[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.