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1. 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
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  • [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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2. 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
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  • 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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3. 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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4. 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


5. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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6. 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


7. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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


8. 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


9. 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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10. 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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  • [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


11. 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


12. 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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  • [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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13. 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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14. Surgit O: Clipless and sutureless laparoscopic adrenalectomy carried out with the LigaSure device in 32 patients. Surg Laparosc Endosc Percutan Tech; 2010 Apr;20(2):109-13
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  • BACKGROUND: Laparoscopic adrenalectomy has become the standard procedure for treating patents with adrenal masses.
  • METHODS: The LigaSure device was used in 32 patients undergoing laparoscopic adrenalectomy for adrenal masses.
  • In 1 patient the adrenal tumor had invaded the ipsilateral kidney, so laparoscopic nephrectomy was also carried out during the same operation.
  • In another patient, a renal cell carcinoma in the left kidney had metastasized to the right adrenal gland.
  • Both the kidney and the contralateral adrenal gland were removed laparoscopically during the same operation.
  • RESULTS: Adrenal masses had a mean greatest diameter of 3.48 cm (range 2 to 11 cm).
  • Adrenal tumor types included adrenocortical adenoma (16 patients), pheochromocytoma (13 patients), malignant pheochromocytoma (1 patient), chromophobic carcinoma (1 patient), and metastasis from a renal cell carcinoma (1 patient).
  • For patients with conditions such as renal cell carcinoma combined with metastasis to the contralateral adrenal gland, nephrectomy, and contralateral adrenalectomy can be carried out during the same laparoscopic operation.
  • [MeSH-minor] Adenoma, Chromophobe / surgery. Adrenal Gland Neoplasms / secondary. Adrenal Gland Neoplasms / surgery. Adrenocortical Adenoma / surgery. Adult. Aged. Blood Loss, Surgical. Carcinoma, Renal Cell / secondary. Carcinoma, Renal Cell / surgery. Female. Humans. Kidney Neoplasms / surgery. Male. Middle Aged. Nephrectomy / methods. Pheochromocytoma / surgery

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  • (PMID = 20393338.001).
  • [ISSN] 1534-4908
  • [Journal-full-title] Surgical laparoscopy, endoscopy & percutaneous techniques
  • [ISO-abbreviation] Surg Laparosc Endosc Percutan Tech
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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15. Nap RR, Meinardi JR, van den Berg G, Dullaart RP, de Vries J, Wolffenbuttel BH: [Long-term follow-up is indicated after surgery for a phaeochromocytoma]. Ned Tijdschr Geneeskd; 2006 May 13;150(19):1045-9
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  • [Title] [Long-term follow-up is indicated after surgery for a phaeochromocytoma].
  • [Transliterated title] Langdurige controle aangewezen na chirurgische behandeling wegens een feochromocytoom.
  • Two patients presented with symptoms that suggested a recurrence of phaeochromocytoma, 17 and 14 years after a first manifestation of this disease for which they had undergone successful surgery.
  • The first patient, a 57-year-old man, had typical symptoms of phaeochromocytoma and had recently experienced deterioration of the glycaemic control of his diabetes mellitus type 2.
  • In addition to peritoneal metastases confirmed to be caused by malignant phaeochromocytoma he also had a carcinoma of the caecum.
  • Ultrasound examination revealed a tumour in the right upper abdomen and a recurrence of phaeochromocytoma was diagnosed; an intact pregnancy was also found.
  • Studies report recurrence ofphaeochromocytoma in 8-20% of cases, and such a recurrence may be either benign or malignant.
  • Factors predictive for recurrence are older age, a malignant primary tumour, tumour weight above 60 g, extra-adrenal origin of the tumour and peroperative tumour spill.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Pheochromocytoma / surgery
  • [MeSH-minor] Adult. Disease-Free Survival. Female. Humans. Male. Middle Aged. Neoplasm Metastasis. Neoplasm Recurrence, Local / surgery. Prognosis. Reoperation. Risk Factors. Time Factors. Treatment Outcome

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  • (PMID = 16733977.001).
  • [ISSN] 0028-2162
  • [Journal-full-title] Nederlands tijdschrift voor geneeskunde
  • [ISO-abbreviation] Ned Tijdschr Geneeskd
  • [Language] dut
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Netherlands
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16. Jerbi Omezzine S, Hafsa C, Letaief R, Krifa H, Gannouni A, Hamza HA: [Paralyzing sciatica as a presenting symptom of extraadrenal malignant pheochromocytoma]. J Radiol; 2008 Feb;89(2):259-61
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  • [Title] [Paralyzing sciatica as a presenting symptom of extraadrenal malignant pheochromocytoma].
  • [Transliterated title] Sciatique paralysante révélant un phéochromocytome malin extra surrénalien.
  • [MeSH-major] Paralysis / diagnosis. Pheochromocytoma / diagnosis. Sacrum / pathology. Sciatica / diagnosis. Spinal Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Fatal Outcome. Female. Humans. Magnetic Resonance Imaging. Neoplasm Invasiveness. Retroperitoneal Neoplasms / diagnosis. Tomography, X-Ray Computed


