[X] Close
You are about to erase all the values you have customized, search history, page format, etc.
Click here to RESET all values       Click here to GO BACK without resetting any value
Items 1 to 12 of about 12
1. He J, Makey D, Fojo T, Adams KT, Havekes B, Eisenhofer G, Sullivan P, Lai EW, Pacak K: Successful chemotherapy of hepatic metastases in a case of succinate dehydrogenase subunit B-related paraganglioma. Endocrine; 2009 Oct;36(2):189-93
Hazardous Substances Data Bank. VINCRISTINE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Successful chemotherapy of hepatic metastases in a case of succinate dehydrogenase subunit B-related paraganglioma.
  • Compared to other familial pheochromocytoma/paragangliomas (PHEO/PGLs), the succinate dehydrogenase subunit B (SDHB)-related PHEO/PGLs often present with aggressive and rapidly growing metastatic lesions.
  • Currently, there is no proven effective treatment for malignant PHEO/PGLs.
  • Here, we present a 35-year-old white man with primary malignant abdominal extra-adrenal 11 cm paraganglioma underwent surgical successful resection.
  • But 6 months later, he developed extensive bone, liver, and lymph nodes metastasis, which were demonstrated by computed tomography scan and the (18)F-fluorodeoxyglucose positron emission tomography.
  • However, his (123)I-metaiodobenzylguanidine scintigraphy was negative; therefore, the cyclophosphamide, vincristine, and dacarbazine (CVD) combination chemotherapy was initiated.
  • The combination chemotherapy was very effective showing 80% overall reduction in the liver lesions and 75% overall reduction in the retroperitoneal mass and adenopathy, and normalization of plasma catecholamine and metanephrine levels.
  • Here, we present an SDHB-related PHEO/PGL patient with extensive tumor burden, numerous organ lesions, and rapidly growing tumors, which responded extremely well to CVD therapy.
  • We conclude patients with SDHB-related PHEO/PGLs can be particularly sensitive to CVD chemotherapy and may have an excellent outcome if this therapy is used and continued on periodic basis.
  • The data in this patient also illustrate the importance of measuring plasma levels of DA and MTY to provide a more complete and accurate assessment of the biochemical response to therapy than provided by measurements restricted to other catecholamines and O-methylated metabolites.
  • [MeSH-major] Abdominal Neoplasms / drug therapy. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Liver Neoplasms / drug therapy. Paraganglioma, Extra-Adrenal / drug therapy
  • [MeSH-minor] Adult. Combined Modality Therapy. Cyclophosphamide / therapeutic use. Dacarbazine / therapeutic use. Humans. Male. Succinate Dehydrogenase / genetics. Treatment Outcome. Vincristine / therapeutic use

  • MedlinePlus Health Information. consumer health - Liver Cancer.
  • Hazardous Substances Data Bank. DACARBAZINE .
  • Hazardous Substances Data Bank. CYCLOPHOSPHAMIDE .
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 19618298.001).
  • [ISSN] 1559-0100
  • [Journal-full-title] Endocrine
  • [ISO-abbreviation] Endocrine
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 HD008735-08
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7GR28W0FJI / Dacarbazine; 8N3DW7272P / Cyclophosphamide; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase; CVD protocol
  • [Other-IDs] NLM/ NIHMS750174; NLM/ PMC4713025
  •  go-up   go-down


2. Gómez Esteban JC, Boyero S, Fernández C, Sagasta A, Pérez T, Velasco F, Allue I, Lezcano E, Zarranz JJ: [Baroreflex failure after chemodectoma resection]. Neurologia; 2004 Oct;19(8):452-5
Hazardous Substances Data Bank. CLONIDINE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Baroreflex failure after chemodectoma resection].
  • [Transliterated title] Fallo barorreflejo tras intervención de un quemodectoma.
  • Baroreflex failure after chemodectoma resection We present a case of baroreflex failure secondary to a unilateral injury of the glossopharyngeal nerve.
  • The patient was operated for a left-sided chemodectoma in the carotid body.
  • The diagnosis was baroreflex failure secondary to unilateral injury of the glossopharyngeal nerve.
  • The case reported herein illustrates the fact that the presence of a bilateral injury is not essential for the occurrence of this disorder.
  • [MeSH-major] Baroreflex. Glossopharyngeal Nerve Injuries. Neurosurgical Procedures / adverse effects. Paraganglioma, Extra-Adrenal / surgery
  • [MeSH-minor] Aged. Angiography. Antihypertensive Agents / therapeutic use. Autonomic Nervous System Diseases / etiology. Blood Pressure / drug effects. Clonidine / pharmacology. Female. Humans. Hypertension / drug therapy

