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肝血管平滑肌脂肪瘤:6例病例系列,重点关注病理与放射学的相关性以及经粗针活检诊断出的不寻常变异型

Hepatic angiomyolipoma: a series of six cases with emphasis on pathological-radiological correlations and unusual variants diagnosed by core needle biopsy.

作者信息

Agaimy Abbas, Vassos Nikolaos, Croner Roland S, Strobel Deike, Lell Michael

机构信息

Institute of pathology, University Hospital, Erlangen, Germany.

出版信息

Int J Clin Exp Pathol. 2012;5(6):512-21. Epub 2012 Jul 29.

Abstract

Hepatic angiomyolipoma is rare and may pose differential diagnostic difficulty, particularly if encountered in core needle biopsy. We studied 6 cases from 5 males and one female (median age, 48.6 yrs). All presented with non-specific symptoms or an incidentally discovered tumor mass. Two patients had a remote history of chemotherapy for hematological neoplasms (acute lymphoblastic leukemia and Hodgkin lymphoma respectively) and another had clear cell renal cell carcinoma and anaplastic pancreatic carcinoma diagnosed at autopsy without definable syndrome. None of the patients had evidence of the tuberous sclerosis complex or renal or other extra-renal angiomyolipoma. Three tumors were resected completely and three have been only biopsied and followed up. None of the resected cases recurred at a mean follow-up of 35 months. Histologically, tumors were classified as classical triphasic (1), lipomatous (2), epithelioid/oncocytoid (1), epithelioid trabecular (1) and myelolipoma-like (1). The adjacent liver parenchyma was normal in 3 cases, showed pigment cirrhosis in one case and mild fatty change in another case. One case had clinically diagnosed but histologically unverified cirrhosis. The initial diagnostic impression/frozen section was misleading in 5 of the cases and included vascular lesion, focal fatty change, myelolipoma, hepatocellular tumor and oncocytic neoplasm. All tumors expressed HMB45 and variably desmin. One epithelioid lesion expressed HMB45 and TFE3, but lacked desmin expression. In conclusion, hepatic angiomyolipomas are increasingly recognized as incidental findings during surveillance for cirrhosis or investigations for unrelated conditions. Awareness of their diverse morphological spectrum in liver biopsy is necessary to avoid misdiagnosis as hepatocellular carcinoma, metastatic melanoma or other malignant neoplasms.

摘要

肝血管平滑肌脂肪瘤较为罕见,可能会造成鉴别诊断困难,尤其是在粗针活检中遇到时。我们研究了来自5名男性和1名女性(中位年龄48.6岁)的6例病例。所有患者均表现为非特异性症状或偶然发现的肿瘤肿块。两名患者分别有血液系统肿瘤(急性淋巴细胞白血病和霍奇金淋巴瘤)的远期化疗史,另一名患者在尸检时被诊断为透明细胞肾细胞癌和间变性胰腺癌,无明确综合征。所有患者均无结节性硬化症或肾或其他肾外血管平滑肌脂肪瘤的证据。3例肿瘤被完全切除,3例仅进行了活检并随访。在平均35个月的随访中,所有切除病例均未复发。组织学上,肿瘤分为经典三相型(1例)、脂肪瘤型(2例)、上皮样/嗜酸性细胞型(1例)、上皮样小梁型(1例)和髓脂肪瘤样(1例)。3例患者的相邻肝实质正常,1例表现为色素性肝硬化,另1例表现为轻度脂肪变性。1例临床诊断为肝硬化但组织学未证实。5例病例的初始诊断印象/冰冻切片具有误导性,包括血管病变、局灶性脂肪变性、髓脂肪瘤、肝细胞肿瘤和嗜酸性细胞瘤。所有肿瘤均表达HMB45,结蛋白表达不一。1例上皮样病变表达HMB45和TFE3,但缺乏结蛋白表达。总之,肝血管平滑肌脂肪瘤越来越多地在肝硬化监测或无关疾病检查中作为偶然发现被识别。认识到它们在肝活检中的多种形态谱对于避免误诊为肝细胞癌、转移性黑色素瘤或其他恶性肿瘤是必要的。

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