17. Almas A, Akhter J, Ali F, Barakzai MA, Mansoor-Ul-Haq: Isolated bony metastasis of malignant pheochromocytoma: a rare clinical entity. J Coll Physicians Surg Pak; 2009 May;19(5):329-30
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  • [Title] Isolated bony metastasis of malignant pheochromocytoma: a rare clinical entity.
  • [MeSH-major] Acetabulum. Adrenal Gland Neoplasms / pathology. Bone Neoplasms / secondary. Pheochromocytoma / secondary


18. 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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19. 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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20. Browne I, Brady I, Hannon V, McKeating K: Anaesthesia for phaeochromocytoma and sickle cell disease in pregnancy. Int J Obstet Anesth; 2005 Jan;14(1):66-9
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  • [Title] Anaesthesia for phaeochromocytoma and sickle cell disease in pregnancy.
  • She also had a phaeochromocytoma with inadequately controlled hypertension.
  • This report describes the multi-disciplinary work-up and peri-operative management necessary to optimise her medical condition before caesarean section at 39 weeks' gestation and subsequent removal of a malignant phaeochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Anemia, Sickle Cell / complications. Anesthesia, Obstetrical / methods. Pheochromocytoma / complications. Pregnancy Complications, Hematologic. Pregnancy Complications, Neoplastic


21. 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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  • [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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22. 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


23. 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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24. 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


25. 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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26. Dalan R, Chadachan V, Leow MK: Isolated adrenal mass in cachectic patients-don't forget malignant pheochromocytoma. Ann Surg Oncol; 2008 Feb;15(2):655-6
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  • [Title] Isolated adrenal mass in cachectic patients-don't forget malignant pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology


27. Ch'ng ES, Hoshida Y, Iizuka N, Morii E, Ikeda JI, Yamamoto A, Tomita Y, Hanasaki H, Katsuya T, Maeda K, Ohishi M, Rakugi H, Ogihara T, Aozasa K: Composite malignant pheochromocytoma with malignant peripheral nerve sheath tumour: a case with 28 years of tumour-bearing history. Histopathology; 2007 Sep;51(3):420-2
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  • [Title] Composite malignant pheochromocytoma with malignant peripheral nerve sheath tumour: a case with 28 years of tumour-bearing history.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Nerve Sheath Neoplasms / pathology. Pheochromocytoma / pathology

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  • (PMID = 17727489.001).
  • [ISSN] 0309-0167
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD56; 0 / Chromogranins; 0 / Synaptophysin
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28. 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


29. 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


30. 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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  • [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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31. 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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32. 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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  • [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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33. 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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34. Koh YH, Wu HY, Lim WP: Pain and nausea in malignant pheochromocytoma: a pharmacological minefield. J Pain Symptom Manage; 2008 Mar;35(3):239-41
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  • [Title] Pain and nausea in malignant pheochromocytoma: a pharmacological minefield.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Antiemetics / adverse effects. Metoclopramide / adverse effects. Nausea / drug therapy. Pheochromocytoma / complications


35. 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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36. 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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37. 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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  • (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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38. Suh I, Shibru D, Eisenhofer G, Pacak K, Duh QY, Clark OH, Kebebew E: Candidate genes associated with malignant pheochromocytomas by genome-wide expression profiling. Ann Surg; 2009 Dec;250(6):983-90
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  • [Title] Candidate genes associated with malignant pheochromocytomas by genome-wide expression profiling.
  • OBJECTIVE: To improve our understanding of the molecular mechanisms involved in malignant pheochromocytoma by examining differences in the gene expression profile between benign and malignant tumors.
  • BACKGROUND: The molecular events involved in the malignant transformation of pheochromocytoma are poorly understood.
  • There are also no reliable and uniformly accepted histopathologic criteria to distinguish benign from malignant pheochromocytoma.
  • METHODS: We performed genome-wide expression profiling of 58 pheochromocytomas (29 benign and sporadic, 16 benign and hereditary, 13 malignant) with technical and biologic replication.
  • Supervised cluster analysis showed almost completely separate clustering between benign and malignant tumors.
  • Gene set enrichment analysis revealed significant correlation between the microarray profiles of malignant pheochromocytomas and several known molecular pathways associated with carcinogenesis and dedifferentiation.
  • Ten differentially expressed genes had high diagnostic accuracy, and 5 of these genes (CFC1, FAM62B, HOMER1, LRRN3, TBX3, ADAMTS) in combination had an area under the receiver operating characteristic (ROC) curve of 0.96 for distinguishing benign versus malignant tumors.
  • CONCLUSIONS: Differentially expressed genes between benign and malignant pheochromocytomas distinguish between these tumors with high diagnostic accuracy.
  • Our findings provide new insight into the genes and molecular pathways that may be involved in malignant pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Gene Expression Regulation, Neoplastic. Genes, Neoplasm / genetics. Genetic Association Studies / methods. Pheochromocytoma / genetics. RNA, Neoplasm / genetics

