METASTATIC NEUROENDOCRINE ANAPLASTIC SMALL-CELL TUMOR IN A PATIENT WITH MULTIPLE ENDOCRINE NEOPLASIA TYPE-1 SYNDROME - ASSESSMENT OF DISEASE STATUS AND RESPONSE TO DOXORUBICIN, CYCLOPHOSPHAMIDE, ETOPOSIDE CHEMOTHERAPY THROUGH SCINTIGRAPHIC IMAGING WITH IN-111-PENTETREOTIDE

Extrapulmonary small cell and small cell neuroendocrine tumors of unkn own primary site are, in general, aggressive neoplasms with a short me dian survival. Like small cell lung cancer (SCLC), they often are resp onsive to chemotherapy and radiotherapy. Small cell lung cancer and we ll differentiated neuroendocrine carcinomas of the gastrointestinal tr act and pancreas tend to express somatostatin receptors. These tumors may be localized in patients by scintigraphic imaging using radiolabel ed somatostatin analogues. A patient with an anaplastic neuroendocrine small cell tumor arising on a background of multiple endocrine neopla sia type 1 syndrome is reported. The patient had a known large pancrea tic gastrinoma and previously treated parathyroid adenopathy. At prese ntation, there was small cell cancer throughout the liver and skeleton . Imaging with a radiolabeled somatostatin analogue, In-111-pentetreot ide (Mallinckrodt Medical B. V., Petten, Holland), revealed all sites of disease detected by routine biochemical and radiologic methods. Aft er six cycles of chemotherapy with doxorubicin, cyclophosphamide, and etoposide, there was almost complete clearance of the metastatic disea se. In-111-pentetreotide scintigraphy revealed uptake consistent with small areas of residual disease in the liver, the abdomen (in mesenter ic lymph nodes), and posterior thorax (in a rib). The primary gastrino ma present before the onset of the anaplastic small cell cancer showed no evidence of response to the treatment. The patient remained well f or 1 year and then relapsed with brain, lung, liver, and skeletal meta stases. Despite an initial response to salvage radiotherapy and chemot herapy with carboplatin and dacarbazine, the patient died 6 months lat er.