Abstract
A 15 year old boy was admitted to our department because of intractable diarrhea, flushes and pulmonary restriction over the last 4 years. Final diagnosis was Multiple Endocrine Neoplasia Type IIb (mucosal neuromas, MTC, marfan-like habitus) with pulmonary and lymphatic metastases of MTC secreting excessive amounts of calcitonin (CT: 150 ng/ml, normal: 300 pg/ml, normal: undetectable) and carcino-embryonic antigen (CEA: 150-223 ng/ml, normal: <10 ng/ml). In adults, the long-acting somatostatin analog has been shown to suppress hormonal hypersecretion in many endocrine tumors including carcinoid syndromes and vipomas. Since no effective therapy of metastatic MTC is available so far, we used SMS 201 - 995 in an attempt to control diarrhea and tumor growth. During therapy with s.c. injections the boy experienced clinical improvement by decrease of stool frequency from 4 - 9 to 2 - 4 daily. VIP and GRP fell toward the normal range (9.94 pM and 15 pg/ml, respectively). However, CT and CEA did not change at all. Endocrine side effects were an inhibition of insulin and HGH secretion. SMS 201 - 995 appears to be a promising agent in the symptomatic therapy of intractable diarrhea. The decrease of VIP and GRP might indicate a partial inhibition of tumor activity and growth.
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