Small Cell Neuroendocrine Carcinoma Of The Cervix Research Articles

Pan-Canadian Analysis of Practice Patterns in Small Cell Carcinoma of the Cervix: Insights from a Multidisciplinary Survey.

Small-cell neuroendocrine carcinoma of the cervix (SCNECC) is a rare cancer with poor prognosis, with limited data to guide its treatment. The objective of this study was to evaluate practice patterns in the management of SCNECC. A 23-question online survey on management of SCNECC was disseminated to Canadian gynecologic oncologists (GO), radiation oncologists (RO) and medical oncologists (MO). In total, 34 practitioners from eight provinces responded, including 17 GO, 13 RO and four MO. During staging and diagnosis, 74% of respondents used a trimodality imaging approach, and 85% tested for neuroendocrine markers. In early-stage (1A1-1B2) SCNECC, 87% of practitioners used a surgical-based approach with various adjuvant and neoadjuvant treatments. In locally advanced (1B3-IVA) SCNECC, 53% favored primary chemoradiation, with cisplatin and etoposide, with the remainder using surgical or radiation-based approaches. In metastatic and recurrent SCNECC, the most common first-line regimen was etoposide and platinum, and 63% of practitioners considered clinical trials in the first line setting or beyond. This survey highlights diverse practice patterns in the treatment of SCNECC. Interdisciplinary input is crucial to individualizing multimodality treatment, and there is a need for prospective trials and intergroup collaboration to define the optimal approach towards managing this rare cancer type.

Pregnancy complicated by small cell neuroendocrine carcinoma of the cervix

Small cell neuroendocrine carcinoma of the cervix (SCNCC) is considered to be an extremely rare, highly invasive and aggressive subtype of squamous cell carcinoma. It is particulary rare during pregnancy. Here we report a case in which a 32-year-old patient who was admitted to our Department at 27 completed weeks of gestation with intermittent vaginal spotting and a large amount of malodorous vaginal discharge. Gynecological examination revealed a 2 cm exophytic polypoid tumor coming out of the cervix. On rectal examination there was no clinically evident parametrial invasion. Biopsy of the cervix was performed and the diagnosis of small cell neuroendocrine carcinoma was established. We reviewed and discussed the features, diagnosis, treatment and prognosis of SCNCC diagnosed during pregnancy.

The impact of neo/adjuvant treatment choices on prognosis for surgically treated small-cell neuroendocrine carcinoma of the cervix.

Small-cell neuroendocrine carcinoma of the cervix (SCNCC) is a rare and aggressive tumor with a poor prognosis. Surgical resection followed by adjuvant therapy is the standard treatment for early-stage disease but the influence of different neo/adjuvant treatment approaches remains unclear. Retrospectively, we collected patients' characteristics and treatments in two medical centers. Disease status and survival outcomes were renewed through follow-up. Statistics analysis mainly included Kaplan-Meier methods for survival curve estimation, log-rank test for survival curve comparison, and Cox proportional hazards models for independent prognostic factors prediction. Finally, 51 patients treated by radical surgery between January 2010 and April 2020 were enrolled with a median age of 50 years (range: 32-68). 12 (23.5%) patients were at stage IIIC1 according to the International Federation of Gynecology and Obstetrics (FIGO) 2018 staging systems and the rest were at the early stage. The mean tumor size was 3.6±1.3 cm. Pathological examination found 24 cases with pure SCNCC and 27 cases with admixed SCCC. 29 (56.9%) patients had deep stromal infiltration and 19 (37.3%) patients had lymphovascular space invasion. 34 (66.7%) patients received neo/adjuvant chemotherapy and pelvic radiation was conducted in 41 (80.39%) patients with a median dose of 46 Gy (range: 40-50.4 Gy). The median follow-up time was 25.0 months. The median disease-free survival (DFS) time was 23.0 months. 27 (52.9%) patients developed distant metastasis and 14 (27.5%) experienced local failure. The median overall survival (OS) was 32.0 months. Univariate and multivariate analysis showed neoadjuvant chemotherapy as negative (HR=2.081, 95% CI 1.030-4.203, p=0.041) and adjuvant chemotherapy (HR=0.409, 95% CI 0.213-0.784, p=0.020) as positive independent prognostic factor for DFS. For OS, only lymph node metastasis was confirmed as an independent prognostic factor in both univariate analysis (HR=1.528, 95% CI 1.011-2.308, p=0.044) and multivariate analysis (HR=1.697, 95% CI 1.041-2.768, p=0.034). In conclusion, for surgically treated SCNCC, adjuvant chemotherapy showed a positive influence on DFS while neoadjuvant chemotherapy harmed DFS. OS was unaffected by either treatment choice.