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  • (PMID = 19661783.001).
  • [ISSN] 1528-1140
  • [Journal-full-title] Annals of surgery
  • [ISO-abbreviation] Ann. Surg.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / RNA, Neoplasm
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39. Yamaguchi S, Shibata H, Miyashita K, Kurihara I, Murai-Takeda A, Mitsuishi Y, Motosugi Y, Saito Y, Hayashi K, Itoh H: Gastrointestinal pseudo-obstruction after debulking surgery of malignant pheochromocytoma, improved by intravenous administration of alpha-adrenergic receptor blocker, phentolamine. Hypertens Res; 2010 Jul;33(7):753-4
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  • [Title] Gastrointestinal pseudo-obstruction after debulking surgery of malignant pheochromocytoma, improved by intravenous administration of alpha-adrenergic receptor blocker, phentolamine.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenergic alpha-Antagonists / administration & dosage. Colonic Pseudo-Obstruction / drug therapy. Megacolon / drug therapy. Phentolamine / administration & dosage. Pheochromocytoma / surgery. Postoperative Complications / drug therapy

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  • (PMID = 20505671.001).
  • [ISSN] 1348-4214
  • [Journal-full-title] Hypertension research : official journal of the Japanese Society of Hypertension
  • [ISO-abbreviation] Hypertens. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Adrenergic alpha-Antagonists; 0 / Catecholamines; Z468598HBV / Phentolamine
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40. Schaefer IM, Martinez R, Enders C, Loertzer H, Brück W, Rohde V, Füzesi L, Gutenberg A: Molecular cytogenetics of malignant pheochromocytoma with cerebral metastasis. Cancer Genet Cytogenet; 2010 Jul 15;200(2):194-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Molecular cytogenetics of malignant pheochromocytoma with cerebral metastasis.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Brain Neoplasms / secondary. Chromosome Aberrations. Pheochromocytoma / genetics


41. 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


42. 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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43. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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44. Grajower MM: Malignant paraganglioma of thyroid. Endocr Pract; 2006 Nov-Dec;12(6):696-7
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  • [Title] Malignant paraganglioma of thyroid.
  • [MeSH-major] Carcinoma, Medullary / diagnosis. Laryngeal Neoplasms / diagnosis. Paraganglioma, Extra-Adrenal / diagnosis. Thyroid Neoplasms / diagnosis
  • [MeSH-minor] Biopsy, Fine-Needle. Diagnosis, Differential. Female. Humans. Thyroid Nodule / diagnosis

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  • [CommentOn] Endocr Pract. 2006 Jul-Aug;12(4):432-5 [16901801.001]
  • (PMID = 17240539.001).
  • [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; Comment; Letter
  • [Publication-country] United States
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45. 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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46. Ricketts CJ, Forman JR, Rattenberry E, Bradshaw N, Lalloo F, Izatt L, Cole TR, Armstrong R, Kumar VK, Morrison PJ, Atkinson AB, Douglas F, Ball SG, Cook J, Srirangalingam U, Killick P, Kirby G, Aylwin S, Woodward ER, Evans DG, Hodgson SV, Murday V, Chew SL, Connell JM, Blundell TL, Macdonald F, Maher ER: Tumor risks and genotype-phenotype-proteotype analysis in 358 patients with germline mutations in SDHB and SDHD. Hum Mutat; 2010 Jan;31(1):41-51
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  • [Title] Tumor risks and genotype-phenotype-proteotype analysis in 358 patients with germline mutations in SDHB and SDHD.
  • Succinate dehydrogenase B (SDHB) and D (SDHD) subunit gene mutations predispose to adrenal and extraadrenal pheochromocytomas, head and neck paragangliomas (HNPGL), and other tumor types.
  • We report tumor risks in 358 patients with SDHB (n=295) and SDHD (n=63) mutations.
  • Risks of HNPGL and pheochromocytoma in SDHB mutation carriers were 29% and 52%, respectively, at age 60 years and 71% and 29%, respectively, in SDHD mutation carriers.
  • Risks of malignant pheochromocytoma and renal tumors (14% at age 70 years) were higher in SDHB mutation carriers; 55 different mutations (including a novel recurrent exon 1 deletion) were identified.
  • However, SDHD mutations predicted to result in loss of expression or a truncated or unstable protein were associated with a significantly increased risk of pheochromocytoma compared to missense mutations that were not predicted to impair protein stability (most such cases had the common p.Pro81Leu mutation).
  • Analysis of the largest cohort of SDHB/D mutation carriers has enhanced estimates of penetrance and tumor risk and supports in silicon protein structure prediction analysis for functional assessment of mutations.
  • The differing effect of the SDHD p.Pro81Leu on HNPGL and pheochromocytoma risks suggests differing mechanisms of tumorigenesis in SDH-associated HNPGL and pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Germ-Line Mutation. Paraganglioma / genetics. Pheochromocytoma / genetics. Succinate Dehydrogenase / genetics

