Furukawa Kenichiro, Niihara Masahiro, Kawata Takuya, Mayanagi Shuhei, Tsubosa Yasuhiro
Division of Esophageal Surgery, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi-cho, Sunto-gun, Shizuoka, 411-8777, Japan.
Division of Pathology, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi-cho, Sunto-gun, Shizuoka, 411-8777, Japan.
Surg Case Rep. 2020 Jan 20;6(1):26. doi: 10.1186/s40792-020-0785-4.
Carcinosarcoma of the esophagus or esophagogastric junction (EGJ) is a rare malignancy with both carcinomatous and sarcomatous components. There is no report of carcinosarcoma arising from the EGJ wherein the carcinomatous element was adenocarcinoma. We describe a patient with carcinosarcoma of the EGJ in which the carcinomatous element was adenocarcinoma.
A 52-year-old man was diagnosed with carcinoma on his EGJ after complaining of appetite loss. All tumor markers (carcinoembryonic antigen, squamous cell carcinoma antigen, alpha-fetoprotein, and carbohydrate antigen 19-9) were within the respective normal ranges. Esophagogastroduodenoscopy showed a 150-mm (100 mm esophageal side and 50 mm gastric side) type 1 tumor on his EGJ. A histopathological examination of a biopsy specimen revealed well-differentiated tubular adenocarcinoma at the gastric side; however, only necrotic tissue was noted on the esophageal side. Contrast-enhanced computed tomography did not reveal any invasion of the adjacent structures; however, it did show five swollen regional lymph nodes. F-Fluorodeoxyglucose positron emission tomography with computed tomography did not reveal distant metastases. We performed thoracic subtotal esophagectomy, total gastrectomy, and two-field plus left cervical paraesophageal lymphadenectomy. Macroscopically, the lesion consisted of two components: a 7.5-cm type 2 tumor and a 9-cm type 1 tumor at the proximal end of the type 2 tumor. Microscopically, the type 2 tumor showed predominantly solid or cribriform proliferation of tumor cells with clear cytoplasm, which was moderately differentiated adenocarcinoma with enteroblastic-like differentiation. The tumor cells of the adenocarcinoma component had periodic acid-Schiff (PAS)-positive globules and were positive for sal-like protein 4 (SALL 4) and negative for α-fetoprotein (AFP) or human epidermal growth factor receptor type 2 (HER2). The type 1 tumors consisted of the adenocarcinoma-like type 2 tumor and spindle cells (sarcomatous component). Part of the sarcomatous component showed cartilage differentiation. The type 2 and type 1 lesions were continuous lesions. The epicenter of the tumor was located at the EGJ. The adenocarcinoma component was present in 10 of 27 resected lymph nodes. The tumor was diagnosed as carcinosarcoma of the EGJ.
We report a rare patient with carcinosarcoma of the EGJ wherein the carcinomatous element was adenocarcinoma.
食管或食管胃交界（EGJ）的癌肉瘤是一种罕见的恶性肿瘤，具有癌性和肉瘤性成分。目前尚无关于起源于EGJ且癌性成分是腺癌的癌肉瘤的报道。我们描述了一名患有EGJ癌肉瘤且癌性成分是腺癌的患者。
一名52岁男性因食欲减退就诊，被诊断为EGJ癌。所有肿瘤标志物（癌胚抗原、鳞状细胞癌抗原、甲胎蛋白和糖类抗原19-9）均在各自正常范围内。食管胃十二指肠镜检查显示EGJ处有一个150毫米（食管侧100毫米，胃侧50毫米）的1型肿瘤。活检标本的组织病理学检查显示胃侧为高分化管状腺癌；然而，食管侧仅见坏死组织。增强计算机断层扫描未显示对相邻结构的侵犯；但显示有五个肿大的区域淋巴结。氟脱氧葡萄糖正电子发射断层扫描与计算机断层扫描未显示远处转移。我们进行了胸段次全食管切除术、全胃切除术和二野加左颈段食管旁淋巴结清扫术。大体上，病变由两个部分组成：一个7.5厘米的2型肿瘤和一个位于2型肿瘤近端的9厘米1型肿瘤。显微镜下，2型肿瘤主要表现为肿瘤细胞呈实性或筛状增生，胞质清亮，为中度分化腺癌，具有成肠细胞样分化。腺癌成分的肿瘤细胞有过碘酸希夫（PAS）阳性小球，盐样蛋白4（SALL 4）阳性，甲胎蛋白（AFP）或人表皮生长因子受体2（HER2）阴性。1型肿瘤由类似腺癌的2型肿瘤和梭形细胞（肉瘤性成分）组成。部分肉瘤性成分显示软骨分化。2型和1型病变为连续病变。肿瘤的中心位于EGJ。27个切除淋巴结中有10个存在腺癌成分。该肿瘤被诊断为EGJ癌肉瘤。
我们报告了一名罕见的EGJ癌肉瘤患者，其癌性成分是腺癌。
