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低恶性潜能软组织巨细胞瘤:关于软组织恶性巨细胞瘤重新分类的提议。

Soft tissue giant cell tumor of low malignant potential: a proposal for the reclassification of malignant giant cell tumor of soft parts.

作者信息

Folpe A L, Morris R J, Weiss S W

机构信息

Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA.

出版信息

Mod Pathol. 1999 Sep;12(9):894-902.

Abstract

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.

摘要

尽管“软组织巨细胞瘤”传统上被视为单一实体,如最初的术语“软组织恶性巨细胞瘤(MGCT)”以及后来的“巨细胞型恶性纤维组织细胞瘤”所反映的那样,但该组肿瘤的异型性程度和有丝分裂活性各不相同,提示生物学异质性。本文报告了31例符合MGCT传统标准但仅具有轻度至中度核异型性的肿瘤的临床病理特征。这些肿瘤患者(19例女性;12例男性)年龄在14至84岁之间(平均40岁),表现为累及浅表(n = 16)或深部(n = 13)软组织的肿块。大多数发生在手臂或手部(n = 16),大小从0.7至6.5 cm不等(平均2.1 cm)。肿瘤由圆形单核细胞的片状和结节状结构组成,与梭形细胞和良性破骨细胞样巨细胞混合存在。未见多形性巨细胞。10例可见骨样组织,但通常与腱鞘巨细胞瘤相关的特征(如致密的基质透明变性、含铁血黄素巨噬细胞和黄色瘤细胞)几乎总是不存在。有丝分裂象为1 - 10/10高倍视野(平均2 - 3/10高倍视野),10例存在血管淋巴管侵犯。然而,未见坏死。单核细胞表达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,免疫表型与骨巨细胞瘤相同。19例患者的随访信息(平均3年;中位数1 - 7年)显示4例复发,但均未发生转移。这种行为与传统上与软组织MGCT相关的高级别行为形成显著对比。即使存在有丝分裂活性和血管侵犯,这些巨细胞瘤也可因缺乏细胞学异型性而被一致识别。尽管其长期转移风险尚未完全明确,但我们建议将它们称为“低恶性潜能巨细胞瘤”,并视为骨巨细胞瘤的软组织类似物。“软组织恶性巨细胞瘤”或巨细胞恶性纤维组织细胞瘤这一术语应仅限于组织学上的高级别病变。

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