Recent Advances in the Management of Small Cell Carcinoma of the Uterine Cervix.

Small cell neuroendocrine carcinoma of the cervix (SCNEC) is a rare disease characterized by a higher incidence of lymphatic invasion, metastasis, and recurrence compared to the squamous cell carcinoma and adenocarcinoma subtypes. Furthermore, it is prone to early distant metastasis and has a poor prognosis. Chemotherapy has an important role in the management of cervical SCNEC. The effective treatment schemes for early-stage SCNEC are local treatment with radical surgery and systemic chemotherapy. However no standard treatment regimen exists because of a rare disease. We reviewed previous reports to determine whether etoposide/platinum, which is used for histopathologically similar small cell carcinoma of the lung, is an appropriate initial chemotherapy regimen for SCNEC of the cervix. In this review the Cochrane library sources, ClinicalTrials.gov, Web of Silence, PubMed and search engine of Google scholar were searched for all interventional studies, reviews, case reports and meta-analyses published in 1997-2021. Etoposide/platinum (EP) is the most commonly used regimen and paclitaxel/carboplatin is the second most common, used as a part of multimodality therapy for SCNEC of the cervix in most studies. Cisplatin/vincristine/bleomycin, cisplatin/irinotecan, cisplatin/ifosfamide/etoposide were also reported in concurrence with EP; however no clinical trials are dedicated to SCNEC. Etoposide and platinum tend to have a better prognosis compared to other regimens used for other subtypes of cervical cancer. For recurrent cervical SCNEC, treatment options for patients are very limited. The application of molecular testing for targeted mutations may suggest potential future therapies that may be useful in this disease.

Small cell neuroendocrine carcinoma of the cervix: Analysis of prognostic factors and patterns of metastasis

ObjectivesTo describe characteristics and outcomes of patients with small cell neuroendocrine carcinoma of the cervix (SCNCC) and determine the staging system most predictive of outcome—the two-tier (limited-stage [LS] vs. extensive-stage [ES]) or International Federation of Gynecology and Obstetrics (FIGO) staging system. MethodsPatients with SCNCC evaluated at our institution from 1/1/1990–6/30/2021 were included. Medical records were reviewed for variables of interest. Appropriate statistical tests were performed to determine associations. Survival curves were created using the Kaplan-Meier method. Concordance probability estimates (CPEs) were calculated to evaluate the prediction probability of the staging systems. ResultsOf 63 patients, 41 had LS and 22 ES SCNCC. Patients with ES disease were significantly older than those with LS disease (median, 54 and 37 years, respectively; p < 0.001). Smoking status, race, and history of HPV were not associated with stage or outcomes. Forty-eight patients had metastatic disease (24 [50%] at initial diagnosis). The most common first sites of metastasis were lung (n = 20/48, 42%), lymph nodes (n = 19/48, 40%), and liver (n = 13/48, 27%). Nine patients had brain metastasis (8 symptomatic at recurrence; 1 asymptomatic at initial diagnosis). Both staging systems were associated with progression-free and overall survival. Adjusted CPE found the FIGO staging system was more predictive of outcomes than the two-tier staging system. ConclusionsProviders should have a low threshold to obtain brain imaging for patients with SCNCC, especially in the presence of visceral metastases. FIGO staging should be used to classify SCNCC. Further research is necessary to understand prognostic factors of this rare disease.

Ubiquitin C-terminal hydrolase L1 promotes lymph node metastasis in small cell neuroendocrine carcinomas of the cervix.

