Markogiannakis Haridimos, Theodorou Dimitrios, Toutouzas Konstantinos G, Larentzakis Andreas, Pattas Michael, Bousiotou Angeliki, Papacostas Pavlos, Filis Konstantinos, Katsaragakis Stilianos
Department of Propaedeutic Surgery, Hippokrateion Hospital, Athens Medical School, University of Athens, Athens, Greece.
J Med Case Rep. 2008 Jan 22;2:15. doi: 10.1186/1752-1947-2-15.
Gastrointestinal tract small cell carcinoma is an infrequent and aggressive neoplasm that represents 0.1-1% of gastrointestinal malignancies. Very few cases of small cell esophageal carcinoma arising in Barrett's esophagus have been reported in the literature. An extremely rare case of primary small cell carcinoma of the distal third of the esophagus arising from dysplastic Barrett's esophagus is herein presented.
A 62-year-old man with gastroesophageal reflux history presented with epigastric pain, epigastric fullness, dysphagia, anorexia, and weight loss. Esophagogastroscopy revealed an ulceroproliferative, intraluminar mass in the distal esophagus obstructing the esophageal lumen. Biopsy showed small cell esophageal carcinoma. Contrast-enhanced chest and abdominal computed tomography demonstrated a large tumor of the distal third of the esophagus without any lymphadenopathy or distant metastasis. Preoperative chemotherapy with cisplatine and etoposide for 3 months resulted in a significant reduction of the tumor. After en block esophagectomy with two field lymph node dissection, proximal gastrectomy, and cervical esophagogastric anastomosis, the patient was discharged on the 14th postoperative day. Histopathology revealed a primary small cell carcinoma of the distal third of the esophagus arising from dysplastic Barrett's esophagus. The patient received another 3 month course of postoperative chemotherapy with the same agents and remained free of disease at 12 month review.
Although small cell esophageal carcinoma is rare and its association with dysplastic Barrett's esophagus is extremely infrequent, the high carcinogenic risk of Barrett's epithelium should be kept in mind. Prognosis is quite unfavorable; a better prognosis might be possible with early diagnosis and treatment strategies incorporating chemotherapy along with oncological radical surgery and/or radiotherapy as part of a multimodality approach. Since treatment protocols are not well established due to the rarity of the neoplasm, multi-institutional studies are needed to obtain sufficiently large populations for investigation and optimization of therapy of the disease.
胃肠道小细胞癌是一种罕见且侵袭性强的肿瘤，占胃肠道恶性肿瘤的0.1%-1%。文献中报道的起源于巴雷特食管的小细胞食管癌病例极少。本文介绍了一例极其罕见的起源于发育异常的巴雷特食管的食管远端三分之一原发性小细胞癌病例。
一名有胃食管反流病史的62岁男性，出现上腹部疼痛、饱胀、吞咽困难、厌食和体重减轻症状。食管胃镜检查显示食管远端有一个溃疡增生性腔内肿物，阻塞食管腔。活检显示为小细胞食管癌。胸部和腹部增强计算机断层扫描显示食管远端三分之一处有一个大肿瘤，无任何淋巴结肿大或远处转移。术前使用顺铂和依托泊苷进行3个月的化疗使肿瘤显著缩小。在进行整块食管切除术、两野淋巴结清扫、近端胃切除术和颈部食管胃吻合术后，患者于术后第14天出院。组织病理学显示起源于发育异常的巴雷特食管的食管远端三分之一原发性小细胞癌。患者接受了另外3个月相同药物的术后化疗，在12个月复查时仍无疾病复发。
尽管小细胞食管癌罕见，且其与发育异常的巴雷特食管的关联极为罕见，但应牢记巴雷特上皮的高致癌风险。预后相当不佳；早期诊断并采用包括化疗以及肿瘤根治性手术和/或放疗的多模式治疗策略可能会有更好的预后。由于该肿瘤罕见，治疗方案尚未完全确立，因此需要多机构研究以获得足够大的人群进行疾病治疗的调查和优化。
