Middleton L P, Duray P H, Merino M J
Laboratory of Pathology, National Cancer Institute, Bethesda, MD 20892, USA.
Hum Pathol. 1998 Jun;29(6):636-40. doi: 10.1016/s0046-8177(98)80015-4.
Hemangiopericytoma (HPC) is an uncommon vascular neoplasm thought to be derived from pericytes. Prediction of patient outcome is difficult based what is currently known about these tumors and histological parameters alone. We compiled 27 cases of HPC and evaluated the spectrum of histological features to investigate whether there was any correlation between histology, immunostaining, prognostic markers, and patient outcome. The following parameters were evaluated: vasculature, histological pattern (solid, myxoid, trabecular, alveolar), degree of cellular pleomorphism, necrosis, mitoses, and giant cell content. Immunohistochemistry was performed to determine the reactivity for CD 31, CD34, vimentin, actin, cytokeratin, S100, actin, and SMA. Proliferative rate was analyzed using antibodies to PCNA and MIB1. Patient's age ranged from 8 months to 75 years (mean, 35; median, 31). Twenty of 27 cases were located in the extremities. The tumors were grossly described as lobulated and well circumscribed (n=12) and nonencapsulated (n=15). By histology, the characteristic ramifying or staghorn vasculature pattern was seen in all cases. A solid histological pattern was mixed with an alveolar pattern in three cases, trabecular pattern in six cases, and myxoid pattern in two cases. Tumor cells were uniform, polygonal to spindle-shaped, often with vesicular nuclei. Tumor giant cells were present in 9 of 27 cases; necrosis, in 11 of 27. Mitoses ranged from 0 to 14 per 10 high-power fields (HPF). Cellular pleomorphism was 1+ in nine cases, 2+ in 12 cases, and 3+ in six cases. Immunohistochemistry showed reactivity for CD34 and vimentin in all cases. Actin was focally positive in one case, and SMA was focally positive in another. CD 31, cytokeratin, and S100 were uniformly nonreactive. Proliferative index measured by PCNA and MIBI ranged between less than 1% and 40% of tumor cells. Follow-up was available in 22 cases and ranged from 1 year to 15 years. Seven patients had metastases, and two recurred locally. Thirteen patients had no evidence of disease at last checkup. Parameters associated with recurrences or metastases include a trabecular pattern, the presence of necrosis, mitoses, vascular invasion, and cellular pleomorphism. Features associated with an aggressive biological behavior can be identified histologically. There was some, but not total, correlation between proliferative markers and tumor aggressiveness.
血管外皮细胞瘤（HPC）是一种罕见的血管肿瘤，被认为起源于周细胞。仅根据目前对这些肿瘤的了解和组织学参数来预测患者的预后是困难的。我们收集了27例HPC病例，评估了组织学特征谱，以研究组织学、免疫染色、预后标志物与患者预后之间是否存在任何相关性。评估了以下参数：脉管系统、组织学模式（实性、黏液样、小梁状、肺泡状）、细胞多形性程度、坏死、有丝分裂和巨细胞含量。进行免疫组织化学以确定对CD 31、CD34、波形蛋白、肌动蛋白、细胞角蛋白、S100、肌动蛋白和平滑肌肌动蛋白（SMA）的反应性。使用针对增殖细胞核抗原（PCNA）和MIB1的抗体分析增殖率。患者年龄范围为8个月至75岁（平均35岁；中位数31岁）。27例中有20例位于四肢。肿瘤大体上描述为分叶状且边界清晰（n = 12）和无包膜（n = 15）。通过组织学检查，在所有病例中均可见特征性的分支状或鹿角状脉管系统模式。3例中实性组织学模式与肺泡状模式混合，6例与小梁状模式混合，2例与黏液样模式混合。肿瘤细胞均匀一致，呈多边形至梭形，细胞核常呈泡状。27例中有9例存在肿瘤巨细胞；27例中有11例存在坏死。每10个高倍视野（HPF）的有丝分裂数范围为0至14个。9例细胞多形性为1+，12例为2+，6例为3+。免疫组织化学显示所有病例中CD34和波形蛋白均有反应性。1例肌动蛋白局灶性阳性，另1例SMA局灶性阳性。CD 31、细胞角蛋白和S100均无反应性。通过PCNA和MIBI测量的增殖指数在肿瘤细胞的不到1%至40%之间。22例有随访资料，随访时间为1年至15年。7例患者发生转移，2例局部复发。13例患者在最后一次检查时无疾病证据。与复发或转移相关的参数包括小梁状模式、坏死的存在、有丝分裂、血管侵犯和细胞多形性。可以通过组织学识别与侵袭性生物学行为相关的特征。增殖标志物与肿瘤侵袭性之间存在一定但不完全的相关性。