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  • [CommentIn] Hum Mutat. 2010 Jun;31(6):761-2 [20513144.001]
  • (PMID = 19802898.001).
  • [ISSN] 1098-1004
  • [Journal-full-title] Human mutation
  • [ISO-abbreviation] Hum. Mutat.
  • [Language] eng
  • [Grant] United Kingdom / Department of Health / / DHCS/06/06/013
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / SDHD protein, human; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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47. 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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48. 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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49. 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


50. Safwat AS, Bissada NK, Seyam RM, Al Sobhi S, Hanash KA: The clinical spectrum of phaeochromocytoma: analysis of 115 patients. BJU Int; 2008 Jun;101(12):1561-4
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  • [Title] The clinical spectrum of phaeochromocytoma: analysis of 115 patients.
  • OBJECTIVE: To analyse the presentation, manifestations and outcome in consecutive patients with phaeochromocytoma, as this disease has a wide range of pathological and clinical expressions.
  • PATIENTS AND METHODS: The records of 115 patients with phaeochromocytoma were analysed retrospectively, recording the patients' age, sex, presenting symptoms and clinical signs, chemical, radiological and pathological findings and associated conditions.
  • RESULTS: Of the 115 patients, 90 had adrenal tumours, 18 extra-adrenal and seven combined adrenal and extra-adrenal tumours.
  • Ten patients had malignant and 105 had benign phaeochromocytoma.
  • Eighty-six patients had sporadic and 29 had familial phaeochromocytoma, comprising eight with von Hippel-Lindau (VHL) disease, 17 with multiple endocrine neoplasia type II (MEN II) and four with von Recklinghausen disease.
  • Two patients with sporadic phaeochromocytoma had Grave's disease.
  • Ten patients (8.7%) had malignant phaeochromocytoma, of whom two had MEN II.
  • CONCLUSION: Phaeochromocytoma is an interesting clinical entity with a wide spectrum of pathological and clinical manifestations.
  • The diagnosis of phaeochromocytoma is confirmed by chemical methods, and located using imaging techniques, with computed tomography, magnetic resonance imaging and (131)I-meta-iodobenzyl guanidine radioisotope scanning being the most common.
  • This series reflects the pathological and clinical spectrum of phaeochromocytoma.
  • The presence of other manifestations of familial phaeochromocytoma influenced the presentation and prognosis of these patients.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Hypertension / etiology. Pheochromocytoma / pathology


51. 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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  • [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


52. 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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53. 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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54. de Lloyd AC, Munigoti S, Davies JS, Scott-Coombes D: A rare and life-threatening cause of pseudo-obstruction in two surgical patients. BMJ Case Rep; 2010;2010
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  • We describe two patients who presented with non-mechanical bowel obstruction as a consequence of an underlying, undiagnosed phaeochromocytoma.
  • An abdominal scan revealed an adrenal tumour (subsequently found to be a phaeochromocytoma) but no structural cause for obstruction.
  • Treatment of the phaeochromocytoma was associated with prompt restoration of bowel function.
  • An abdominal scan identified an infiltrative adrenal tumour but no mechanical cause for bowel obstruction.
  • Histology confirmed a malignant phaeochromocytoma.
  • Bowel obstruction as the presenting symptom of phaeochromocytoma is well described but rarely identified.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Intestinal Pseudo-Obstruction / etiology. Pheochromocytoma / complications
  • [MeSH-minor] Aged. Diagnosis, Differential. Female. Humans. Male

