Small cell carcinoma with concomitant adenocarcinoma arising in a Barrett’s oesophagus: report of a case with a favourable behaviour

Abstract

Most Barrett's oesophagus-associated cancers are adenocarcinomas which occur in a pure form. They are rarely combined with another type of malignancy, such as endocrine tumours. Within the endocrine spectrum, small cell carcinomas (SmCC) usually have a highly aggressive behaviour with a poor prognosis. We report a case of composite SmCC and adenocarcinoma in the setting of a Barrett's oesophagus, in a 54-year-old man. This tumour was identified on a surgical specimen after neoadjuvant treatment with radiotherapy and 5-FU-Cis-platin based chemotherapy. The SmCC component was positive for chromogranin A, synaptophysin, neural cell adhesion molecule and neuron-specific enolase and negative for high molecular weight cytokeratin. The adenocarcinoma component showed a converse phenotype. In our case, the origin of the SmCC component could be explained by the numerous chromogranin A-positive cells observed in the Barrett's oesophagus or by the potential progenitor cells that may be located in the submucosal oesophageal gland ducts and the Barrett's metaplasia. Our report is thus indicative of the high and totipotential risk of Barrett's oesophagus. Moreover, it is particular because of its favourable behaviour, with a 6-year disease-free survival, after neoadjuvant chemoradiation, surgery and postoperative chemotherapy.

Primary small cell carcinoma of the esophagus: patient data metaanalysis and review of the literature

“…One reason for the coexistence of small cell carcinoma, squamous cell carcinoma and glandular elements in the same lesion is that small cells have the potential for further differentiation into either mucin-producing or keratin-forming cells [18]. Besides, SCCE can also arise in Barrett’s esophagus [19], [20]. Another viewpoint is that the SCCE’s origin is from neuroendocrine cells of the submucosal gland or stratum basal, i.e.…”

A Complete Response in Small Cell Carcinoma of the Esophagus Treated by S-1/Cisplatin Combined Chemotherapy and Radiotherapy

“…Because the histological and clinical characteristics of SmCCE closely resemble those of small cell carcinoma of the lung, combined therapy is often used, such as VP16/cisplatin [8] or CPT11/cisplatin therapy [12]. The treatment of SmCCE has also been reported to consist of 5-FU/cisplatin therapy according to the conventional chemotherapy for SqCCE [13,14]. Yamashita et al [8] reported the efficacy of VP16/ cisplatin and radiotherapy, and its response rate was 77.8% (seven out of nine cases) in primary SmCCE.…”

“…4 After treatment, an upper gastrointestinal series (a) and endoscopy (b) showed no elevated lesion of the tumor, but instead an ulcerative lesion to be chosen in most cases [3,4,[7][8][9][10][11]. However, CPT11/ cisplatin, 5-FU, or some other therapy has also been selected recently [12][13][14]. These results indicated that no standard regimen for SmCCE has yet been established owing to the paucity of cases studied and the lack of any large studies.…”

“…Therefore, chemotherapy plays a very important role in the primary treatment of SmCCE. Various chemotherapy protocols for SmCCE have been reported, such as VP16/cisplatin [8], CPT11/cisplatin therapy [12], and 5-fluorouracil (5-FU)/ cisplatin therapy [13,14] according to the conventional chemotherapy for small cell carcinoma of the lung and SqCCE, respectively. However, no standard therapeutic protocol has yet been established because of the rarity of this disease.…”

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

“…2,5,9,[14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31] The baseline characteristics of all patients are shown in Table 1. The characteristics of patients from our institution are summarized in Table 2.…”