ObjectiveTo screen for specific differentially expressed genes in small cell neuroendocrine carcinoma of the cervix (SCNEC) and to further explore their roles and mechanisms in tumor progression.MethodsDifferentially expressed genes in SCNEC compared with squamous cell carcinoma (SCC) and adenocarcinoma (AC) were screened by microarray and immunohistochemical analyses. The biological functions of the identified genes were examined in a SCNEC cell line using RNA interference and over-expression plasmid-transfection technologies. Co-expression network analysis and immunoprecipitation technology were used to explore the potential mechanisms.ResultsCompared with SCC and AC, UCHL1 (encoding ubiquitin C-terminal hydrolase L1) was identified as a specific differentially expressed gene in SCNEC, which was positively related to lymph node metastasis (LNM). Migration and invasion of SCNEC tumor cells were induced by UCHL1 over-expression and suppressed by UCHL1 down-regulation, as shown by scratch and transwell invasion assays. Co-expression network analysis suggested that Prospero homeobox protein 1 (PROX1) might interact with UCHL1, and in vivo immunoprecipitation and western blots verified that levels of ubiquitinated PROX1 were significantly decreased following UCHL1 overexpression.ConclusionUCHL1 is a potential biomarker of LNM in SCNEC. UCHL1 might promote SCNEC cell migration and invasion by reducing PROX1 ubiquitination.

Data from small cell neuroendocrine carcinoma of the cervix: FIGO 2018 staging is more accurate than FIGO 2009.

ObjectiveTo compare the prognostic value of International Federation of Gynecology and Obstetrics (FIGO) 2009 and 2018 staging systems in surgical patients with small cell neuroendocrine carcinoma of the cervix (SCNEC).MethodsWe re-staged 64 surgical IB–IIA (FIGO 2009) SCNEC patients according to the FIGO 2018 system and refined stage IIIC of FIGO 2018 based on tumor local invasion. The prognostic factors were analyzed, and the advantages of FIGO 2018 were compared with 2009.ResultsThe 5-year overall survival rate (OS) was 78.5% for stage I and 22.2% for stage II (FIGO 2009). In FIGO 2018, there was no difference between stage I and II, and the 5-year OS was 74.1%, 60.2%, and 0% for stage I/II, IIIC1, and IIIC2. After combining stage IIIC with the local invasion stage (T1 was limited to the cervix and vagina; T2 involved the parametrium; T3 involved the pelvic or abdominal cavity), the 5-year OS for stage IIICT1, IIICT2, and IIICT3 was 83.3%, 30.0%, and 0%, respectively.ConclusionsFor stage II SCNEC patients, FIGO 2009 underestimated the prognosis, while FIGO 2018 was more accurate. For stage IIIC, FIGO 2018 might be more individualized and accurate after combining stage IIIC with tumor local invasion.

Diagnostic value of four neuroendocrine markers in small cell neuroendocrine carcinomas of the cervix: a meta-analysis

Small cell neuroendocrine carcinoma of the cervix (SCNECC) is a highly invasive cervical cancer. The immunohistochemical criteria is an important aspect for assistant diagnosis of SCNECC. However, which markers can be appropriate selection for diagnosing SCNECC were not determined. The aim was to systematically evaluate expression levels of four neuroendocrine markers (containing synaptophysin (Syn), neural cell adhesion molecules (CD56), neuron-specific enolase (NSE) and chromograninA (CgA)) and to find out the appropriate selection for diagnosing SCNECC. Four English and three Chinese libraries were retrieved between 1984 and 2020. 23 studies about NSE, 36 studies about Syn, 23 studies about CD56 and 36 studies about CgA (all studies containing 581 patients) were eligible for meta-analyses. The pooled positive expression percentages (95% CI; I2) were as follows: 84.84% (79.41–90.27%; 76.7%) for Syn, 84.53% (79.43–89.96%; 37.5%) for CD56, 77.94% (69.13–86.76%; 83.5%) for NSE, and 72.90% (67.40–78.86%; 59.7%) for CgA. The positive proportions (95% CI; I2) ranked top three of simultaneous expressions of two markers were 87.75% (82.03–93.87%, 33.3%) for Syn and CD56, 70.92% (50.50–87.68%, 82.7%) for Syn and NSE, 65.65% (53.33–76.98%, 73.5%) for Syn and CgA. This confirms that Syn and CD56 are reliable indicators for diagnosing SCNECC.

