Should extrapulmonary small cell cancer be managed like small cell lung cancer?

Abstract

BACKGROUND:The aim of this study was to determine if extrapulmonary small cell carcinomas (EPSCC) should be managed using protocols similar to those for small cell lung cancer (SCLC). METHODS: Treatment strategies, survival, patterns of failure, and prognostic factors for patients with EPSCC were analyzed retrospectively at a large cancer center. SCLC was excluded by thoracic computed tomography (75%) or chest radiography (25%). RESULTS: Of 120 eligible patients, 70% had limited disease (LD). Treatment modalities included chemotherapy (n ¼ 82; 68%), radiotherapy (RT) (n ¼ 80; 67%), and surgery (n ¼ 41, 34%). The median survival for patients with LD and extensive disease was 1.4 years and 0.7 years, respectively. Gynecologic (n ¼ 31) and gastrointestinal (n ¼ 28) were the most common primary tumor sites. Gynecologic and head and neck primary tumor sites had better 1-year survival than other sites (P ¼ .019 and 0.005, respectively). Brain metastasis was the site of first distant failure in 4.1% of patients versus 35% for soft tissue metastases. The lifetime risk of brain metastasis was 13%. Definitive RT (P ¼ .004), LD (P ¼ .028), and prophylactic cranial irradiation (PCI) (P ¼ .022) were found to be positive prognostic factors and weight loss (P < .001) was a negative prognostic factor on multivariate analysis. CONCLUSIONS: Patients with EPSCC usually experienced short survival, often with early distant metastasis. Although PCI was associated with improved overall survival, brain metastasis was less frequent than in patients with SCLC, and therefore the potential benefit of PCI was less than in patients with SCLC. Definitive chemoradiotherapy was associated with better outcomes and should be delivered whenever feasible. Cancer 2010;116:888-95.

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

“…In the present study, the most common primary sites of the EP-NECs were the stomach, pancreas, and esophagus. On the other hand, relatively few patients had tumors arising in the reproductive tract, breast, or head and neck region, which have been reported to be associated with a better prognosis [4,9,10,11,12]. At our institution, genitourinary EP-NECs tend to be resected, and head and neck EP-NECs are often treated using chemoradiotherapy.…”

“…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].…”

Stage 1 small cell cancer of the vagina

“…Although genital small cell carcinoma is the most common form of extrapulmonary small cell carcinoma,2 vaginal small cell carcinoma is a very rare disease with only about 30 cases reported in the literature. It has no treatment guidelines and carries a significantly poor prognosis 3…”

Extrapulmonary small cell carcinoma: the University of Kansas experience and review of literature

“…Oat cell carcinoma of the mediastinal glands was first described as a distinct clinicopathologic disease entity in 1930—1934 years after small cell carcinoma of the lung had been described—and since that time, its presence has been increasingly recognized [1–3]. Though small cell carcinoma makes up 20–25 % of bronchogenic carcinomas, primary sites outside the lung are much more rare [4].…”

“…Though small cell carcinoma makes up 20–25 % of bronchogenic carcinomas, primary sites outside the lung are much more rare [4]. Many different extrapulmonary sites can be involved, with the GI and GU systems being the most common [1]. Within the GU tract, the bladder and prostate are frequently affected, with the first case of bladder primary described in 1981 [5].…”