dominant syndrome manifested by a variety of benign and malignant tumors.
  • In this paper we report the presence of a single nucleotide mutation in exon 3 of VHL gene c499 C>T causing substitution of Arginine by Tryptophan at position 167 (R 167 W).
  • It was detected in a family with bilateral malignant pheochromocytoma who has been followed for at least 9 years as RET negative isolated familial pheochromocytoma, finally diagnosed as von Hipple-Lindau disease according to retinal angioma and VHL gene mutation.
  • VHL type 2 presenting with both pheochromocytoma and retinal angioma in this family found to be associated with the new missense mutation (c499 C>T) of VHL gene.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Pheochromocytoma / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics. von Hippel-Lindau Disease / genetics

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  • (PMID = 19649731.001).
  • [ISSN] 1573-7292
  • [Journal-full-title] Familial cancer
  • [ISO-abbreviation] Fam. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] EC 2.3.2.27 / Von Hippel-Lindau Tumor Suppressor Protein; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret; EC 2.7.10.1 / RET protein, human; EC 6.3.2.- / VHL protein, human
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95. Nocca D, Aggarwal R, Mathieu A, Blanc PM, Denève E, Salsano V, Figueira G, Sanders G, Domergue J, Millat B, Fabre PR: Laparoscopic surgery and corticoadrenalomas. Surg Endosc; 2007 Aug;21(8):1373-6
NCI CPTAC Assay Portal. NCI CPTAC Assay Portal .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • However, the situation is not so clearcut when the issue is laparoscopic excision of malignant adrenal tumors.
  • We present our results of laparoscopic adrenalectomy for treating malignant tumors over the past decade.
  • Twelve patients (9%) had a malignant tumor: nine corticoadrenalomas, one pleomorphic sarcoma, one metastatic deposit from a previously excised colonic cancer, and one malignant pheochromocytoma.
  • Five years later, the same patient had a reoperation for an intra-abdominal retrogastric recurrence of her tumor and continues to do well.
  • Considering the results presented within this study, it seems that the laparoscopic removal of a corticoadrenaloma should not worsen the prognosis, provided the surgeon respects the primary rules of oncologic resectional surgery.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenalectomy. Laparoscopy

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  • (PMID = 17356945.001).
  • [ISSN] 1432-2218
  • [Journal-full-title] Surgical endoscopy
  • [ISO-abbreviation] Surg Endosc
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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96. Fuentes C, Menéndez E, Pineda J, Martínez De Esteban JP, Anda E, Goñi MJ, Bausch B, Neumann HP: The malignant potential of a succinate dehydrogenase subunit B germline mutation. J Endocrinol Invest; 2006 Apr;29(4):350-2

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The malignant potential of a succinate dehydrogenase subunit B germline mutation.
  • We diagnosed a malignant retroperitoneal paraganglioma in a 64-yr-old man with bone metastasis in 2001.
  • Two years later a retroperitoneal benign paraganglioma was found and resected in his 32-yr-old daughter.
  • Thus we diagnosed in this family a paraganglioma syndrome.
  • We detected in the SDHB gene the mutation SDHB c. 558-3 C> G affecting the splice site of exon 5.
  • We conclude that the SDHB mutation predisposes to abdominal extra-adrenal and potential malignant pheochromocytoma with incomplete penetrance.
  • [MeSH-major] Germ-Line Mutation. Iron-Sulfur Proteins / genetics. Paraganglioma / genetics. Protein Subunits / genetics. Retroperitoneal Neoplasms / genetics. Succinate Dehydrogenase / genetics

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  • (PMID = 16699302.001).
  • [ISSN] 0391-4097
  • [Journal-full-title] Journal of endocrinological investigation
  • [ISO-abbreviation] J. Endocrinol. Invest.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Iron-Sulfur Proteins; 0 / Protein Subunits; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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97. Niveditha SR, Suguna BV, Krishnamurthy, Rajnikanth, Babu KG, Shukla AK: Cytologic features of malignant cystic pheochromocytoma: a case report. Acta Cytol; 2007 Mar-Apr;51(2):200-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cytologic features of malignant cystic pheochromocytoma: a case report.
  • BACKGROUND: Image-guided fine needle aspiration (FNA) biopsy of deep-seated lesions (e.g., retroperitoneal) has aided preoperative diagnosis and treatment planning.
  • Cytologic features are described for a malignant pheochromocytoma (greater percentage of smaller cells and absence of hyaline globules) with extensive cystic degeneration that was later confirmed by histopathologic, immunohistochemical and ultrastructural studies.
  • FNA performed on the mass revealed interesting features of pheochromocytoma with an abundance of small cells.
  • CONCLUSION: The cytopathologist should be aware of the cytologic features of pheochromocytoma so as to detect a clinically unsuspected lesion.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Medulla / pathology. Carcinoma, Renal Cell / diagnosis. Cysts / pathology. Paraganglioma / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Biopsy, Fine-Needle. Diagnosis, Differential. Duodenum / pathology. Fatal Outcome. Humans. Hypertension / etiology. Kidney / pathology. Male. Middle Aged. Neoplasm Invasiveness. Predictive Value of Tests