  • Genetic Alliance. consumer health - Baroreflex Failure.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 15470587.001).
  • [ISSN] 0213-4853
  • [Journal-full-title] Neurología (Barcelona, Spain)
  • [ISO-abbreviation] Neurologia
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Antihypertensive Agents; MN3L5RMN02 / Clonidine
  •  go-up   go-down


3. Pacheco-Ojeda L: Malignant carotid body tumors: report of three cases. Ann Otol Rhinol Laryngol; 2001 Jan;110(1):36-40

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The first was in a 40-year-old man who presented with pulmonary metastases 6 years after resection of a CBT.
  • He was treated with chemotherapy and interferon, but died with disseminated disease 2 years later.
  • This lesion was completely resected, and a shunt and reconstruction with a saphenous vein graft were performed.
  • Pathology revealed a malignant chemodectoma with invasion to 2 of the 5 lymph nodes removed.
  • Radiotherapy (50 Gy) was given after the operation.
  • She is well and free of disease 68 months after the resection.
  • On computed tomography, the tumor extended to the infratemporal fossa with no bone involvement.
  • The lesion was embolized with a 40% reduction in vascularity.
  • At surgical exploration, the tumor involved the sternocleidomastoid muscle and the lymph nodes at levels II and III, and the internal carotid artery could not be dissected free at the skull base, so only a partial resection was performed.
  • Current multidisciplinary treatments have been unsuccessful in controlling disseminated disease.
  • [MeSH-major] Carotid Body Tumor. Paraganglioma, Extra-Adrenal

  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 11201806.001).
  • [ISSN] 0003-4894
  • [Journal-full-title] The Annals of otology, rhinology, and laryngology
  • [ISO-abbreviation] Ann. Otol. Rhinol. Laryngol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 38
  •  go-up   go-down


Advertisement
4. Kavakli K, Ozturk M, Ongoru O, Gürkök S, Genc O: Primary pulmonary paraganglioma with Hodgkin's lymphoma. Thorac Cardiovasc Surg; 2009 Sep;57(6):375-7
MedlinePlus Health Information. consumer health - Lung Cancer.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary pulmonary paraganglioma with Hodgkin's lymphoma.
  • Extra-adrenal paragangliomas are uncommon tumors arising from neuroectodermal-derived paraganglionic tissue.
  • There are very few case reports on primary pulmonary paraganglioma in the literature.
  • We present the case of a 21-year-old man who was treated with chemotherapy for Hodgkin's lymphoma.
  • Only a partial response was noted so he underwent additional chemotherapy and involved field radiotherapy.
  • Subsequent bronchoscopic biopsy showed paraganglioma and the patient underwent a left lower lobectomy.
  • [MeSH-major] Hodgkin Disease / diagnosis. Lung Neoplasms / diagnosis. Paraganglioma, Extra-Adrenal / diagnosis
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols. Biopsy. Bronchoscopy. Chemotherapy, Adjuvant. Humans. Lymph Node Excision. Male. Pneumonectomy. Positron-Emission Tomography. Radiotherapy, Adjuvant. Tomography, X-Ray Computed. Treatment Outcome. Young Adult

  • MedlinePlus Health Information. consumer health - Hodgkin Disease.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • [Copyright] Georg Thieme Verlag KG Stuttgart New York.
  • (PMID = 19707986.001).
  • [ISSN] 1439-1902
  • [Journal-full-title] The Thoracic and cardiovascular surgeon
  • [ISO-abbreviation] Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 7
  •  go-up   go-down


5. Leshnower BG, Morris RJ, Pechet TT: Management of an anterior mediastinal pheochromocytoma causing tracheomalacia. Ann Thorac Surg; 2007 Dec;84(6):2088-90
MedlinePlus Health Information. consumer health - Tracheal Disorders.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Thoracic paragangliomas are rare tumors that arise from extra-adrenal chromaffin cells and have the capacity to secrete catecholamines.
  • Surgical excision is the optimal treatment of these tumors as they are resistant to chemotherapy and radiation therapy.
  • In this report we describe the treatment of an anterior mediastinal pheochromocytoma that presented with tracheal obstruction and required pulmonary artery reconstruction and airway stenting.

