Malignant Extrarenal Rhabdoid Tumors of the Omentum with Hemoperitoneum: Report of a Case

網膜之腎臟外類橫紋肌惡性瘤導致血腹是一件相當稀少且不尋常的事情。我們於此報告一位25歲男性因網膜之腎臟外類橫紋肌惡性瘤自發性破裂導致血腹的病例。這位病患因十一天以來食慾不振且伴隨亦趨嚴重的腹脹來到急診，腹部電腦斷層發現網膜及腸系膜周圍處有軟組織浸潤之現象，強烈懷疑是網膜餅，並且有一富含血管且中央成低衰減性之肝臟腫瘤。血管攝影沒發現任何出血點。但住院後，越來越嚴重的血腹加重血液產生低血容且提高了腹腔內的壓力。在緊急剖腹探查後，發現有7,000 CC含血塊的血於腹腔內，網膜餅合併腹膜內腫瘤轉移，且持續不斷從腫瘤表面緩慢出血。在切除整塊網膜餅後，免疫化學分析發現細胞角蛋白(cytokeratin)及波形蛋白(vimentin)成陽性反應。然而在CD31、CD34、肌間線蛋白(desmin)及INI1呈現陰性反應。病人在術後殘存腫瘤並無出血的狀況，然而接受化學治療（ifosfamide及carboplatin）後卻導致腫瘤出血的狀況，最終病人不治而死亡。

關鍵字

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並列摘要

Malignant extrarenal rhabdoid tumors (MERT) of the omentum which results in massive hemoperitoneum is extremely rare. We herein report a case of a 25 year-old male with a spontaneously ruptured MERT from the omentum presenting with hemoperitoneum. The patient was brought to our emergency department with an 11- day history of poor appetite and progressive abdominal distension. An abdominal computed tomography scan revealed soft tissue infiltration into the omentum and mesenteric fat of the bowel loop, which caused suspicion of omentum cake formation, and a hypervascular liver mass with a hypoattenuated center was also noted. Abdominal angiography showed no active bleeders. After admission, progressive hemoperitoneum with exacerbated hypovolemia and elevated intraabdominal pressure were noted. At emergent laparotomy, massive hemoperitoneum of about 7,000 ml, and an omentum cake with diffuse peritoneal tumor seeding and oozing from the surface of the tumor were noted. Debulking resection of the omental tumor was done. Immunohistochemical examination revealed a tumor positive for cytokeratin and vimentin, but negative for CD31, CD34, desmin and INI1. The patient had an uneventful postoperative course without residual tumor bleeding. However, tumor bleeding induced by chemotherapy with ifosfamide and carboplatin occurred.

並列關鍵字

malignant rhabdoid tumor ； hemoperitoneum ； extrarenal rhabdoid tumor ； omentum

國際替代計量

Malignant Extrarenal Rhabdoid Tumors of the Omentum with Hemoperitoneum: Report of a Case

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