[Composite pheochromocytoma associated with multiple endocrine neoplasia type 2B].
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.
Charfi
Slim
S
Laboratoire d'anatomie et de cytologie pathologique, CHU Habib-Bourguiba, rue El-Ain, 3029 Sfax, Tunisia.
Ayadi
Lobna
L
Ellouze
Sameh
S
Ghorbel
Raoudha
R
Khabir
Abdelmajid
A
Gouiaa
Naourez
N
Bahri
Ibticem
I
Fakhfakh
Ines
I
Makni
Salwa
S
Sellami-Boudawra
Tahya
T
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