| Composite pheochromocytoma-ganglioneuroma: a rare experiment of nature. | |
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MedLine Citation:
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PMID: 19833581 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: To present a rare case of composite pheochromocytoma-ganglioneuroma (Pheo-GN) of the adrenal medulla, review the related literature, and discuss the clinical features, pathologic findings, behavior, and management of such tumors. METHODS: A case report of a patient with composite Pheo-GN of the adrenal gland is presented. Using the online database PUBMED, we searched and analyzed all cases of composite pheochromocytoma reported in the English-language literature during the past 70 years. RESULTS: On computed tomography, a 61-year-old man was incidentally found to have a 3.8-cm nonadenomatous right adrenal lesion. Adrenalectomy revealed a 5-cm mass consistent with composite Pheo-GN. To date, 45 cases of composite pheochromocytomas have been reported during the past 70 years, 71% of which coexisted with ganglioneuromas. These tumors occurred with approximately equal frequency in male and female patients, the majority of whom were from 40 to 60 years old. Only 14 cases have been reported in the United States. Bilateral tumors were found in 3 cases. The mean size was 4 to 6 cm. Preoperatively, functional evidence was found in 76.3% of all composite pheochromocytomas (and in 67% of Pheo-GN). Only one Pheo-GN was found to have liver metastatic lesions at the time of autopsy; the rest were not aggressive. CONCLUSION: To our knowledge, this is the first literature review describing the characteristics and behavior of all reported cases of composite pheochromocytomas, with an emphasis on those with ganglioneuromas. Composite pheochromocytoma is a rare variant of a relatively uncommon disease diagnosed by pathologists only. Fortunately, the treatment of such an entity remains the same as for any pheochromocytoma. |
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Authors:
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Amna N Khan; Solomon S Solomon; Richard Dale Childress |
Publication Detail:
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Type: Case Reports; Journal Article; Review |
Journal Detail:
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Title: Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists Volume: 16 ISSN: 1934-2403 ISO Abbreviation: Endocr Pract Publication Date: 2010 Mar-Apr |
Date Detail:
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Created Date: 2010-03-30 Completed Date: 2010-06-24 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9607439 Medline TA: Endocr Pract Country: United States |
Other Details:
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Languages: eng Pagination: 291-9 Citation Subset: IM |
Affiliation:
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Department of Medicine, Section of Endocrinology, Tulane University, New Orleans, Louisiana 70112, USA. amnakhan@tulane.edu |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Ganglioneuroma
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diagnosis*,
pathology Humans Male Middle Aged Pheochromocytoma / diagnosis*, pathology |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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