Extrapleural solitary fibrous tumor: clinicopathologic study of 17 cases and molecular analysis of the p53 pathway
Extrapleural solitary fibrous tumor: clinicopathologic study of 17 cases and molecular analysis of the p53 pathway
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DOI：
10.1034/j.1600-0463.2000.d01-105.x
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发表时间：
2000-09-01
期刊：
影响因子：
2.8
通讯作者：
Hashimoto, H
中科院分区：
文献类型：
--
作者：
Morimitsu, Y;Nakajima, M;Hashimoto, H
由于其组织学变异性，发生在不同胸膜外部位的孤立性纤维瘤（SFT）有时难以诊断。虽然孤立性纤维瘤通常生长缓慢，预后良好，但也有少数恶性病例报道。在本研究中，我们检查了 17 例胸膜外 SFT 的临床行为、组织学、免疫组织化学和分子特征。 4例肿瘤位于盆腔，2例位于鼻腔，2例局限于肺实质，脑膜、肾脏、纵隔、腹膜后、颞区、颈部、腹股沟、臀部和大腿各各1例。在组织学上，所有肿瘤的特征是存在由平淡梭形细胞增殖和不同数量的厚的、通常透明样或瘢痕疙瘩样细胞间胶原束组成的区域。在三个特纳中观察到高细胞区域，在两个中观察到频繁的有丝分裂，在各一个中观察到细胞多形性和肿瘤坏死。所有 17 个肿瘤均表现出对 CD34 的免疫反应性，其中 15 个肿瘤（88%）对 bcl-2 蛋白表现出免疫反应性。 p53、mdma蛋白和Ki-67的标记指数普遍较低。 PCR-SSCP 和随后的 p53 基因序列分析揭示了所分析的 13 个病例之一的第 5 外显子密码子 161 处的点突变。根据随访信息，所有患者均未出现局部复发或远处转移。我们的结果表明，即使在组织学级别较高的组中，大多数胸膜外 SFT 也表现出良性，并且很难预测其临床结果。对于胸膜外 SFT 患者，建议进行完整手术切除以获得清晰的切缘并进行长期随访。
Solitary fibrous tumor (SFT) occurring at various extrapleural sites is sometimes difficult to diagnose because of its histologic variability. Although a solitary fibrous tumor is usually a slow-growing tumor with favorable prognosis, a small number of malignant cases have been reported. In the present study, we examined the clinical behavior, histologic, immunohistochemical and molecular features of 17 cases of extrapleural SFT. Four tumors were located in the pelvic cavity, two in the nasal cavity, two were confined to the pulmonary parenchyma, and there was one each in the meninges, kidney, mediastinum, retroperitoneum, temporal region, neck, groin, buttock and thigh. Histologically, all the tumors were characterized by the presence of areas consisting of a proliferation of bland spindle cells with variable amounts of thick, often hyalinized or keloid-like intercellular collagen bundles. Highly cellular areas were observed in three turners, frequent mitoses in two, and cellular pleomorphism and tumor necrosis in one each. All 17 tumors showed immunoreactivity to CD34 and 15 (88%) to bcl-2 protein. The labeling indices of p53, mdma protein and Ki-67 were generally low. PCR-SSCP and a subsequent sequence analysis of the p53 gene disclosed point mutation at codon 161 in exon 5 in one of the 13 cases analyzed. According to follow-up information, none of the patients had developed local recurrence or distant metastasis. Our results suggest that most extrapleural SFTs behave in a benign fashion even in a higher histologic grade group, and it is difficult to predict their clinical outcome. Complete surgical excision in order to obtain clear margins and long-term follow-up is advisable for patients with an extrapleural SFT.