  • Genetic Alliance. consumer health - Pheochromocytoma.
  • MedlinePlus Health Information. consumer health - Pheochromocytoma.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 18036944.001).
  • [ISSN] 1552-6259
  • [Journal-full-title] The Annals of thoracic surgery
  • [ISO-abbreviation] Ann. Thorac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
  •  go-up   go-down


6. Lehmen JA, Babbel DM, Mikhitarian K, Choma TJ: Paraganglioma presenting as metastatic lesion in a cervical vertebra: a case report and review of the literature. Spine (Phila Pa 1976); 2010 Mar 1;35(5):E152-4
NCI CPTAC Assay Portal. NCI CPTAC Assay Portal .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Paraganglioma presenting as metastatic lesion in a cervical vertebra: a case report and review of the literature.
  • OBJECTIVE: To discuss the spinal presentation and treatment of paraganglioma, a rare tumor of the extra-adrenal paraganglia, derived from neural crest cells in sympathetic ganglions.
  • SUMMARY OF BACKGROUND DATA: Malignant paragangliomas are a very rare entity.
  • The diagnosis of malignant paraganglioma is made by local recurrence after total resection of the primary mass, or findings of distant metastases.
  • Our imaging workup, including plain radiograph, computerized tomography scan and MRI, revealed an intraosseous lesion at C6 with vertebral body destruction.
  • The histologic and immunohistochemical assays revealed a malignant paraganglioma.
  • RESULTS: Paragangliomas are often highly vascular tumors.
  • CONCLUSION: We report a rare case of malignant paraganglioma and discussed adjuvant treatments to consider for distant metastases, such as I-MIBG, conventional radiotherapy, and chemotherapy.
  • [MeSH-major] Cervical Vertebrae. Neck Pain / etiology. Paraganglioma / diagnosis. Spinal Neoplasms / diagnosis
  • [MeSH-minor] Aged. Decompression, Surgical. Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male. Treatment Outcome

  • MedlinePlus Health Information. consumer health - Neck Injuries and Disorders.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 20118832.001).
  • [ISSN] 1528-1159
  • [Journal-full-title] Spine
  • [ISO-abbreviation] Spine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  •  go-up   go-down


7. Plouin PF, Amar L, Lepoutre C: Phaeochromocytomas and functional paragangliomas: clinical management. Best Pract Res Clin Endocrinol Metab; 2010 Dec;24(6):933-41
MedlinePlus Health Information. consumer health - Pheochromocytoma.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Phaeochromocytomas and functional paragangliomas: clinical management.
  • Phaeochromocytomas (PH) and functional paragangliomas (FPGL) are neoplasms of adrenal (PH) or extra-adrenal (FPGL) chromaffin tissue that synthesize catecholamines.
  • Catecholamines are converted into inactive metabolites, metanephrines, within the tumour and the diagnosis of PH/FPGL is therefore based on the quantification of plasma or urinary metanephrines.
  • The tumour can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine scintigraphy.
  • Patients with PH/FPGL should be followed up indefinitely as the disease may recur, particularly if they have inherited or extra-adrenal tumours.
  • Recurrences and malignancy are more frequent in cases with large or extra-adrenal tumours, and in SDHB mutation carriers.
  • Treatments for progressive malignant PH/FPGL include tumour debulking, metabolic radiotherapy, chemotherapy, and possibly tyrosine kinase inhibitors.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Paraganglioma / diagnosis. Paraganglioma / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy

  • MedlinePlus Health Information. consumer health - Adrenal Gland Cancer.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • [Copyright] 2010 Elsevier Ltd. All rights reserved.
  • (PMID = 21115162.001).
  • [ISSN] 1878-1594
  • [Journal-full-title] Best practice & research. Clinical endocrinology & metabolism
  • [ISO-abbreviation] Best Pract. Res. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Netherlands
  •  go-up   go-down


