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一名成年患者的肝脏未分化胚胎性肉瘤:病例报告

Undifferentiated embryonal sarcoma of the liver in an adult patient: case report.

作者信息

Varol Umut, Karaca Burçak, Muslu Uğur, Doğanay Latife, Değirmenci Mustafa, Uslu Rüçhan, Göker Erdem

机构信息

Department of Medical Oncology, Ege University Faculty of Medicine, İzmir, Turkey.

出版信息

Turk J Gastroenterol. 2012 Jun;23(3):279-83. doi: 10.4318/tjg.2012.0339.

Abstract

Undifferentiated embryonal sarcoma of the liver is a rare and aggressive tumor in adults, with an unfavorable prognosis. We present a 33-year-old female patient who was admitted with fatigue, weight loss and right upper abdominal pain. Magnetic resonance imaging of the liver revealed a bulky malignant mass with necrotic and cystic center, which nearly occupied the left lobe and invaded the middle-left hepatic vein and left portal vein. The patient was evaluated as inoperable, and in order to determine the histologic diagnosis, a Tru-cut biopsy was performed. Both histopathologic features and the immunocytochemical stainings revealed the diagnosis of hepatic embryonal sarcoma. Since the patient had no chance of surgery due to the advanced stage of the tumor and progressive hepatic failure, a combination chemotherapy was applied. Unfortunately, the patient did not respond to treatment at all and died in the second post-therapy week. Undifferentiated embryonal sarcoma of the liver is a highly chemo-sensitive tumor. Radical resection may be possible after combination chemotherapy in the childhood period, but since the disease is extremely rare in adults, an optimal treatment approach is still unknown.

摘要

肝未分化胚胎性肉瘤是一种在成人中罕见且侵袭性强的肿瘤,预后不良。我们报告一名33岁女性患者,因疲劳、体重减轻和右上腹疼痛入院。肝脏磁共振成像显示一个巨大的恶性肿块,中心有坏死和囊性变,几乎占据左叶并侵犯左中肝静脉和左门静脉。该患者被评估为无法手术,为明确组织学诊断,进行了粗针活检。组织病理学特征和免疫细胞化学染色均确诊为肝胚胎性肉瘤。由于肿瘤分期较晚且出现进行性肝衰竭,患者已无手术机会,遂给予联合化疗。不幸的是,患者对治疗完全无反应,在治疗后第二周死亡。肝未分化胚胎性肉瘤是一种对化疗高度敏感的肿瘤。儿童期联合化疗后可能可行根治性切除,但由于该疾病在成人中极为罕见,最佳治疗方法仍不明确。

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