AI Article Synopsis
- Composite pheochromocytoma (CP) is a rare tumor found in the adrenal glands, combining elements of pheochromocytoma and neuroblastoma, with limited information available about it.
- A case study detailed a 56-year-old woman with CP containing a ganglioneuroma, contributing to a literature review that identified 110 total cases, with a median age of 51.5 and a majority being female.
- Genetic syndromes related to CP were found in 20% of cases, with the most common being neurofibromatosis type 1, and the review emphasized that most neuroblastic components were ganglioneuromas, while only a small percentage of patients experienced metastasis or
Article Abstract
Composite pheochromocytoma (CP) is a rare adrenal tumor, composed of ordinary pheochromocytoma and neuroblastic components. There is a paucity of information in the literature regarding this entity. We report the case of a 56-year-old woman with a CP of the left adrenal gland with a ganglioneuroma component. A review of the published literature found 110 cases of CP. The median age was 51.5 (5.86) years, and 59/110 (53.6%) were female. Association with genetic predisposition syndromes was found in 22/110 (20%), the most common of which was neurofibromatosis type 1, in 15/110 (13.6%). The most common histologic type of the neuroblastic component was ganglioneuroma in 83/110 (75.5%). Twenty-seven cases reported SDHB immunohistochemistry results; none of which was positive. Nine patients (8.2%) presented/developed metastatic disease, and 9 patients (8.2%) died from disease. To our knowledge, this is the largest review describing clinical, histopathological, molecular, and prognostic features of CP.
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| http://dx.doi.org/10.1007/s00428-023-03492-y | DOI Listing |
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