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55. 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


56. 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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57. Carvalho MR, Dias T, Rodrigues A, Machado AP, Esteves R, do Carmo I: Alpha blockade with doxazosin in pheochromocytoma--report of three cases. Rev Port Cardiol; 2010 Feb;29(2):299-308
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  • [Title] Alpha blockade with doxazosin in pheochromocytoma--report of three cases.
  • Pheochromocytoma is a catecholamine-secreting tumor, for which the treatment of choice is complete surgical resection, when possible.
  • The authors report three different cases in which doxazosin was used: as preoperative preparation of a patient with pheochromocytoma, as preparation for therapy in I-131-MIBG of a patient with a malignant pheochromocytoma, and as a pre-cesarean preparation in a pregnant woman with multiple endocrine neoplasia type 2A (MEN-2A).
  • [MeSH-major] Adrenal Gland Neoplasms / drug therapy. Adrenergic alpha-Antagonists / therapeutic use. Doxazosin / therapeutic use. Pheochromocytoma / drug therapy

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  • (PMID = 20545254.001).
  • [ISSN] 0870-2551
  • [Journal-full-title] Revista portuguesa de cardiologia : orgão oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology
  • [ISO-abbreviation] Rev Port Cardiol
  • [Language] eng; por
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Portugal
  • [Chemical-registry-number] 0 / Adrenergic alpha-Antagonists; NW1291F1W8 / Doxazosin
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58. 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


59. 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


60. Fitzgerald PA, Goldsby RE, Huberty JP, Price DC, Hawkins RA, Veatch JJ, Dela Cruz F, Jahan TM, Linker CA, Damon L, Matthay KK: Malignant pheochromocytomas and paragangliomas: a phase II study of therapy with high-dose 131I-metaiodobenzylguanidine (131I-MIBG). Ann N Y Acad Sci; 2006 Aug;1073:465-90
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  • [Title] Malignant pheochromocytomas and paragangliomas: a phase II study of therapy with high-dose 131I-metaiodobenzylguanidine (131I-MIBG).
  • Thirty patients with malignant pheochromocytoma (PHEO) or paraganglioma (PGL) were treated with high-dose 131I-MIBG.
  • 131I-MIBG was synthesized on-site, by exchange-labeling 131I with 127I-MIBG in a solid-phase Cu2+-catalyzed exchange reaction.
  • High-dose 131I-MIBG resulted in the following overall tumor responses in 30 patients: 4 sustained complete remissions (CRs); 15 sustained partial remissions (PRs); 1 sustained stable disease (SD); 5 progressive disease (PD); 5 initial PRs or SD but relapsed to PD.
  • [MeSH-major] 3-Iodobenzylguanidine / therapeutic use. Adrenal Gland Neoplasms / radiotherapy. Iodine Radioisotopes / therapeutic use. Paraganglioma / radiotherapy. Pheochromocytoma / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Child. Diagnosis, Differential. Dose-Response Relationship, Radiation. Female. Humans. Male. Middle Aged

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  • (PMID = 17102115.001).
  • [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 / 2M01 RR01271
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Iodine Radioisotopes; 35MRW7B4AD / 3-Iodobenzylguanidine
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61. 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


62. 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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63. 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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64. 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
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  • [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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65. Mezzadri NA, Catalina Mandry A, Sinagra DL, Eduardo Falco J, Fernández Vila JM: [Laparoscopic approach in the treatment of malignant adrenal tumours]. Cir Esp; 2010 May;87(5):306-11
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  • [Title] [Laparoscopic approach in the treatment of malignant adrenal tumours].
  • BACKGROUND: Malignant primary or secondary adrenal tumours are uncommon.
  • Although controversy exists on this issue, the increasing experience in laparoscopic surgery extends the indication for laparoscopic adrenalectomy to potentially malignant and to metastatic adrenal tumours.
  • Our aim was to evaluate the technical feasibility of laparoscopic adrenalectomy for malignant neoplasias, describing the results of our consecutive series of patients.
  • MATERIAL AND METHODS: We retrospectively analysed 13 patients who underwent laparoscopic adrenalectomy for malignant neoplasia between March 1999 and June 2009, at the Hospital de Clínicas of the Universidad of Buenos Aires and at the Hospital Alemán of Buenos Aires.
  • RESULTS: Thirteen laparoscopic adrenalectomies were performed due to malignant neoplasia.
  • Five patients had an adrenal carcinoma, 1 patient a malignant phaeochromocytoma, and 7 patients had metastatic tumours.
  • CONCLUSION: Laparoscopic adrenalectomy is a reasonable technique for malignant adrenal tumours, when the open oncological resection can be reproduced by the laparoscopic approach.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Laparoscopy / methods