Prognostic factors and outcomes of early-stage small cell neuroendocrine carcinoma of the cervix: 37 cases from a single center

BackgroundThe objective of this study is to investigate small cell neuroendocrine carcinoma of the cervix (SCCC), using a retrospective clinicopathological characteristic and treatment approach.MethodWe retrospectively analyzed cases of early-stage SCCC, identified between 2006 and 2016, in women who received radical surgery and adjuvant chemotherapy with or without radiotherapy. Kaplan–Meier and one-way ANOVA analyses were performed.ResultA total of 37 cases of SCCC are presented in this study, of which 21 had stage IB1 SCCC, 12 had stage IB2, 3 had stage IIA1, and 1 had stage IIA2. All patients were treated with radical surgery and adjuvant chemotherapy, specifically, 26 with radical surgery followed by adjuvant chemotherapy plus radiation and 11 with neoadjuvant chemotherapy (NACT) followed by radical surgery. After a median follow-up time of 27 months (range, 8–115 months), the 2-year and 5-year disease-free survival rate for all patients was 51.9% and 34.1%, respectively, and the overall survival rate was 60.3% and 38.6%, respectively. Univariate analysis showed that International Federation of Gynecology and Obstetrics (FIGO) stage and tumor size may be a predictor of a poor prognosis. NACT and adjuvant radiation did not improve survival over adjuvant chemotherapy alone but should not be a significant independent prognostic factor for survival.ConclusionEven in patients with early-stage SCCC, the prognosis is poor, although FIGO stage and tumor size may act as surrogate factors prognostic of survival.

A clinical analysis of small-cell neuroendocrine carcinoma of the gynecologic tract: report of 20 cases.

The aim of this retrospective observational study was to analyze the clinical and pathological characteristics of small-cell neuroendocrine carcinoma of the gynecologic tract (SCNCGT). Twenty patients with SCNCGT were enrolled and their clinic-pathological features were analyzed. All patients were treated at the Beijing Obstetrics and Gynecology Hospital, Capital Medical University, China, and were followed up until December 31, 2017. (1) Patient characteristics: The incidence of SCNCGT was 0.3% (20/6578) of gynecologic cancer in our hospital from January 1, 2007, to December 31, 2017. The average age of the patients was 42.0 ± 11.8 (23-63years). Out of 20 patients enrolled, seven (35.0%) had lymph node metastasis. Out of 17 patients treated with complete surgery, 14 (82.4%) had lymph-vascular space invasion. (2) Treatment: Eleven out of the 14 patients with small-cell neuroendocrine carcinoma of the cervix (SCNCC) were treated with radical surgery; all the 11 patients received chemotherapy and radiotherapy postoperatively. The remaining three patients received comprehensive chemotherapy and/or radiotherapy instead of radical surgery. The six patients who had one or the other type of SCNCGT (involving the ovary, endometrium, or vagina) were all treated with comprehensive surgery. (3) Prognosis: The follow-up time for the study ranged from 8 to 87months. Three (15.0%) of the 20 patients were diagnosed with distant metastasis at the beginning of the study. Eight (40.0%) patients died as of December 31, 2017, while the other 12 patients were in follow-up. The average survival time was 43.6months (16-77months). SCNCGT is a highly malignant tumor characterized by rare morbidity, a propensity for metastasis, and poor prognosis. Comprehensive treatment may be a good approach to prolong survival in some patients.

Cycles of cisplatin and etoposide affect treatment outcomes in patients with FIGO stage I-II small cell neuroendocrine carcinoma of the cervix.

This study sought to explore the outcomes and prognostic factors of patients with small cell neuroendocrine carcinoma of the cervix (SCNEC) and to determine the effects of adjuvant treatment on survival in patients with FIGO stage I-II SCNEC after radical surgery. A single-institution retrospective analysis was performed in 92 patients who underwent radical surgery for SCNEC. All clinicopathological variables and treatment strategies were reviewed. Kaplan-Meier and Cox regression methods were used for survival analyses. During a median follow-up period of 38months (23.6-52.4), 43 (46.7%) patients experienced disease recurrence, and distant metastases were documented in 35 (81.4%) patients. The 3-year recurrence-free survival (RFS) for the entire group was 50.1%. The median RFS was 39months. The multivariate analysis confirmed that lymph node metastasis, positive parametrial extension and cycles of etoposide plus platinum (EP) were independent prognostic factors for disease recurrence. Adjuvant chemotherapy for at least 5cycles of EP (EP 5+, n=39) was associated with improved 5-year RFS compared with other treatments (n=46) (67.6% vs. 20.9%, p<0.001). Additional radiotherapy or concurrent chemoradiation failed to validate further improved RFS in patients with EP 5+, and this finding was consistent in the subset of patients with high-risk factors (positive lymph nodes or positive parametrium). Half of stage I-II SCNEC patients experienced disease failure within 3years, and distant metastasis was an outstanding issue. EP regimen for at least 5cycles improved long-term RFS after radical surgery. Additional radiation might be unnecessary, even in patients with high-risk factors.

