title: [Composite pheochromocytoma associated with multiple endocrine neoplasia type 2B].
abstract: Composite tumors of the adrenal medulla are rare and have been reported in both the presence and the absence of phacomatosis. Composite pheochromocytoma of the adrenal gland in multiple endocrine neoplasia 2B has not been reported so far. We report a case of a 27-year-old woman with marfanoid habitus and numerous mucosal neuromas of the oral cavity and the eyelids. Clinical investigations revealed a left adrenal medullary tumor and bilateral thyroid nodules. Histologic examination confirmed the presence of typical pheochromocytoma with large areas of ganglioneuroma and multifocal medullary carcinoma with cervical lymph nodes metastases. Our report is the first to describe composite pheochromocytoma with multiple endocrine neoplasia 2B; this report underlines the diversity of neoplasms that could be encountered in this disease and the complex mechanisms involved in its pathogenesis.
authors.affiliation: Laboratoire d'anatomie et de cytologie pathologique, CHU Habib-Bourguiba, rue El-Ain, 3029 Sfax, Tunisia.
authors.forename: Slim
authors.initials: S
authors.lastname: Charfi
authors.forename: Lobna
authors.initials: L
authors.lastname: Ayadi
authors.forename: Sameh
authors.initials: S
authors.lastname: Ellouze
authors.forename: Raoudha
authors.initials: R
authors.lastname: Ghorbel
authors.forename: Abdelmajid
authors.initials: A
authors.lastname: Khabir
authors.forename: Naourez
authors.initials: N
authors.lastname: Gouiaa
authors.forename: Ibticem
authors.initials: I
authors.lastname: Bahri
authors.forename: Ines
authors.initials: I
authors.lastname: Fakhfakh
authors.forename: Salwa
authors.initials: S
authors.lastname: Makni
authors.forename: Tahya
authors.initials: T
authors.lastname: Sellami-Boudawra
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000473:pathology* / Q000601:surgery; D000328:Adult; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008382:Marfan Syndrome / Q000473:pathology; D018814:Multiple Endocrine Neoplasia Type 2b / Q000150:complications / Q000473:pathology*; D009463:Neuroma / Q000473:pathology; D010673:Pheochromocytoma / Q000150:complications / Q000473:pathology* / Q000601:surgery