Kamińska I, Szczepulska-Wójcik E, Rudziński P, Orłowski T, Langfort R
Zakład Patomorfologii IGiChP.
Pneumonol Alergol Pol. 2001;69(5-6):290-4.
Hemangiopericytoma (HPC) is a rare neoplasm arising from pericytes that occur mostly around smaller vessels. Up to now only about 100 cases have been reported to arise primarily in the lung. The behavior of pulmonary hemangiopericytomas is difficult to predict and all tumors should be considered potentially malignant, even though the criteria for malignancy are not well developed. The diagnosis of HPC is known to confound even experienced pathologist. Pericytes lack readily identifiable morphologic features, therefore hemangiopericytomas are often confused with other soft tissue tumors that may have hemangiopericytoma--like pattern. We report a rare case of primary HPC of the lung with an asymptomatic, long course of the disease. The diagnosis of hamartoma was established after the first operation. Subsequently, seven years later, a chest radiograph revealed new lesions. Histological examination, including immunohistochemistry lead to the final diagnosis of hemangiopericytoma. The small number of cases of primary pulmonary hemangiopericytoma makes it difficult to define the correct histopathological diagnosis especially without modern methods.
血管外皮细胞瘤（HPC）是一种罕见的肿瘤，起源于主要分布在小血管周围的周细胞。截至目前，仅有约100例主要发生于肺部的病例报告。肺血管外皮细胞瘤的行为难以预测，所有肿瘤都应被视为具有潜在恶性，尽管恶性标准尚未完善。已知HPC的诊断即使对经验丰富的病理学家来说也具有挑战性。周细胞缺乏易于识别的形态学特征，因此血管外皮细胞瘤常与其他可能具有血管外皮细胞瘤样模式的软组织肿瘤相混淆。我们报告一例罕见的原发性肺HPC病例，该病例病程无症状且较长。首次手术后诊断为错构瘤。随后，七年后，胸部X线片显示出新的病变。组织学检查，包括免疫组织化学检查，最终确诊为血管外皮细胞瘤。原发性肺血管外皮细胞瘤病例数量较少，使得准确的组织病理学诊断难以确定，尤其是在没有现代方法的情况下。