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  • (PMID = 17425203.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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98. Opocher G, Schiavi F, Iacobone M, Toniato A, Sattarova S, Erlic Z, Martella M, Mian C, Merante Boschin I, Zambonin L, De Lazzari P, Murgia A, Pelizzo MR, Favia G, Mantero F: Familial nonsyndromic pheochromocytoma. Ann N Y Acad Sci; 2006 Aug;1073:149-55
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  • [Title] Familial nonsyndromic pheochromocytoma.
  • The molecular basis for the familial pheochromocytoma has been largely elucidated and the role of germline mutation of the VHL, RET, SDHB, and SDHD genes has been established.
  • The proband of family 1 is a man who had a bilateral pheochromocytoma at the age of 32 and a local recurrence at the age of 48 years.
  • His brother died of malignant pheochromocytoma and his nephew died suddenly of an undiagnosed pheochromocytoma.
  • The proband of family 2 is a female who had a 5-cm benign adrenal pheochromocytoma at the age of 34 years, while her cousin (maternal branch) had a monolateral pheochromocytoma at the age of 42 years.
  • The proband of family 3 is a female who had a bilateral pheochromocytoma at the age of 66 years.
  • Her sister had a bilateral pheochromocytoma and breast cancer at the age of 54 years.
  • Several other tumors were recorded in this family, including laryngeal cancer, leukemia, and a case of medullary thyroid carcinoma (MTC) in one brother.
  • In family 4, the proband was a female who had a bilateral pheochromocytoma at the age of 46 years and a local recurrence a few years later, with liver metastases from the pheochromocytoma.
  • Her brother had a monolateral benign pheochromocytoma.
  • This case revealed a VHL sequence variant IVS2+43 A>G, which was also found in one other unrelated sporadic pheochromocytoma.
  • In family 5, the proband was a female who had a right adrenal pheochromocytoma at the age of 50 years and a breast cancer at 49 years of age.
  • Her mother had had a right adrenal pheochromocytoma at 61 years of age.
  • Although other molecular mechanisms, such as particular variants in untranslated regions or partial gene deletions, cannot be ruled out, we think finding families with nonsyndromic pheochromocytoma without any RET, VHL, SDHB, SDHC, SDHD, or EGLN3 mutation may argue in favor of the presence of other pheochromocytoma susceptibility genes.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Pheochromocytoma / genetics
  • [MeSH-minor] Genetic Predisposition to Disease. Humans. Proto-Oncogene Proteins c-ret / genetics. Succinate Dehydrogenase / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics

