Reiner S A, Siegel G J, Clark K F, Min K W
Dept. of O.R.L., University of Oklahoma, Oklahoma City.
Rhinology. 1990 Jun;28(2):129-36.
Hemangiopericytomas are unusual vascular tumours that rarely occur in the paranasal sinuses and nasal cavity. They are thought to arise from pericytes that surround capillaries, however, there is no proven etiology of these tumours. The diagnosis cannot be made based on gross morphologic characteristics nor on frozen section biopsy. Instead, the diagnosis is dependent on careful histologic examination and reticulum staining. The diagnosis can be confirmed by electron microscopy. The histologic picture is greatly varied from tumour to tumour and within a given tumour itself. The clinical course of hemangiopericytomas ranges from benign to malignant with possible distant metastases and cannot be reliably predicted by histologic criteria. Hemangiopericytomas of the nose and paranasal sinuses are thought to behave less aggressively than those occurring in other parts of the body. Lymph node metastasis is rare and elective neck dissection is not indicated. The accepted treatment of these tumours is wide surgical excision, however adequate surgical margins are usually difficult in the sinonasal region. As a result, hemangiopericytomas can exhibit a high recurrence rate. Therefore, it is mandatory that these patients be followed carefully for the remainder of their lives.
血管外皮细胞瘤是一种罕见的血管肿瘤，很少发生于鼻窦和鼻腔。它们被认为起源于围绕毛细血管的周细胞，然而，这些肿瘤尚无已证实的病因。其诊断不能基于大体形态特征或冰冻切片活检做出。相反，诊断依赖于仔细的组织学检查和网状纤维染色。通过电子显微镜检查可确诊。不同肿瘤之间以及同一肿瘤内部的组织学表现差异很大。血管外皮细胞瘤的临床病程从良性到恶性不等，可能发生远处转移，且无法通过组织学标准可靠预测。鼻窦和鼻腔的血管外皮细胞瘤被认为比发生于身体其他部位的血管外皮细胞瘤侵袭性小。淋巴结转移罕见，不建议进行选择性颈部清扫术。这些肿瘤公认的治疗方法是广泛手术切除，然而在鼻窦区域通常难以获得足够的手术切缘。因此，血管外皮细胞瘤可能具有较高的复发率。所以，必须对这些患者进行终身密切随访。