Treatment and outcomes of small cell neuroendocrine carcinoma of the cervix (SCCC).

5531 Background: Extrapulmonary small cell carcinoma is rare. SCCC represent 2% of cervical cancers and can portend a poor prognosis. Treatment standardization is challenging given its rarity. We describe management of limited stage (LS; disease could be encompassed within one radiation port) at a large tertiary referral center and the characteristics and outcomes in a cohort of patients (pts) with LS and extensive stage (ES) SCCC. Methods: Pts with SCCC diagnosed from 1/1990-1/2016 were identified following IRB approval. Clinicopathologic, treatment, and follow-up data were recorded. Descriptive statistics were provided. Median PFS/OS or PFS/OS rate were estimated using Kaplan-Meier method. Results: 39 pts were identified, 29 with LS. Select characteristics are shown in table. Tumor molecular profiling revealed MYC amplifications, TP53 mutations, PIK3CA mutation among the small subset of pts who had this performed. LS SCCC was treated with whole pelvic radiation therapy (RT) (4500-5040cGy) and concurrent IV cisplatin (60mg/m2) on day 1 and etoposide (120mg/m2) on days 1, 3, and 5 during RT and days 1-3 post RT to complete a total of 4 cycles. 26 pts, all had LS, underwent initial surgical management. No pt had prophylactic cranial RT. 3 pts (8%), all had LS, developed brain metastases. Median follow-up was 59.5 months (1.9-234.1). Median PFS (95%CI) for LS pts was 39.2 months (15.1-not estimable) vs 2.9 months (0.9-4.6) for ES. Median OS(95%CI) was 31.8 months (16.3-56.0) for the whole cohort, 52.8 months (31.8-not estimable) for LS and 5.9 months(1.8-16.3) for ES. Conclusions: In the LS SCCC cohort treated with concurrent cisplatin/etoposide chemo/RT and outback cis/etoposide +/- post initial radical hysterectomy the 5-year PFS (95%CI) was 37.5% (19.2-55.9%). Clinicopathologic characteristics and risk factors for SCCC appear distinct to cervical cancers and lung small cell cancers. Further investigation of molecular alterations and treatment of this rare tumor is needed to improve pt outcomes. [Table: see text]

Metastatic small cell neuroendocrine carcinoma of the cervix treated with the PD-1 inhibitor, nivolumab: a case report

BackgroundNivolumab is an immune checkpoint inhibitor specific for the programmed death 1 (PD-1) receptor that has led to clinical responses in many cancer types. Identifying biomarkers predictive of response to PD-1 blockade is an area of active investigation.Case presentationWe present a patient with recurrent, metastatic, PD-L1-negative small cell neuroendocrine carcinoma of the cervix (SCNEC) who experienced a complete response to nivolumab. Though nivolumab was discontinued over 4 months ago due to treatment-related adverse events, she continues to have no evidence of disease.ConclusionsImmune checkpoint inhibitors may be active in neuroendocrine cervical cancer, with potential for dramatic responses in a modest subset of patients.

Carcinomatous meningitis during systematic chemotherapy in a patient with advanced small‐cell neuroendocrine carcinoma of the uterine cervix

Carcinoma meningitis with uterine cervical cancer is an extremely rare event. We present the clinicopathologic characteristics and diagnostic imaging findings of a patient with advanced small-cell neuroendocrine carcinoma of the cervix who had developed carcinoma meningitis during systematic chemotherapy.