8. Ilias I, Pacak K: Diagnosis and management of tumors of the adrenal medulla. Horm Metab Res; 2005 Dec;37(12):717-21
The Lens. Cited by Patents in .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Diagnosis and management of tumors of the adrenal medulla.
  • The adrenal medulla consists of chromaffin cells, the site of catecholamine biosynthesis.
  • Pheochromocytomas are chromaffin-cell tumors; 80-85 % arise from the adrenal medulla and 15-20 % arise from extra-adrenal chromaffin tissues (paragangliomas).
  • Pheochromocytomas account for 6.5 % of incidentally discovered adrenal tumors; they are found in 50 % of patients with multiple endocrine neoplasia 2A (MEN 2A) and 5-25 % of patients with von Hippel-Lindau (VHL) syndrome.
  • Neuroblastomas are the most common solid extra-cranial tumors in children, and account for 7-10 % of all tumors.
  • The diagnosis of pheochromocytoma should first be established biochemically by measuring plasma free metanephrines (the measurement of urinary fractionated metanephrines is the second choice).
  • Anatomical (radiological) imaging with computed tomography (CT) or magnetic resonance imaging (MRI) is necessary for both pheochromocytomas and neuroblastomas.
  • Scintigraphy with [123I]-metaiodobenzylguanidine is the specific functional imaging test of first choice; if this is not available, scintigraphy with [131I]-MIBG is the second choice.
  • Other newer specific modalities that have been used for evaluating pheochromocytomas include positron emission tomography (PET) with [18F]-F-fluorodopamine (F-DA) and [18F]-F-dihydroxyphenylalanine (DOPA).
  • Primary treatment for both types of tumor is surgical; chemotherapy is used for inoperable disease.
  • Depending on the extent of disease and age, patients with neuroblastomas have cure rates of 15-90 %.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Adrenal Medulla / physiopathology. Neuroendocrine Tumors / diagnosis. Neuroendocrine Tumors / therapy
  • [MeSH-minor] Humans. Multiple Endocrine Neoplasia Type 2a / diagnosis. Multiple Endocrine Neoplasia Type 2a / therapy. Neuroblastoma / diagnosis. Neuroblastoma / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy. Prognosis

  • MedlinePlus Health Information. consumer health - Adrenal Gland Cancer.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 16372223.001).
  • [ISSN] 0018-5043
  • [Journal-full-title] Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et métabolisme
  • [ISO-abbreviation] Horm. Metab. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 39
  •  go-up   go-down


9. Oleaga A, Goñi F: Pheochromocytoma: diagnostic and therapeutic update. Endocrinol Nutr; 2008 May;55(5):202-16

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pheochromocytoma: diagnostic and therapeutic update.
  • Pheochromocytomas are catecholamine-secreting tumors that arise from chromaffin cells of the sympathetic nervous system.
  • In 80-85% of cases, these tumors are located in the adrenal medulla while the remainder is located in extra-adrenal chromaffin tissues (paragangliomas).
  • Pheochromocytomas account for 6.5% of incidentally discovered adrenal tumors.
  • These tumors may be sporadic or the result of several genetic diseases: multiple endocrine neoplasia type 2, von Hippel-Lindau syndrome, neurofibromatosis type 1, and familial paraganglioma associated with mutations in succinate dehydrogenase subunits.
  • Diagnosis of pheochromocytoma should first be established biochemically by measuring plasma free metanephrines and urinary fractionated metanephrines.
  • The radiological imaging tests of choice are computed tomography (CT) or magnetic resonance imaging (MRI).
  • Positron emission tomography (PET) with (18)F-F-fluorodopamine (F-DA) is useful in metastatic disease.
  • The treatment of choice is laparoscopic surgery after adequate alpha adrenergic blockade.
  • Chemotherapy is used for inoperable disease.
  • Prognosis is good except in malignant disease, in which 5-year survival is less than 50%.

  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • [Copyright] Copyright © 2008 Sociedad Española de Endocrinología y Nutrición. Published by Elsevier Espana. All rights reserved.
  • (PMID = 22967914.001).
  • [ISSN] 1575-0922
  • [Journal-full-title] Endocrinología y nutrición : órgano de la Sociedad Española de Endocrinología y Nutrición
  • [ISO-abbreviation] Endocrinol Nutr
  • [Language] eng; spa
  • [Publication-type] Journal Article
  • [Publication-country] Spain
  •  go-up   go-down


10. Joynt KE, Moslehi JJ, Baughman KL: Paragangliomas: etiology, presentation, and management. Cardiol Rev; 2009 Jul-Aug;17(4):159-64
The Lens. Cited by Patents in .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Paragangliomas: etiology, presentation, and management.
  • Paragangliomas are catecholamine-secreting tumors arising from the chromaffin cells of the sympathetic ganglia, and are known as extra-adrenal pheochromocytomas.
  • Diagnosis is made by serum and urine analysis for catecholamines and metanephrines, and confirmed with imaging studies including computed tomography scanning, magnetic resonance imaging, or 123-I metaiodobenzylguanidine imaging.
  • Although the majority of paragangliomas are sporadic, a growing percentage of cases are found to be part of a familial genetic syndrome.
  • Genetic testing should be offered to patients diagnosed with paraganglioma, particularly in patients who are young, have multiple tumors, or have a family history of malignancy.
  • Management of paraganglioma is predicated on surgical resection, and careful perioperative management with alpha- and beta-adrenergic blockade is imperative for optimal outcomes.
  • The majority of these tumors are benign, but for patients with malignant disease, chemotherapy, and radiation therapy may provide modest benefit.
  • Long-term follow-up is essential, as paragangliomas can recur many years after initial diagnosis.
  • Ongoing research into the genetic underpinnings of this tumor may allow for more targeted molecular therapies in the future.
  • [MeSH-major] Adrenal Gland Neoplasms / therapy. Paraganglioma, Extra-Adrenal / therapy. Pheochromocytoma / therapy

