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Multiple mucosal neuromata with endocrine tumors syndrome (multiple mucosal neuromas, pheochromocytoma, and medullary thyroid carcinoma syndrome)


Multiple Mucosal Neuromata with Endocrine Tumors Syndrome (Multiple Mucosal Neuromas, Pheochromocytoma, and Medullary Thyroid Carcinoma Syndrome)

General: Pheochromocytoma can be inherited and found in combination with neurofibromatosis and other brain tumors (meningioma, spongioblastoma, ependymoma, astrocytoma, cerebellar hemangioblastoma), multiple mucosal neuromas, and medullary carcinoma of the thyroid.

Ocular: Conjunctival neuromas; multiple, white, myelinated nerve fibers in corneal stroma arising at limbus with anastomoses in center of cornea.



Clinical: Neuromas of lips or anterior portion of tongue or, more rarely, of buccal, gingival, or laryngeal mucosa; hypertension; flushing; weakness; sweating; palpitations; headaches.

Burgdorf W. Letter: multiple mucosal neuroma syndrome. Arch Dermatol 1975; 111:931.

Paloyan E, et al. familial pheochromocytoma, medullary thyroid carcinoma and parathyroid adenomas. JAMA 1970; 214:1443.

Williams ED, Pollock DJ. Multiple mucosal neuromata with endocrine tumors: a syndrome allied to von Recklinghausen's disease. J Pathol Bacteriol 1966; 91:71.




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