Patterns of relapse in extrapulmonary small cell carcinoma: retrospective analysis of outcomes from two cancer centres

Abstract

ObjectivesWe conducted a retrospective review of patients with extrapulmonary small cell carcinomas (EPSCCs) to explore the distribution, treatments, patterns of relapse and outcomes by primary site.SettingWe have reviewed the outcomes of one of the largest data sets of consecutive patients with EPSCC identified from two major cancer centres.ParticipantsConsecutive patients with a histopathological diagnosis of EPSCC from the two institutions were retrospectively identified.Primary and secondary outcome measuresOutcomes were evaluated including stage at presentation, treatments given, sites of relapse, time to distant relapse, progression-free survival and overall survival (OS).ResultsFrom a total 159 patients, 114 received first-line chemotherapy, 80.5% being platinum-based. Response rate was 48%. Commonest primary sites were genitourinary and gynaecological. 44% of patients presented with metastatic disease. 55.9% relapsed with liver the commonest site, whereas only 2.5% developed brain metastases. Median OS was 13.4 months for all patients, 7.6 months and 19.5 months for those with metastatic and non-metastatic disease, respectively. Gynaecological and head and neck patients had significantly better OS compared to gastrointestinal patients.ConclusionsEPSCCs demonstrate high response rates to chemotherapy and high rates of distant metastases. Primary sites may influence prognosis, and survival is optimal with a radical strategy. Brain metastases are rare and we therefore do not recommend prophylactic cranial irradiation.

Genetic and molecular alterations in olfactory neuroblastoma: implications for pathogenesis, prognosis and treatment

“…In a population based study on SNEC not divided into small cell and large cell neuroendocrine type the 5 year survival was similar (50,8%) to that of small cell type (46.3%) [100, 101]. In one large study from two central university hospitals the prognosis of head and neck SNEC was higher compared to gastrointestinal type tumors.…”

Controversies in small cell carcinoma of the head and neck: Prophylactic cranial irradiation (PCI) after primary complete initial remission

“…Overall, literature data do not support the routine use of PCI in extrapulmonary SmCC because the incidence of brain metastasis for different SmCC anatomical sites ranged from 2.5% to more than 40% [31,27]. All of these studies demonstrate the absence of brain metastasis in patients who received PCI, but they were not able to show an absolute benefit as they were not randomized studies.…”

“…Both PCI patients were alive at data analysis with an overall survival of 11 and 33 months, respectively. In a two-Institutes retrospective analysis [31], of the 159 SmCC reviewed, 14 (8.8%) were primary SmCCHN. Brain metastasis were reported in only 4 patients (2.5%) of the total patients population but there were no specific documentation of SmCCHN cases.…”

“…In all, of 1012 patients with extrapulmonary SmCC, 29 received PCI, none of whom developed brain metastasis. Of the 983 who did not have PCI, 106 (10.8%) developed brain metastasis [7,[12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31] ( Table 1).…”

Extrapulmonary small cell carcinoma of lymph node: Pooled analysis of all reported cases

“…Eight studies (n = 14) were included in the current analysis ( Figure 6) [16][17][18][19][20][21][22][23] . Studies that did not provide separate information for patients with LNEPSCC [29][30][31][32][33][34][35][36][37][38][39][40][41] , and the treatment given or follow up were not included in the current review [42][43][44][45][46][47][48][49][50] . A total of 17 patients including the three index cases (mean ± SD, 59.5 ± 10.8 years; 81.8% males) with LNEPSCC were included in the current analysis (Table 1).…”