Clinicopathologic analysis of small cell neuroendocrine carcinoma of the cervix

To analyse the clinico-pathologic characteristics, diagnosis, therapy and prognostic of small cell neuroendocrine carcinoma of the cervix (SCNCC). The clinic-pathological features of 12 patients with SCNCC treated in Tumor Hospital of Guangxi Medical University, admitted during March 2006 to July 2010, were analyzed retrospectively. Of 12 patients, the mean age was 38.7 years (rang 28 - 57 years), 6 had stages Ib1-IIa, 6 had stages IIb-IV. Among 8 patients (Ib1-IIIb) underwent surgery, 4 of them received neoadjuvant chemotherapy, 8 of them received adjuvant chemotherapy and(or) radiotherapy. All had greater than one-half stromal invasion, 4 patients had positive pelvic lymph nodes metastases. The positive ratio of the chromogranin (CgA), synaptophysin, neuronspecific enolase (NSE), cytokeratins (CK), CD(56) tested by immunohistochemical staining were 8/12, 9/10, 4/4, 4/4, 4/4, respectively. Median follow-up period was 3 months (1 - 22 months). Among 8 patients underwent surgery, 2 patients developed lung metastases, 1 patient developed liver and lung metastases, 1 patient developed liver metastases concurrently with bone metastases, disease-free survival (DFS) were 3 months (Ib2 with positive lymph nodes), 4.6 months (IIa), 7 months (Ib1), 17 months (Ib2); 2 patient died (8.5 and 11.3 months, respectively) after surgery; 4 patients are alive and show no evidence of disease. Among 4 patients untreated, 1 patients received concurrent chemoradiation and are alive for 10.1 months. Two patient untreated (IIIb, IV) died after 0.6 and 1.3 months final diagnosis, respectively. One patient was lost follow-up. SCNCC is a highly malignant tumor with rare morbility, propensity for distant spread and dismal prognosis. Final diagnosis of SCNCC depends on pathomorphology and immunohistochemical analysis. Combined therapeutic modalities may in favor of survival in some patients.

Unfavorable Prognosis of Small Cell Neuroendocrine Carcinoma of the Uterine Cervix

This study was a matched case-control study to analyze the clinical and pathological characteristics and the prognosis of patients with small cell neuroendocrine carcinoma in the uterine cervix. Thirty-two patients were treated for small cell neuroendocrine carcinoma of the cervix (SCNEC) at Asan Medical Center between January 1996 and June 2008. For a 1-to-2 matched case-control study, 64 patients with squamous cell carcinoma of the cervix (SCC) whose clinical stage and age are consistent with the SCNEC group were selected. Medical records were retrospectively reviewed to analyze and compare the clinical and pathological outcomes. In the SCNEC group, the median age was 45 years, and early stage (stage IIA or below) was 75.0%. The postoperative adjuvant therapy was given more frequently in the SCNEC group. The recurrence rate was 59.4%, and lung, bone, and liver were common sites in the SCNEC group. Parametrial involvement and lymphovascular space invasion were risk factors in the SCNEC group, whereas lymph node metastasis is the risk factor in the SCC group. The progression-free survival and overall survival were significantly shorter in the SCNEC group than in the SCC group (16.9 and 30.6 months vs 47.7 and 49.1 months, P < 0.05). Interestingly, survival outcomes in the early stage of SCNEC were remarkably worse than those of SCC and almost identical to those of the advanced stage of SCNEC. We confirmed the unfavorable prognosis related to hematogenous metastasis in SCNEC. The treatment modality of early-stage SCNEC, which is radical hysterectomy followed by adjuvant therapy, needs to be reevaluated.

Small cell neuroendocrine carcinoma of the cervix: Analysis of outcome, recurrence pattern and the impact of platinum-based combination chemotherapy

Objectives To analyze progression-free (PFS) and overall survival (OS) in patients with small cell neuroendocrine carcinoma of the cervix (SCNEC), and to determine whether platinum-based combination chemotherapy is beneficial for this population. Methods We performed a retrospective analysis of all patients with SCNEC who were treated at our institution between 1/1990 and 2/2007. Patients were excluded if pathologic diagnosis was not confirmed at our institution. Standard statistical methods were utilized. Results Seventeen patients met inclusion criteria. The estimated 3-year PFS and OS rates for the entire group were 22% and 30%, respectively. Median time to progression was 9.1 months. Extent of disease was the only significant prognostic factor. Median OS for patients with early stage disease (IA1–IB2) was 31.2 months and 6.4 months for patients with advanced stage disease (IIB–IV, P = 0.034). In the early-stage disease group, the 3-year distant recurrence-free survival rate was 83% for patients who received chemotherapy and 0% for patients who did not receive chemotherapy as part of their initial treatment ( P = 0.025). The estimated 3-year OS rate was 83% for patients who received and 20% for patients who did not receive chemotherapy as part of their initial treatment ( P = 0.36). Conclusion Given the rarity of SCNEC this retrospective analysis is limited by a small number of patients. However, the natural history of this rare disease is akin to small cell lung cancer and the prognosis is poor due to the tumor's propensity for distant spread. The treatment should conform to the treatment of small cell lung cancer.