title: A 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 111In-pentetreotide.
abstract: Extrapulmonary small cell and small cell neuroendocrine tumors of unknown primary site are, in general, aggressive neoplasms with a short median survival. Like small cell lung cancer (SCLC), they often are responsive to chemotherapy and radiotherapy. Small cell lung cancer and well differentiated neuroendocrine carcinomas of the gastrointestinal tract and pancreas tend to express somatostatin receptors. These tumors may be localized in patients by scintigraphic imaging using radiolabeled somatostatin analogues. A patient with anaplastic neuroendocrine small cell tumor arising on a background of multiple endocrine neoplasia type 1 syndrome is reported. The patient had a known large pancreatic gastrinoma and previously treated parathyroid adenopathy. At presentation, there was small cell cancer throughout the liver and skeleton. Imaging with a radiolabeled somatostatin analogue, 111In-pentetreotide (Mallinckrodt Medical B. V., Petten, Holland), revealed all sites of disease detected by routine biochemical and radiologic methods. After six cycles of chemotherapy with doxorubicin, cyclophosphamide, and etoposide, there was almost complete clearance of the metastatic disease. 111In-pentetreotide scintigraphy revealed uptake consistent with small areas of residual disease in the liver, the abdomen (in mesenteric lymph nodes), and posterior thorax (in a rib). The primary gastrinoma present before the onset of the anaplastic small cell cancer showed no evidence of response to the treatment. The patient remained well for 1 year and then relapsed with brain, lung, liver, and skeletal metastases. Despite an initial response to salvage radiotherapy and chemotherapy with carboplatin and dacarbazine, the patient died 6 months later.
authors.affiliation: Department of Clinical Medicine, St. James's Hospital and Trinity College, Dublin, Ireland.
authors.forename: K J
authors.initials: KJ
authors.lastname: O'Byrne
authors.forename: M G
authors.initials: MG
authors.lastname: Goggins
authors.forename: G S
authors.initials: GS
authors.lastname: McDonald
authors.forename: P A
authors.initials: PA
authors.lastname: Daly
authors.forename: D P
authors.initials: DP
authors.lastname: Kelleher
authors.forename: D G
authors.initials: DG
authors.lastname: Weir
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D018288:Carcinoma, Small Cell / Q000000981:diagnostic imaging* / Q000188:drug therapy* / Q000556:secondary; D003520:Cyclophosphamide / Q000008:administration & dosage; D018450:Disease Progression; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007205:Indium Radioisotopes; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000000981:diagnostic imaging* / Q000188:drug therapy* / Q000556:secondary; D011877:Radionuclide Imaging; D016879:Salvage Therapy; D013004:Somatostatin / Q000031:analogs & derivatives
chemical_list: D007205:Indium Radioisotopes; D013004:Somatostatin; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; C081788:pentetreotide