A Case of Hepatic Malignant Solitary Fibrous Tumor: A Case Report and Review of the Literature.

IF 0.7 Q4 PATHOLOGY
Zhiyan Fu, Evita B Henderson-Jackson, Barbara A Centeno, Gregory Y Lauwers, Mihaela Druta, Daniel A Anaya, Yukihiro Nakanishi
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Abstract

A 73-year-old man with a history of atrial myxoma and basal cell carcinoma presented with unexplained fever. Contrast-enhanced CT abdomen showed a large left hepatic lobe mass with early enhancement and delayed venous washout, concerning for hepatocellular carcinoma. Fine needle aspiration showed numerous spindle cells with malignant nuclear features, suggestive of malignant spindle cell neoplasm. The patient underwent left hepatectomy. The surgical specimen showed a well-circumscribe solid mass (14.6 × 13.0 × 10.0 cm) with necrosis. Histopathological examination revealed a proliferation of spindle tumor cells with characteristic staghorn-shaped blood vessels, frequent mitoses, and necrosis. The tumor cells showed strong and diffuse expression of CD34 and STAT6, confirming the diagnosis of malignant solitary fibrous tumor. Solitary fibrous tumor is a rare fibroblastic tumor characterized by a staghorn vasculature and NAB2-STAT6 gene rearrangement. Solitary fibrous tumor of the liver is a rare occurrence. Although most solitary fibrous tumors behave in a benign fashion, solitary fibrous tumors might act aggressively. This case is unique in that it demonstrates an excellent correlation between radiologic, macroscopic, and microscopic features which can contribute to the improvement of radiologic and pathologic diagnostic accuracy.

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肝脏恶性孤立性纤维性肿瘤1例报告及文献复习。
一位73岁男性,有心房黏液瘤和基底细胞癌病史,表现为不明原因的发热。腹部增强CT显示左侧肝叶大肿块,早期增强,迟发性静脉冲洗,可能为肝细胞癌。细针穿刺示大量梭形细胞伴恶性核征,提示恶性梭形细胞肿瘤。患者行左肝切除术。手术标本显示一个边界良好的固体肿块(14.6 × 13.0 × 10.0 cm)伴坏死。组织病理学检查显示梭形肿瘤细胞增生,伴有特征性的鹿角状血管,频繁的有丝分裂和坏死。肿瘤细胞中CD34和STAT6表达强烈且呈弥漫性表达,证实为恶性孤立性纤维性肿瘤。孤立性纤维性肿瘤是一种罕见的纤维母细胞肿瘤,其特征为鹿角状血管和NAB2-STAT6基因重排。单发的肝纤维性肿瘤是罕见的。虽然大多数孤立性纤维性肿瘤表现为良性,但孤立性纤维性肿瘤可能具有侵袭性。该病例的独特之处在于它显示了放射学、宏观和微观特征之间的良好相关性,这有助于提高放射学和病理学诊断的准确性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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