Nonaka Daisuke, Rodriguez Jaime, Rosai Juan
Department of Pathology, New York University Medical Center, 560 First Avenue, New York, NY 10016, USA.
Am J Surg Pathol. 2007 Oct;31(10):1545-51. doi: 10.1097/PAS.0b013e3180457bfc.
Hemangioblastoma is a morphologically distinctive tumor that can occur sporadically or in association with von Hippel-Lindau disease, and which involves the central nervous system in the majority of the cases. Rare occurrences of hemangioblastoma in peripheral nerves and extraneural tissues have been reported. The histogenesis of this tumor remains uncertain. Various cell lineages such as vascular, glial, neural, fibrohistiocytic, and smooth muscle/myofibroblastic have been proposed for the so-called stromal cells, which are thought to represent the neoplastic component of these lesions. We report on 5 cases of hemangioblastoma arising in extraneural tissues. Two of the tumors were located in the presacral region, and one each in the maxilla, kidney, and adrenal glands. All 5 cases were morphologically indistinguishable from central nervous system hemangioblastoma. The existence of these cases suggests that the "stromal" cells of hemangioblastoma can demonstrate a variety of mature specific lineages, such as smooth muscle/myofibroblastic, or neuroendocrine, depending on the location and possibly the microenvironment.
血管母细胞瘤是一种形态独特的肿瘤，可散发性发生或与冯·希佩尔-林道病相关，且在大多数病例中累及中枢神经系统。已有报道血管母细胞瘤罕见地发生于外周神经和神经外组织。该肿瘤的组织发生仍不确定。对于所谓的基质细胞，已提出多种细胞谱系，如血管、神经胶质、神经、纤维组织细胞和平滑肌/肌纤维母细胞谱系，这些基质细胞被认为代表了这些病变的肿瘤成分。我们报告了5例发生于神经外组织的血管母细胞瘤。其中2例肿瘤位于骶前区，上颌骨、肾脏和肾上腺各有1例。所有5例在形态上与中枢神经系统血管母细胞瘤无法区分。这些病例的存在表明，血管母细胞瘤的“基质”细胞可根据位置以及可能的微环境表现出多种成熟的特定谱系，如平滑肌/肌纤维母细胞或神经内分泌谱系。
