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一名61岁女性预后不良的肝血管肉瘤:病例报告及文献综述

Liver Angiosarcoma with Poor Prognosis in a 61-Year-Old Woman: A Case Report and Literature Review.

作者信息

Manh Hung Tran, Tran Thi Phuong Thao

机构信息

Department of Surgery, Bach Mai Hospital, Hanoi, Vietnam.

Center for Population Health Sciences, Hanoi University of Public Health, Hanoi, Vietnam.

出版信息

Int J Womens Health. 2022 Jul 27;14:957-963. doi: 10.2147/IJWH.S369271. eCollection 2022.

Abstract

PURPOSE

Hepatic angiosarcoma is very rare malignancy and more common in men than in women. To date, only a few female cases of liver angiosarcoma have been reported. Here, we report a female case of liver angiosarcoma, first detected in Vietnam, with a high malignancy stage, rapid progression, and poor prognosis.

CASE PRESENTATION

A 61-year-old woman was admitted to the Bach Mai Hospital with fatigue, anorexia, weight loss, and severe pain in the right upper quadrant for 2 weeks prior. Clinical examination detected a firm 4-cm hepatomegaly below the right costal margin and grade I splenomegaly. Abdominal ultrasonography and CT revealed diffuse lesions in the entire liver parenchyma, spreading to the spleen, while MRI showed signs of bone metastasis. Blood tests showed elevated transaminase enzymes, especially Gamma Glutamyl Transferase 501 U/L; thrombocytopenia; no anemia; and other tumor markers such as AFP, CEA, and CA19-9 were within normal limits. On CT images, the dots and nodules in the liver and spleen appeared hyperenhanced in the arterial phase and washout in the venous phase. The results of both histopathology and immunohistochemistry showed liver angiosarcoma. Surgery and radiation were not indicated due to the suspicion of bone metastasis. Chemotherapy with doxorubicin at a dose of 60 mg/m2 and intravenous infusion once every 21 days was administered. Unfortunately, during the first dose of chemotherapy with doxorubicin, side effects appeared. Since the disease developed continuously and uncontrollably, the patient was subsequently exhausted, anemic, presented peritoneal fluid, and eventually died of intra-abdominal bleeding.

CONCLUSION

For the diagnosis of liver angiosarcoma, ultrasound-guided liver biopsy could be applied for safe and effective histopathology, and selective embolization of the hepatic artery is necessary to prevent bleeding complications. The disease has a very poor prognosis, and if chemotherapy does not respond, the patient can die within six months of diagnosis.

摘要

目的

肝血管肉瘤是一种非常罕见的恶性肿瘤,男性比女性更常见。迄今为止,仅有少数女性肝血管肉瘤病例被报道。在此,我们报告一例在越南首次发现的女性肝血管肉瘤病例,其恶性程度高、进展迅速且预后不良。

病例介绍

一名61岁女性因疲劳、厌食、体重减轻以及右上腹剧痛2周前入住白梅医院。临床检查发现右肋缘下有一个质地坚硬、直径4厘米的肝脏肿大以及I级脾肿大。腹部超声和CT显示整个肝实质弥漫性病变,并蔓延至脾脏,而MRI显示有骨转移迹象。血液检查显示转氨酶升高,尤其是γ-谷氨酰转移酶501 U/L;血小板减少;无贫血;其他肿瘤标志物如甲胎蛋白、癌胚抗原和CA19-9均在正常范围内。在CT图像上,肝脏和脾脏中的点状和结节状病变在动脉期呈高增强,在静脉期呈消退。组织病理学和免疫组化结果均显示为肝血管肉瘤。由于怀疑有骨转移,未进行手术和放疗。给予阿霉素化疗,剂量为60 mg/m²,每21天静脉输注一次。不幸的是,在首次使用阿霉素化疗时出现了副作用。由于疾病持续且无法控制地发展,患者随后出现衰竭、贫血、腹腔积液,最终死于腹腔内出血。

结论

对于肝血管肉瘤的诊断,超声引导下肝活检可用于安全有效的组织病理学检查,肝动脉选择性栓塞对于预防出血并发症是必要的。该疾病预后极差,如果化疗无反应,患者可在诊断后六个月内死亡。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d1e/9342427/16d9e49a694f/IJWH-14-957-g0001.jpg

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