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  • (PMID = 17102081.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] EC 1.3.99.1 / Succinate Dehydrogenase; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret; EC 2.7.10.1 / RET protein, human; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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99. Meyer-Rochow GY, Jackson NE, Conaglen JV, Whittle DE, Kunnimalaiyaan M, Chen H, Westin G, Sandgren J, Stålberg P, Khanafshar E, Shibru D, Duh QY, Clark OH, Kebebew E, Gill AJ, Clifton-Bligh R, Robinson BG, Benn DE, Sidhu SB: MicroRNA profiling of benign and malignant pheochromocytomas identifies novel diagnostic and therapeutic targets. Endocr Relat Cancer; 2010 Sep;17(3):835-46
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] MicroRNA profiling of benign and malignant pheochromocytomas identifies novel diagnostic and therapeutic targets.
  • There is increasing evidence to suggest that miRNAs could be useful in cancer diagnosis, prognosis, and therapy.
  • We performed miRNA microarray expression profiling on a cohort of 12 benign and 12 malignant pheochromocytomas and identified a number of differentially expressed miRNAs.
  • These results were validated in a separate cohort of ten benign and ten malignant samples using real-time quantitative RT-PCR; benign samples had a minimum follow-up of at least 2 years.
  • It was found that IGF2 as well as its intronic miR-483-5p was over-expressed, while miR-15a and miR-16 were under-expressed in malignant tumours compared with benign tumours.
  • These miRNAs were found to be diagnostic and prognostic markers for malignant pheochromocytoma.
  • The functional role of miR-15a and miR-16 was investigated in vitro in the rat PC12 pheochromocytoma cell line, and these miRNAs were found to regulate cell proliferation via their effect on cyclin D1 and apoptosis.
  • These data indicate that miRNAs play a pivotal role in the biology of malignant pheochromocytoma, and represent an important class of diagnostic and prognostic biomarkers and therapeutic targets warranting further investigation.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Biomarkers, Tumor / genetics. Gene Expression Profiling. Gene Expression Regulation, Neoplastic. MicroRNAs / physiology. Pheochromocytoma / genetics
  • [MeSH-minor] Adrenal Glands / metabolism. Adrenal Glands / pathology. Animals. Apoptosis. Blotting, Western. Cell Cycle. Cohort Studies. Follow-Up Studies. Humans. Oligonucleotide Array Sequence Analysis. Prognosis. RNA, Messenger / genetics. Rats. Reverse Transcriptase Polymerase Chain Reaction. Survival Rate. Tumor Cells, Cultured


100. Jimenez C, Cabanillas ME, Santarpia L, Jonasch E, Kyle KL, Lano EA, Matin SF, Nunez RF, Perrier ND, Phan A, Rich TA, Shah B, Williams MD, Waguespack SG: Use of the tyrosine kinase inhibitor sunitinib in a patient with von Hippel-Lindau disease: targeting angiogenic factors in pheochromocytoma and other von Hippel-Lindau disease-related tumors. J Clin Endocrinol Metab; 2009 Feb;94(2):386-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Use of the tyrosine kinase inhibitor sunitinib in a patient with von Hippel-Lindau disease: targeting angiogenic factors in pheochromocytoma and other von Hippel-Lindau disease-related tumors.
  • EVIDENCE ACQUISITION: We present a patient with von Hippel-Lindau disease with multiple renal and pancreatic tumors and a malignant pheochromocytoma infiltrative of the sacrum and associated with lymph nodule metastases.
  • The pheochromocytoma expressed high protein level of vascular endothelial growth factor and platelet-derived growth factor-beta receptor.
  • EVIDENCE SYNTHESIS: Treatment against malignant pheochromocytoma with surgery, chemotherapy, or participation in clinical trials was not feasible because of the patient's poor performance status, the presence of multiple tumors, and the extension of the pheochromocytoma into the bones.
  • Six months of treatment with sunitinib was associated with normalization of the patient's performance status and blood pressure, absence of symptoms of catecholamine excess, weight gain, disappearance of pain, shrinkage of each of the tumors (50% in the largest renal tumor, 38% in the largest islet cell tumor, 21% in the pelvic malignant pheochromocytoma), and reduction of plasma normetanephrines and chromogranin A.
  • CONCLUSION: This study provides evidence that targeting tyrosine kinase receptors such as the vascular endothelial growth factor pathway and the platelet-derived growth factor-beta receptor may have value in the treatment of VHL-related tumors including pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / drug therapy. Indoles / therapeutic use. Neoplasms, Multiple Primary / drug therapy. Pheochromocytoma / drug therapy. Pyrroles / therapeutic use. von Hippel-Lindau Disease / drug therapy
  • [MeSH-minor] Adult. Angiogenesis Inducing Agents / antagonists & inhibitors. Angiogenesis Inhibitors / therapeutic use. Drug Delivery Systems / methods. Female. Humans. Protein Kinase Inhibitors / therapeutic use. Treatment Outcome. Von Hippel-Lindau Tumor Suppressor Protein / genetics

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  • MedlinePlus Health Information. consumer health - Von Hippel-Lindau Disease.
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  • (PMID = 19017755.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Case Reports; Evaluation Studies; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Angiogenesis Inducing Agents; 0 / Angiogenesis Inhibitors; 0 / Indoles; 0 / Protein Kinase Inhibitors; 0 / Pyrroles; 0 / sunitinib; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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