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  • [Copyright] Copyright 2009 AEC. Published by Elsevier Espana. All rights reserved.
  • (PMID = 20382378.001).
  • [ISSN] 1578-147X
  • [Journal-full-title] Cirugía española
  • [ISO-abbreviation] Cir Esp
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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66. 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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67. 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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  • [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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68. Strong VE, Kennedy T, Al-Ahmadie H, Tang L, Coleman J, Fong Y, Brennan M, Ghossein RA: Prognostic indicators of malignancy in adrenal pheochromocytomas: clinical, histopathologic, and cell cycle/apoptosis gene expression analysis. Surgery; 2008 Jun;143(6):759-68
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  • [Title] Prognostic indicators of malignancy in adrenal pheochromocytomas: clinical, histopathologic, and cell cycle/apoptosis gene expression analysis.
  • BACKGROUND: Pheochromocytomas are malignant in approximately 10% of patients.
  • The histologic differentiation between benign and malignant tumors is difficult, the latter diagnosed by the presence of metastatic disease or recurrence.
  • AIM: To determine if postoperative histologic evaluation using the previously proposed Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and cell cycle/apoptosis markers can predict patients at risk for recurrence.
  • METHODS: Using the Memorial Sloan-Kettering Cancer Center adrenal database, we identified 48 patients with 51 resected pheochromocytomas (1987-2006).
  • This pheochromocytoma scoring system is based on the presence of 12 different histologic parameters, including tumor necrosis, mitotic rate, tumor cell spindling, and the presence of large cell nests.
  • In addition, we constructed a tissue microarray of all 5 malignant tumors and 41 of the benign tumors.
  • RESULTS: Forty-three patients had a benign clinical course while 5 patients harbored a clinically malignant pheochromocytoma.
  • Tumor necrosis (focal or confluent) was a particularly powerful indicator of malignancy present in 4 of 5 patients (80%) with malignant tumors, but only in 3 of 42 cases (7%) with benign neoplasms (P = .0009).
  • The presence of a high mitotic rate (>3/10 high power fields) and tumor cell spindling significantly correlated with malignancy (P = .026 and .041, respectively).
  • High cellularity was more often present in the malignant lesions (P = .050).
  • There was a highly significant difference in PASS scores between benign and malignant cases (P = .0003).
  • All malignant pheochromocytomas had a PASS score >/=6, well above the previously proposed >/=4 cutoff value.
  • Two of the 4 patients testing positive for Ki-67 (>2% nuclear staining) had a clinically malignant course while only 3 (7%) of the 41 cases with lower Ki-67 positivity rate behaved in a malignant fashion (P = .055).
  • Ki-67-positive tumor had a significantly higher chance of harboring tumor necrosis than Ki-67-negative neoplasms (P < .01).
  • There was no difference in staining between benign and malignant pheochromocytomas using p53, Bcl-2, mdm-2, cyclin D1, p21, and p27. CONCLUSIONS:.
  • (1) A PASS score of <4 predicted benign pheochromocytomas. (2) All malignant pheochromocytomas had a PASS score >/=6, which was significantly higher compared with the benign lesions.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / pathology. Apoptosis. Cell Cycle. Gene Expression Regulation, Neoplastic. Neoplasm Recurrence, Local / genetics. Pheochromocytoma / diagnosis. Pheochromocytoma / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Biomarkers, Tumor / metabolism. Diagnosis, Differential. Female. Humans. Ki-67 Antigen / genetics. Ki-67 Antigen / metabolism. Male. Middle Aged. Necrosis / diagnosis. Necrosis / genetics. Necrosis / pathology. Predictive Value of Tests. Prognosis. Prospective Studies. Risk Factors. Severity of Illness Index. Single-Blind Method


69. Gonias S, Goldsby R, Matthay KK, Hawkins R, Price D, Huberty J, Damon L, Linker C, Sznewajs A, Shiboski S, Fitzgerald P: Phase II study of high-dose [131I]metaiodobenzylguanidine therapy for patients with metastatic pheochromocytoma and paraganglioma. J Clin Oncol; 2009 Sep 1;27(25):4162-8
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  • [Title] Phase II study of high-dose [131I]metaiodobenzylguanidine therapy for patients with metastatic pheochromocytoma and paraganglioma.
  • PURPOSE: To evaluate the safety and efficacy of high-dose [(131)I]metaiodobenzylguanidine ([(131)I]MIBG) in the treatment of malignant pheochromocytoma (PHEO) and paraganglioma (PGL).

