Limited-Stage Extrapulmonary Small Cell Carcinoma: Outcomes After Modern Chemotherapy and Radiotherapy

Abstract

Despite modern chemotherapy and RT, patients with limited-stage EPSCC do poorly. Consistent with previous findings the majority of the first failures are distant. Brain failures in this series were uncommon despite no prophylactic cranial radiation. These findings support the need for further studies in an attempt to improve systemic therapies for this disease.

Extrapulmonary small cell carcinoma: An indication for prophylactic cranial irradiation?

“…Two recent studies suggested that there may be a subset of patients, e.g., patients with primary tumors in the head and neck region and patients with urothelial small cell cancer, who potentially benefit from PCI [37,48]. However, most studies have reported brain metastases to occur less frequently than in SCLC, therefore, questioning the role of PCI in EPSCC [4,5,24,35,40,46]. Our study results showed an incidence of 13%, which also does not support the routine use of PCI.…”

“…Most of the series addressing the question of PCI in EPSCC were small and often included less than 20 patients [4,5,24,35,40,46]. Two recent studies suggested that there may be a subset of patients, e.g., patients with primary tumors in the head and neck region and patients with urothelial small cell cancer, who potentially benefit from PCI [37,48].…”

Current approaches for prophylactic cranial irradiation in extrapulmonary small cell carcinoma

“…In this series, most of the patients had distant failure (78%, 14 of 18). According to this report PCI was not necessary 11 .…”

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

“…The incidence of EPSCC in North America is estimated to be in the range of 0.1–0.4 %, making it account for approximately 1 in 20 cases of small cell [1, 7–9]. Much like pulmonary small cell carcinoma, EPSCC has an aggressive natural history with dissemination typically occurring early and with a high rate of recurrence or relapse [2].…”

“…Because of its low incidence, treatment recommendations for EPSCC have originated from case reports, single institution case series, or the extrapolation of results from pulmonary small cell trials [1, 7, 9]. No consensus guidelines exist and further work is needed to improve our understanding of the best way to manage these patients.…”