Small-cell carcinoma of the gastrointestinal tract: a retrospective study of 64 cases

Abstract

Small-cell carcinoma (SmCC) of the gastrointestinal tract is a very rare and aggressive malignancy. To better define its clinicopathological features, the records of all patients with this disease seen at Memorial Sloan Kettering Cancer Center between 1980 and 2002 (n ¼ 64) were reviewed. The most common primary tumour locations were in the large bowel and oesophagus. Predisposing medical conditions for non-small-cell cancers, positive family cancer history, and metachronous tumours were common. In all, 37% had mixed tumour histology and 48% presented with extensive disease, according to the Veterans' Administration Lung Study group (VALSG) staging system used for small-cell lung cancer. Treatment outcome in limited disease (LD) suggested a role for surgery and chemotherapy. Platinum-based regimens resulted in a 50% response rate. The 2-year survival was 23% and two prognostic factors were identified, the extent of disease according to the VALSG system (Po0.01) and TNM stage (P ¼ 0.03). Anatomic location had no clinical impact. In conclusion, SmCC from various gastrointestinal sites can be viewed as one clinical entity. Mixed tumour histology is common and may affect therapy. Surgery, combined with chemotherapy, should be considered for LD. The value of the VALSG system was implied and possible differences from small-cell lung cancer were noted.

Comparison of Chemotherapeutic Treatment Outcomes of Advanced Extrapulmonary Neuroendocrine Carcinomas and Advanced Small-Cell Lung Carcinoma

“…The objective response rate to platinum-based chemotherapy was 77.8% for the patients with SCLC, and the MST in the present study was 13.6 months, which was similar to previously reported survival times [33,34,36]. On the other hand, the efficacy of such regimens against EP-NECs seemed to be worse in the present study, with a response rate of 31.7% compared with 33–66.7% in previous reports [5,7,10,20,21]. Although the reason for the difference in the treatment efficacies was unknown, possible reasons include the differences in the criteria used to evaluate response and the relatively small number of patients.…”

“…Additionally, the response rates of EP-NECs with different primary sites were significantly different, with the HBP group showing the lowest response rate. This finding also disagreed with Brenner et al’s [5 ]report, which showed no differences in the response to chemotherapy among the different anatomical locations. The patients with EP-NECs tended to have unfavorable outcomes compared with the patients with SCLC in our study.…”

“…The definition of NEC refers to neoplasms previously classified as small-cell carcinoma or poorly differentiated (neuro)endocrine carcinoma (PDNEC). Since the first report of ‘extrapulmonary oat cell carcinoma’ of the mediastinum by Duguid and Kennedy [3 ]in 1930, extrapulmonary NECs (EP-NECs) have been reported to arise in a variety of organs, such as the gastrointestinal tract, pancreas, head and neck region, or urogenital tract [4,5,6,7,8,9,10,11,12,13,14,15,16]. EP-NECs are a fairly rare, heterogeneous disease entity, and no standard treatment has been established [17,18].…”

Extrapulmonary small cell carcinoma: Single center experience with 61 patients

“…Diagnoses were confirmed immunohistochemically, using antibodies to chromogranin A, synaptophysin and neuron specific enolase (NSE). Mixed SCC was defined as tumors containing both SCC and non-SCC components, regardless of the proportion of the latter [12].…”

Progress in the treatment of esophageal neuroendocrine carcinoma

“…The most common type is medullary, followed by mushroom, and ulcerative. 17 These tumors can be divided into different subtypes according to their histopathological and morphological characteristics and degree of differentiation. The majority are NECs and MANECs.…”