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  • (PMID = 19636009.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] ENG
  • [Grant] United States / NCRR NIH HHS / RR / UL1 RR024131; United States / NCRR NIH HHS / RR / 2MO1 RR0127
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Catecholamines; 0 / Chromogranin A; 0 / Iodine Radioisotopes; 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine
  • [Other-IDs] NLM/ PMC2734428
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70. 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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  • [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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71. 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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72. 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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73. 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


74. 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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75. 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


76. 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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  • [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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77. 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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78. 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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79. 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


80. Donckier JE, Michel L: Phaeochromocytoma: state-of-the-art. Acta Chir Belg; 2010 Mar-Apr;110(2):140-8
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  • [Title] Phaeochromocytoma: state-of-the-art.
  • Phaeochromocytomas are catecholamine-secreting tumours that arise from chromaffin cells of the adrenal medulla and extra-adrenal sites.
  • Extra-adrenal phaeochromocytomas are called paragangliomas.
  • A diagnosis of phaeochromocytoma is suspected by typical paroxysmal symptoms, unusual or refractory hypertension, discovery of an adrenal incidentaloma or a family history of phaeochromocytoma or paraganglioma, possibly associated with other genetic syndromes (multiple endocrine neoplasia type 2 A or B, neurofibromatosis type 1 and von Hippel-Lindau disease).
  • Increased serum chromogranin-A levels, combined with high catecholamine or metanephrine in a patient with normal renal function is also a tool, virtually diagnostic of phaeochromocytoma.
  • Recent studies have suggested that 25% of patients with phaeochromocytoma have germline mutations of several genes (NF1, VHL, SDHD, SDHB and RET).
  • Once a biochemical diagnosis of phaeochromocytoma is made, a CT scan or MRI of the abdomen and pelvis should be performed first.
  • After localization, the treatment of phaeochromocytoma is a surgical resection, which may be laparoscopic.
  • Malignant phaeochromocytoma is rare and its treatment still unsatisfying.
  • Phaeochromocytoma during pregnancy is also rare and its diagnosis easily missed because of its clinical resemblance to pre-eclampsia.
  • [MeSH-major] Adrenal Gland Neoplasms. Pheochromocytoma


81. 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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82. Qin ZK, Zhou FJ, Dai YP, Chen W, Hou JH, Han H, Liu ZW, Yu SL, Zhang DZ, Yang JA: [Expression and clinical significance of survivin and PTEN in adrenal tumors]. Ai Zheng; 2007 Oct;26(10):1143-7
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  • [Title] [Expression and clinical significance of survivin and PTEN in adrenal tumors].
  • BACKGROUND & OBJECTIVE: Since the histomorphology of adrenal tumor is very special, it is difficult to assess the malignancy of the tumors.
  • This study was to explore the expression and clinical significance of Survivin and PTEN proteins in adrenal tumors.
  • METHODS: The expression of Survivin and PTEN in 116 specimens of adrenal tumors, including 39 cases of cortex adenoma, 22 cases of cortex adenocarcinoma, 35 cases of pheochromocytoma, and 20 cases of malignant pheochromocytoma, were detected by LSAB immunohistochemistry.
  • The expression intensity of Survivin was correlated to that of PTEN in adrenal tumors (r=-0.486, P<0.05).
  • The expression intensity of Survivin and PTEN in adrenal tumors were not related to patient's age, sex, tumor position, and so on (P>0.05).
  • The expression intensity of Survivin was significantly lower in adrenal cortex adenoma than in adrenal cortex adenocarcinoma (P<0.05), and was significantly lower in pheochromocytoma than in malignant pheochromocytoma (P<0.05).
  • The expression intensity of PTEN was related to the differentiation of adrenal tumor.
  • The expression intensity of PTEN was significantly higher in adrenal cortex adenoma than in adrenal cortex adenocarcinoma (P<0.05), and was significantly higher in pheochromocytoma than in malignant pheochromocytoma (P<0.05).
  • The expression of Survivin protein and PTEN protein was correlated to the prognosis of adrenal cortex adenocarcinoma and malignant pheochromocytoma: the higher the expression intensity of Survivin protein and the lower the expression intensity of PTEN protein, the worse the patient's prognosis (P<0.05).
  • CONCLUSION: The expression of Survivin and PTEN proteins are closely related to the prognosis of adrenal tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Adrenocortical Adenoma / metabolism. Adrenocortical Carcinoma / metabolism. Microtubule-Associated Proteins / metabolism. PTEN Phosphohydrolase / metabolism. Pheochromocytoma / metabolism

