The pathology of extrapulmonary small cell carcinoma.

Primary high-grade neuroendocrine carcinoma of the esophagus (HNCE) is rare and poorly understood. In this study, we aimed at delineating the clinicopathologic and immunohistochemical characteristics of HNCE diagnosed on the basis of the World Health Organization criteria for pulmonary neuroendocrine carcinomas. We identified 42 (3.8%) consecutive resection cases of HNCE among 1105 esophageal cancers over a 7-year period. Patients' mean age was 62 years (range, 47 to 79 y) with a male to female ratio of 3.7. Dysphagia was present in 79% of patients and tobacco abuse in 50%. Most tumors were centered in the middle (52%) or lower (36%) esophagus; 48% were ulcerated and 31% exophytic. All tumors were sharply demarcated with a pushing border in either solid sheet (83%) or nodular (17%) growth patterns. Pure HNCE was found in 57%, and the remainder also exhibited small components of squamous cell carcinoma (SqCC) or glandular, signet ring cell differentiations. SqCC in situ was present in 50%. Most tumors (88%) were the small cell type with pure oat-like cells in 52%, and the larger spindled, anaplastic, and giant cells were common. Tumor crush artifact (98%) and the Azzopardi effect (88%) were widespread. Extensive lymphovascular (50%) and perineural (33%) invasion and metastasis to regional (48%) and abdominal celiac lymph nodes (29%) were observed. Neoplastic cells were immunoreactive to synaptophysin (100%), CD56 (93%), chromogranin A (67%), p63 (55%), TTF-1 (71%), CK8/18 (90%), CD117 (86%), HER2 (16%), and p16 (84%) antibodies. The 5-year survival rate was 25%, similar to that of SqCC. Lymphovascular and perineural invasion was associated with a worse prognosis.

Primary high-grade neuroendocrine carcinoma of the esophagus: a clinicopathologic and immunohistochemical study of 42 resection cases.

Small cell carcinomas of the gastrointestinal tract: clinicopathological features and treatment approach.

AIM: To analyze the clinical manifestations, pathological features and treatment of primary small cell carcinoma (SCC) of the esophagus and to review the literature on this entity.

METHODS: The records of 9 patients with primary esophageal small cell carcinoma were examined and the demographic data, presenting symptoms, methods of tumor diagnosis, and types of treatment given, response to treatment, pathologic findings, and clinical outcome were reviewed. Features of mixed patterns of histological differentiation and lymph node metastases were specifically sought.

RESULTS: All the patients reported dysphagia, weight loss and chest pain as the initial symptoms. In 5 cases the tumors were located in the mid-esophagus, 3 cases in the lower third of the esophagus and 1 case in the upper third. The average length of esophageal involvement was 5 cm. They underwent radical resection, regional lymph node clearance and esophageal-stomach anastomosis in thorax or at neck. Two patients had a stage IIa disease, five had a stage IIb disease, and the other two had a stage III disease of International Union Contrele Cancer (UICC). All of them were histologically and immunohistochemically confirmed SCC of esophagus. Immunohistochemical staining for neuron-specific enolase (NSE), synaptophsin (Syn) and chromogranin A exhibited strong immunoreactivity in all specimens. Three of the nine resected specimens showed foci of squamous cell carcinoma in situ. Metastasis was present in 7 of 9 adjacent lymph nodes. All the patients survived the operations and made an uneventful postoperative recovery. They received adjuvant systemic chemotherapy and local radiation therapy after discharge. During follow-up, three patients developed multiple liver, brain, lung and bone metastases and died between 5 and 18 mo after the diagnosis. Three patients developed widespread metastasis disease and died between 18 and 37 mo after the diagnosis. There was no local tumor recurrence in these 6 patients. The other three patients were lost during follow-up.

CONCLUSION: Primary small cell carcinoma of the esophagus is a rare but very malignant tumor. Radical resection combined with chemotherapy and radiotherapy is helpful in limited stage cases.

Primary small cell carcinoma of esophagus: Report of 9 cases and review of literature

Primary small cell carcinoma of esophagus (SCCE) is a relatively rare and highly aggressive tumor characterized by early dissemination and poor prognosis. The optimal treatment has not yet been established, and the role of surgery has remained controversial. Most of the limited diseases were treated conventionally by surgery, but the five-year survival rate was still very low. This retrospective study was designed to investigate the clinical characteristics, treatment, and prognostic factors of limited disease SCCE. Clinical data of 40 SCCE patients with clinically limited disease who received transthoracic esophagectomy with lymphadenectomy at the Cancer Hospital of Shantou University Medical College from November 1990 to December 2009 were reviewed to summarize the clinical characteristics and treatment impacted on the survival. Twenty-five cases of the 40 patients were treated with surgery alone, eight cases were treated with surgery + postoperative chemotherapy, four cases were treated with surgery + postoperative radiotherapy, and the other three were treated with surgery + postoperative radiochemotherapy. The Kaplan-Meier and log-rank methods were used to estimate and compare survival rates. Cox's hazard regression model was used to identify the prognostic factors with the entry factors of gender, age (≤ 60 years versus > 60 years), length of the primary lesion (≤ 5 cm versus > 5 cm), location of the primary lesion, macroscopic tumor type, pT, pN, pTNM stage, operation (radical/palliative), and chemotherapy (yes/no). The mean follow-up duration of this series was 24.7 months (1-121 months). Thirty-four patients died of the disease during the follow-up, five were still alive, and one was lost of follow-up. The median survival time of the 40 patients was 13.0 months (95% confidence interval 4.7-21.3), and the 6-, 12-, 24-, 36-, and 60-month overall survival rates (OS) were 77.5%, 56.4%, 28.9%, 23.7%, 10.5%, respectively. In univariate analysis, age (≤ 60 years versus >60 years) (P=0.049), operation (radical/palliative) (P=0.008), and chemotherapy (yes/no) (P= 0.013) significantly influenced the OS of the SCCE patients. In multivariate analysis, operation (P=0.015) and chemotherapy (P=0.031) were independent prognostic factors. The patients who received radical surgery and postoperative chemotherapy had relatively better survival. Surgical resection combined with chemotherapy should be recommended to patients with limited disease SCCE.

Treatment and prognosis of limited disease primary small cell carcinoma of esophagus

https://www.annalsthoracicsurgery.org/article/S0003-4975(00)02528-5/pdf

Long-term survival after resection for small cell carcinoma of the esophagus

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Long-term survival after resection for small cell carcinoma of the esophagus