Case Report of a Hemangioblastoma With Large Blood Vessels and Rare Vascular Anomalies: Is It Fibromuscular Dysplasia or Arteriovenous Malformation Association?

一例伴有大血管和罕见血管畸形的血管母细胞瘤病例报告:是纤维肌性发育不良还是动静脉畸形?

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Abstract

Hemangioblastoma is considered a benign neoplasm characterized by abnormal vasculature and stromal cells; several pathophysiological mechanisms have been proposed, such as genetic predisposition, hormonal factors, and arterial wall ischemia. Fibromuscular dysplasia is characterized by hyperplasia or thinning of the smooth muscle, elastic fibre destruction, fibrous tissue proliferation, and arterial wall disorganization. We present a cerebellar hemangioblastoma case not associated with Von Hippel Lindau syndrome. Histologically we evidenced big vessels with anomalies of the vascular walls corresponding to fibromuscular dysplasia, and those changes have not been described in these types of tumors. In this light, rare findings could be called vascular malformations or degenerative vascular changes, fibromuscular dysplasia or vascular anomalies. Arterio-venous malformation and hemangioblastoma pathology are rarely presented together. Notwithstanding, we could say that it is a stromal stem cell tumor in a varied stage of differentiation.

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