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  • (PMID = 17927889.001).
  • [Journal-full-title] Ai zheng = Aizheng = Chinese journal of cancer
  • [ISO-abbreviation] Ai Zheng
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / BIRC5 protein, human; 0 / Inhibitor of Apoptosis Proteins; 0 / Microtubule-Associated Proteins; EC 3.1.3.48 / PTEN protein, human; EC 3.1.3.67 / PTEN Phosphohydrolase
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83. 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
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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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84. 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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85. 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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86. Ku CF, Lo CY, Chan WF, Chiu SW, Fan ST, Lam KS: Resection of phaeochromocytoma extending into the right atrium in a patient with multiple endocrine neoplasia type 2A. Hong Kong Med J; 2005 Feb;11(1):59-62
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  • [Title] Resection of phaeochromocytoma extending into the right atrium in a patient with multiple endocrine neoplasia type 2A.
  • We report the first case of successful surgical resection of a malignant phaeochromocytoma with tumour extension into vena cava and right atrium in a patient with multiple endocrine neoplasia type 2A.
  • A 21-year-old woman with genetic confirmation of multiple endocrine neoplasia type 2A syndrome was diagnosed with a very rare case of malignant phaeochromocytoma with tumour thrombus extension into vena cava and right atrium causing Budd-Chiari syndrome.
  • Reviewing the limited literature, surgical resection by means of cardiopulmonary bypass with hypothermic circulatory arrest has been reported with success in phaeochromocytoma with advance vascular involvement.


87. 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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88. 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


89. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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


90. Conlon JM: Granin-derived peptides as diagnostic and prognostic markers for endocrine tumors. Regul Pept; 2010 Nov 30;165(1):5-11
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  • However, circulating concentrations of CgA-LI are elevated in several non-neoplastic diseases and in patients receiving acid-suppression therapy which may lead to false positive diagnosis.
  • Other CgA-derived peptides with potential as tumor markers are vasostatin-1, WE-14, catestatin, GE-25, and EL-35 but their value has yet to be fully assessed.
  • Measurement of concentrations of a second secretogranin II-derived peptide, EM-66 in tumor tissue has been used to differentiate between benign and malignant pheochromocytoma.
  • These examples point to a limited although potentially valuable role for granin-derived peptides as tumor markers.
  • [MeSH-major] Chromogranins / metabolism. Endocrine Gland Neoplasms / diagnosis. Endocrine Gland Neoplasms / pathology

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  • [Copyright] Copyright © 2009 Elsevier B.V. All rights reserved.
  • (PMID = 19931574.001).
  • [ISSN] 1873-1686
  • [Journal-full-title] Regulatory peptides
  • [ISO-abbreviation] Regul. Pept.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Chromogranin A; 0 / Chromogranin B; 0 / Chromogranins; 0 / Secretogranin II
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91. 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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  • (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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92. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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


93. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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94. 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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  • [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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  • (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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95. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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


96. Young WF Jr: Endocrine hypertension: then and now. Endocr Pract; 2010 Sep-Oct;16(5):888-902
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVE: To review the first reported cases of successfully treated pheochromocytoma and primary aldosteronism and to document the diagnostic and therapeutic advances that have occurred since the initial descriptions.
  • RESULTS: The successful management of the initial cases of pheochromocytoma in 1926 and primary aldosteronism in 1954 was highlighted by keen clinical observation, clinical intuition, and application of scientific principles.
  • Since those prismatic case descriptions, the technological advances in laboratory-based diagnosis, radiology-based tumor localization, and surgical approaches to the adrenal glands have been truly remarkable.
  • CONCLUSIONS: The evolution in the diagnosis and treatment of pheochromocytoma will continue to progress as we identify more genetic causes, develop biochemical markers for "preclinical" pheochromocytoma, identify better markers for malignant disease, and develop more effective treatment options for malignant pheochromocytoma.
  • [MeSH-minor] Adrenal Gland Neoplasms / complications. Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Adult. Endocrine System / physiopathology. Female. History, 20th Century. History, 21st Century. Humans. Pheochromocytoma / complications. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy

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  • (PMID = 20713331.001).
  • [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; Historical Article; Journal Article; Review
  • [Publication-country] United States
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97. Hanna-Moussa A, Kurukulasuriya LR, Sowers JR: Malignant pheochromocytoma presenting with uncontrolled hypertension after kidney transplant. J Clin Hypertens (Greenwich); 2010 Feb 1;12(2):105-8
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  • [Title] Malignant pheochromocytoma presenting with uncontrolled hypertension after kidney transplant.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Hypertension / diagnosis. Kidney Transplantation. Pheochromocytoma / complications


98. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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


99. 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


100. 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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