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- Title
Bilateral pheochromocytoma with ganglioneuroma component associated with multiple neuroendocrine neoplasia type 2A: a case report.
- Authors
Efared, Boubacar; Atsame-Ebang, Gabrielle; Tahirou, Soufiane; Mazaz, Khalid; Hammas, Nawal; El Fatemi, Hinde; Chbani, Laila
- Abstract
<bold>Background: </bold>Composite pheochromocytoma/paragangliomas are very rare tumors composed of ordinary pheochromocytoma paragangliomas associated with neurogenic tumors. Several hereditary susceptibility disorders are known to be associated with pheochromocytoma/paragangliomas such as multiple endocrine neoplasia type 2 (2A or B). To the best of our knowledge, only four cases of composite pheochromocytoma/paragangliomas associated with multiple endocrine neoplasia type 2 have been reported.<bold>Case Presentation: </bold>A 40-year-old Arabic woman presented with headache, palpitations, paroxysmal hypertension, and weight loss, which she had had for the last 3 years. She had a familial history of diabetes and multiple endocrine neoplasia type 2. A radiological examination revealed thyroid lesions and bilateral adrenal medulla tumors. Our patient had undergone bilateral adrenalectomy, total thyroidectomy with cervical lymphadenectomy, and parathyroidectomy. A pathological examination confirmed the multiple endocrine neoplasia type 2A consisting of left medullary pheochromocytoma, right medullary composite pheochromocytoma-ganglioneuroma, medullary carcinoma of the thyroid with lymph node metastasis and parathyroid hyperplasia. A genetic analysis also revealed that our patient had a RET germline mutation.<bold>Conclusion: </bold>Composite pheochromocytoma/paraganglioma associated with multiple endocrine neoplasia type 2 is a very rare occurrence, as the current literature provides only a few cases. Further reported cases are needed in order to understand the behavior and the pathogenesis of this uncommon entity.
- Publication
Scandinavian Journal of Trauma, Resuscitation & Emergency Medicine, 2017, Vol 25, p1
- ISSN
1757-7241
- Publication type
journal article
- DOI
10.1186/s13256-017-1364-6