Soft tissue giant cell tumor of low malignant potential: a proposal for the reclassification of malignant giant cell tumor of soft parts.
Soft tissue giant cell tumor of low malignant potential: a proposal for the reclassification of malignant giant cell tumor of soft parts.
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低度恶性潜能软组织巨细胞瘤：软组织恶性巨细胞瘤重新分类的建议。
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发表时间：
1999
期刊：
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通讯作者：
S. Weiss
中科院分区：
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作者：
A. Folpe;R. Morris;S. Weiss
尽管“软组织巨细胞瘤”传统上被认为是单个实体，如最初的术语“软组织的恶性巨细胞瘤”和后来的术语“恶性纤维组织细胞瘤，巨细胞型”所反映的，但在这一组中，异型性和有丝分裂活性的程度各不相同，表明生物异质性。本文报告了31例符合传统MGCT诊断标准但仅有轻度至中度核异型性的肿瘤的临床病理特征。女性19例，男性12例，年龄14~84岁，平均40岁，表现为浅表软组织肿块16例，深层软组织肿块13例。大多数发生在手臂或手部(n=16)，大小0.7~6.5 cm(平均2.1 cm)。肿瘤由片状和结节组成，圆形单核细胞与梭形细胞和良性破骨细胞巨细胞混合在一起。未见多形性巨细胞。10例有骨样改变，但通常与腱滑膜巨细胞瘤相关的特征(如致密的间质透明、噬铁质细胞和黄色瘤细胞)几乎总是缺失。核分裂像范围为1~10/10HPF(平均2~3/10高倍视野)，10例有血管淋巴系侵犯。然而，没有出现坏死。单个核细胞表达CD68、抗酒石酸酸性磷酸酶和平滑肌肌动蛋白，但缺乏CD45、S100蛋白、结蛋白和溶菌酶，其免疫表型与骨巨细胞瘤相同。19例患者的随访资料(平均3年，中位数1-7年)显示有4例复发，但无一例发生转移。这一行为与传统上与软零件的MGCT相关的高级行为形成了鲜明对比。即使在有丝分裂活动和血管侵犯的情况下，这些巨细胞瘤也可以通过缺乏细胞学上的异型性而被一致识别。虽然它们的长期转移风险尚未完全确定，但我们建议将其命名为“低恶性潜能的骨巨细胞瘤”，并将其视为骨巨细胞瘤的软组织类似物。术语“软组织部位恶性巨细胞瘤”或巨细胞恶性纤维组织细胞瘤应仅限于组织学高度分级的病变。
Although "giant cell tumor of soft parts" has traditionally been considered a single entity as reflected in the original term "malignant giant cell tumor of soft parts (MGCT)" and later by the term "malignant fibrous histiocytoma, giant cell type" the degree of atypia and mitotic activity varies in this group, suggesting biologic heterogeneity. The clinicopathologic features of 31 tumors meeting the traditional criteria of MGCT but having only mild to moderate nuclear atypia are presented. Patients with these tumors (19 females; 12 males) ranged in age from 14 to 84 years (mean, 40 years) and presented with masses of involving either superficial (n = 16) or deep (n = 13) soft tissue. Most occurred on the arm or hand (n = 16) and ranged in size from 0.7 to 6.5 cm (mean, 2.1 cm). The tumors consisted of sheets and nodules of rounded mononuclear cells that blended with spindled cells and benign osteoclastic giant cells. Pleomorphic giant cells were absent. Osteoid was noted in 10 cases, but features typically associated with tenosynovial giant cell tumors (such as dense stromal hyaline, siderophages, and xanthoma cells) were nearly always absent. Mitotic figures ranged from 1-10/10 HPF (mean, 2-3/10 high-powered field), and angiolymphatic invasion was present in 10 cases. Necrosis was absent, however. The mononuclear cells expressed CD68, tartrate-resistant acid phosphatase, and smooth muscle actin, but lacked CD45, S100 protein, desmin, and lysozyme, an immunophenotypic profile identical to that of giant cell tumor of bone. Follow-up information in 19 patients (mean, 3 yrs; median, 1-7 yrs) indicated recurrences in four patients, but none developed metastasis. This behavior contrasts significantly with the high-grade behavior traditionally associated with MGCT of soft parts. These giant cell tumors can be consistently recognized by the lack of cytologic atypia even in the face of mitotic activity and vascular invasion. Although their long term metastatic risk is not fully defined, we propose they be termed "giant cell tumors of low malignant potential" and regarded as the soft tissue analogue of giant cell tumor of bone. The term "malignant giant cell tumor of soft parts" or giant cell malignant fibrous histiocytoma should be restricted to histologically high-grade lesions.