成人弥漫性肝血管瘤病
Adult diffuse hepatic hemangiomatosis.
作者信息
Bhardwaj Neha, Parkhi Mayur, Kumar Manish, Kaman Lileswar, Mitra Suvradeep
机构信息
Post Graduate Institute and Medical Education and Research (PGIMER), Department of Pathology, Chandigarh, India.
Post Graduate Institute and Medical Education and Research (PGIMER), Department of Histopathology, Chandigarh, India.
出版信息
Autops Case Rep. 2022 Sep 23;12:e2021401. doi: 10.4322/acr.2021.401. eCollection 2022.
Diffuse hepatic hemangiomatosis (DHH) is an uncommon vascular lesion, though hemangiomas are the commonest benign tumors of the liver. The etiology is largely unknown to date; however, its association with giant cavernous hemangiomas (GCH) has been reported in the literature. We present herein, the case of a 37-year-old hypothyroid woman with abdominal fullness for 2 months. The contrast-enhanced computed tomography revealed multiple well-encapsulated lesions involving the liver lobes and was diagnosed as giant cavernous hemangiomas. Most of them, except the deep-seated ones, were enucleated. Histopathological examination highlighted the presence of GCH with irregular margin, replacement of hepatic parenchyma, and presence of multiple micro-hemangiomas suggesting the possibility of DHH further substantiated by retrospective radiological assessment. No extrahepatic vascular lesion was noted, and the post-operative recovery and follow-up were uneventful. Adult DHH is an uncommon entity. The diagnosis of DHH and its distinction from GCH are important from the management and prognostic point of view as recurrence, extrahepatic manifestations, features of consumption coagulopathy, and death from the complications are not uncommon.
弥漫性肝血管瘤病(DHH)是一种罕见的血管病变,尽管血管瘤是肝脏最常见的良性肿瘤。迄今为止,其病因在很大程度上尚不清楚;然而,文献中已报道其与巨大海绵状血管瘤(GCH)有关。我们在此报告一例37岁甲状腺功能减退的女性,有2个月的腹部胀满症状。增强计算机断层扫描显示多个边界清晰的病变累及肝叶,诊断为巨大海绵状血管瘤。除深部病变外,大多数病变均被摘除。组织病理学检查显示存在边界不规则的GCH,肝实质被替代,并有多个微血管瘤,提示可能为DHH,回顾性放射学评估进一步证实了这一点。未发现肝外血管病变,术后恢复及随访情况良好。成人DHH是一种罕见的疾病。从管理和预后的角度来看,DHH的诊断及其与GCH的鉴别很重要,因为复发、肝外表现、消耗性凝血病的特征以及并发症导致的死亡并不少见。
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