  • MedlinePlus Health Information. consumer health - Adrenal Gland Cancer.
  • MedlinePlus Health Information. consumer health - Pheochromocytoma.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 19525677.001).
  • [ISSN] 1538-4683
  • [Journal-full-title] Cardiology in review
  • [ISO-abbreviation] Cardiol Rev
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 45
  •  go-up   go-down


11. Sawhney SA, Chapman AD, Carney JA, Gomersall LN, Dempsey OJ: Incomplete Carney triad--a review of two cases. QJM; 2009 Sep;102(9):649-53
Hazardous Substances Data Bank. IMATINIB MESYLATE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 'Carney's triad' characteristically includes multifocal pulmonary chondroma, gastric stromal sarcoma and extra-adrenal paraganglioma.
  • [MeSH-major] Chondroma / diagnosis. Gastrointestinal Stromal Tumors / diagnosis. Liver Neoplasms / diagnosis. Lung Neoplasms / diagnosis. Paraganglioma, Extra-Adrenal / diagnosis
  • [MeSH-minor] Adult. Anti-Bacterial Agents / therapeutic use. Antineoplastic Agents / therapeutic use. Benzamides. Bronchial Diseases / drug therapy. Bronchial Diseases / etiology. Female. Humans. Imatinib Mesylate. Neoplasms, Multiple Primary / diagnosis. Piperazines / therapeutic use. Pyrimidines / therapeutic use. Tomography, X-Ray Computed

  • MedlinePlus Health Information. consumer health - Liver Cancer.
  • MedlinePlus Health Information. consumer health - Lung Cancer.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 19561114.001).
  • [ISSN] 1460-2393
  • [Journal-full-title] QJM : monthly journal of the Association of Physicians
  • [ISO-abbreviation] QJM
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate
  •  go-up   go-down


12. Horenstein MG, Hitchcock TA, Tucker JA: Dual CD117 expression in gastrointestinal stromal tumor (GIST) and paraganglioma of Carney triad: a case report. Int J Surg Pathol; 2005 Jan;13(1):87-92
MedlinePlus Health Information. consumer health - Lung Cancer.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Dual CD117 expression in gastrointestinal stromal tumor (GIST) and paraganglioma of Carney triad: a case report.
  • Carney triad is a rare syndrome, with only 20 complete cases reported.
  • We report a 36-year-old white woman with complete Carney triad, including metastatic gastric stromal tumor (GIST), pulmonary chondroma, and nonfunctioning extra-adrenal paraganglioma.
  • Immunohistochemistry was positive for CD34 and CD117 (c-kit) in the GIST, and positive for chromogranin and CD117 in the paraganglioma.
  • To our knowledge, this is the 21st complete Carney triad case reported and the first report of dual expression CD117 in both GIST and paraganglioma, a finding with intriguing pathogenetic implications related to the organization of the autonomic nervous system.
  • [MeSH-major] Chondroma / pathology. Gastrointestinal Stromal Tumors / pathology. Leiomyosarcoma / secondary. Lung Neoplasms / pathology. Paraganglioma, Extra-Adrenal / pathology. Proto-Oncogene Proteins c-kit / analysis
  • [MeSH-minor] Adult. Antigens, CD34 / analysis. Antineoplastic Agents / therapeutic use. Biomarkers, Tumor / analysis. Chromogranins / analysis. Drug Therapy. Female. Humans. Palliative Care. Syndrome

  • Genetic Alliance. consumer health - Gastrointestinal Stromal Tumors.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 15735861.001).
  • [ISSN] 1066-8969
  • [Journal-full-title] International journal of surgical pathology
  • [ISO-abbreviation] Int. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD34; 0 / Antineoplastic Agents; 0 / Biomarkers, Tumor; 0 / Chromogranins; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
  •  go-up   go-down






Advertisement