- 期刊
Undifferentiated Embryonal Sarcoma of the Liver in an Adolescent with Mesocolon Involvement Mimicking a Colon Tumor with Liver Invasion
肝臟未分化胚胎肉瘤合併結腸繫膜侵犯疑似結腸腫瘤
摘要
肝臟未分化胚胎肉瘤是一個少見且由間葉細胞起源之高度惡性腫瘤。此腫瘤最常發生在6－10歲之幼兒時期，但也有零星成人個案曾被報告。此一名稱最早是由Stocker與Ishak在1978年描述並認定為臨床病理學上之獨特分類。在小兒肝臟腫瘤中，未分化胚胎肉瘤是第三位常見之惡性腫瘤約佔有13%。病患常以非特異性症狀如發燒、腹痛，及可觸摸之右上腹腫瘤來表現，而其實驗室檢查通常為正常且無可信賴之腫瘤標記可供參考。影像學檢查通常可發現巨大，異質性，及低血管性之病灶。此腫瘤最早被認為較具侵襲性且預後差，但近幾年長期存活已被發現於接受完整切除合併術前或術後化學治療之病人族群身上。此病例報告為一18歲年輕成人患有肝臟未分化胚胎肉瘤合併結腸繫膜侵犯之外科完整切除治療經驗。
並列摘要
Undifferentiated embryonal sarcoma (UES) of the liver is an uncommon and highly malignant tumor of mesenchymal origin. It almost exclusively occurs in childhood with a peak incidence between 6-10 years of age but rare occurrences in adults have also been reported. The synonym was first described by Stocker and Ishak in 1978 as a distinct clinicopathologic entity. In reviews of pediatric liver tumor, UES was found to be the third most common malignant liver tumor accounting for 13% of hepatic malignancy. UES patients often show nonspecific symptoms including fever, abdominal pain, and RUQ palpable mass with normal laboratory study and no reliable tumor marker has been confirmed. Imaging study often reveals large, heterogenous, and hypovascular lesion. Tumors are thought to be aggressive with very poor prognosis initially but long term survival following complete resection with neo- and/or adjuvant chemotherapy has been reported in recent years. We report a case of UES with invasion to mesocolon in an 18-year-old young adult who was treated at our institution with combined liver resection and right hemicolectomy.