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肝脏未分化胚胎性肉瘤:1例成年患者的罕见病例报告

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

作者信息

Marques Ana M, Fontinha Guilherme, Ferreira Maria João, Sá Milene, Constantino Júlio, Pereira Jorge

机构信息

General Surgery Department, Centro Hospitalar Tondela-Viseu, Rei Dom Duarte, 3504-509 Viseu, Portugal.

Pathological Anatomy Department, Centro Hospitalar e Universitário de Coimbra, Praceta Professor Mota Pinto, 3004-561 Coimbra, Portugal.

出版信息

J Surg Case Rep. 2023 Oct 21;2023(10):rjad578. doi: 10.1093/jscr/rjad578. eCollection 2023 Oct.

Abstract

Undifferentiated embryonal sarcoma of the liver is a rare primary mesenchymal hepatic tumor that usually occurs in pediatric patients. In adulthood, this aggressive neoplasm represents only 7% of the liver sarcomas. This case reports a liver sarcoma occurring in a 49-year-old female patient. The patient was admitted in the emergency room with abdominal pain. Computerized tomography scan and magnetic resonance imaging showed a giant lobulated cystic mass in the right hepatic lobe, suggesting an atypical hemangioma. Right hepatectomy was performed. This rare case promotes a review of the differential diagnosis of liver primary neoplasms including sarcoma. The histological examination revealed an undifferentiated embryonal sarcoma. The patient underwent adjuvant chemotherapy. Currently, our patient is in complete sustained remission 4 years after chemotherapy.

摘要

肝未分化胚胎性肉瘤是一种罕见的原发性间质性肝脏肿瘤,通常发生于儿童患者。在成人中,这种侵袭性肿瘤仅占肝脏肉瘤的7%。本病例报告了一名49岁女性患者发生的肝脏肉瘤。该患者因腹痛入住急诊室。计算机断层扫描和磁共振成像显示右肝叶有一个巨大的分叶状囊性肿块,提示为非典型血管瘤。遂行右肝切除术。这个罕见病例促使我们对包括肉瘤在内的肝脏原发性肿瘤的鉴别诊断进行回顾。组织学检查显示为未分化胚胎性肉瘤。该患者接受了辅助化疗。目前,我们的患者在化疗后4年处于完全持续缓解状态。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e36/10590654/da808ea6e532/rjad578f1.jpg

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