Small cell carcinoma of the esophagus: a multicentre Rare Cancer Network study

Abstract

Small cell carcinoma of the esophagus (SCCE) is a rare and aggressive malignant tumor with a poor prognosis. The aims of this retrospective study were to analyze the epidemiology, clinical characteristics, and treatment outcomes of these patients. Between 1994 and 2004, 24 patients with SCCE from several centers were reviewed for data on demographics, presenting symptoms, diagnosis, disease stage, type of treatment, and outcome. SCCE occurs in the sixth decade: median age (interquartile range [IQR]): 65 (59-69) years with a male predominance (63%). The most common complaining symptoms were rapidly progressive dysphagia (79%), weight loss (54%), and retrosternal/epigastric pain (46%). The tumor arises primarily in the middle (52%) or in the lower (35%) third of the esophagus. History of tobacco and alcohol exposure was present in 90% and 70% of case, respectively. Extensive disease was present in 13 cases (54%) at initial diagnosis. The overall median survival (IQR) was 11 (8-20) months for all 24 patients, and the 2-year overall survival was 25.1%. Four patients were alive more than 2 years after treatment. Chemotherapy increased the survival compared with symptomatic management in extensive disease (median survival [IQR]: 9.5 [6-14] vs. 6 [4-7] months, P= 0.05). In limited disease, concurrent chemo-radiotherapy was more effective than non-concurrent treatment (median survival [IQR]: 36 [14-93] vs. 11 [9-15] months, P= 0.04). Two patients were treated by surgery and chemoradiation therapy with a survival of 35 and 66 months. Chemotherapy is the cornerstone of treatment of SCCE in all stage. For limited disease SCCE, concurrent chemo-radiotherapy is the primary choice compared with sequential approach. The role of surgery was not assessable in our study.

Neuroendocrine carcinoma of the esophagus: clinical characteristics and prognostic evaluation of 49 cases with surgical resection

“…However, in present study, patients have showed a median survival time of 22.4 months after radical esophagectomy and regional lymphadectomy with or without adjutant therapy for esophageal NEC. To our best knowledge, recently published studies with relatively large cases exploring the role of surgery in treating limited disease stage esophageal NEC have yield a median survival time of 10.1 to 28.5 months (9,16,(22)(23)(24), while patients treated with chemoradiotherapy alone yielded a median survival time of 8.0 to 16.1 months (25)(26)(27). Therefore, it seemed that surgery may benefit patients with limited stage esophageal NEC, but the role of surgery in treating esophageal NEC still remains to be confirmed and validated (28).…”

Small-cell neuroendocrine carcinoma of the esophagus: an autopsy case report

“…However, its extra pulmonary origin represents 2.5-5.9% of all cases, represented by gastrointestinal (GI) and genitourinary systems, plus skin and thymus involvement 1 - 6…”

“…Among the environmental factors, smoking and alcohol consumption are the main components 2 , 6 , 8. Men are 1.3-3.2 times more affected than women mainly in the fifth or sixth decade of life.…”

Multimodality Therapy Is Recommended for Limited-Stage Combined Small-Cell Esophageal Carcinoma

“…4,33 In addition, change in histology during the treatment period also makes the diagnosis more challenging, 34 akin to C-SCLC. 35 In this study, the vast majority (55/72 [76%]) of patients were diagnosed by postoperative pathology, which illustrates the importance of histopathological diagnosis.…”