Type 2b multiple endocrine adenomatosis
Lehnert, H.; Hogan, M.; Schrezenmeir, J.; Beyer, J.
Schweizerische Rundschau für Medizin Praxis 78(26): 741-744
1989
ISSN/ISBN: 1013-2058 PMID: 2569226 Document Number: 343505
The case report presented here demonstrates the typical diagnostic and therapeutic problems in a young patient with a multiple endocrine adenomatosis type 2b. The patient exhibited the complete syndrome with its endocrine and non-endocrine organ manifestations (medullary thyroid cancer, bilateral pheochromocytoma, mucosal neuromas, marfanoid habitus, pes equinus, diverticulosis). First presenting symptom was a diverticulitis that led to an emergency surgical intervention because of perforation of the sigmoid colon. Perioperatively, blood pressure increases were noted that were then followed by further diagnostic measures leading to adrenalectomy, thyroidectomy and surgery for local metastases of the thyroid cancer. Family screening demonstrates so far a possible sporadic form of the disease.