Fibrosarcomatous ("high-grade") dermatofibrosarcoma protuberans - Clinicopathologic and immunohistochemical study of a series of 41 cases with emphasis on prognostic significance
Fibrosarcomatous ("high-grade") dermatofibrosarcoma protuberans - Clinicopathologic and immunohistochemical study of a series of 41 cases with emphasis on prognostic significance
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DOI：
10.1097/00000478-199805000-00009
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发表时间：
1998-05-01
影响因子：
5.6
通讯作者：
Fletcher, CDM
中科院分区：
文献类型：
--
作者：
Mentzel, T;Beham, A;Fletcher, CDM
隆突性皮肤纤维肉瘤的纤维肉瘤变异型（FS-DFSP）是一种不常见的DFSP，其纤维肉瘤成分对预后的影响仍有争议。我们分析了41例患者的临床病理和免疫组化特征。患者年龄范围为8 - 87岁（中位数为48岁，19例患者为女性）。躯干25个病灶，上肢6个，下肢4个，5个肿瘤位于头颈部; 1例患者的确切解剖来源不明。27例肿瘤仅累及真皮和皮下组织，10例累及更深的组织，1例发生在乳房，3例无法确定病变的确切深度。术前持续时间范围为1个月至60年（中位数，3年）。26例肿瘤局部切除，边缘清晰，7例广泛切除，3例不完全切除，4例病灶切除。在1例病例中，确切治疗不详。此外，对3名患者进行了放射治疗，对1名患者进行了化疗。组织学上，病变显示与纤维肉瘤区域相邻的典型低度DFSP区域。4例单纯DFSP复发为单纯纤维肉瘤，2例FS-DFSP局部复发为单纯DFSP。纤维肉瘤样改变在原发病灶中比复发病灶中更常见（3.6：1）。21例纤维肉瘤占< 30% in 6 cases to >肿瘤组织的70%。在19例病例中观察到两种成分之间的突然过渡。7例纤维肉瘤显示局灶性坏死，与普通DFSP区域相比，有丝分裂率更高（10个高倍视野中平均13.4对2.3个有丝分裂）。其他组织学特征包括2例复发病例进展为多形性肉瘤，1例黑色素沉着细胞（Bednar FS-DFSP），13例局灶性粘液样改变，3例斑块或瘢痕疙瘩样透明变性，9例肌样束和结节。免疫组化显示DFSP区肿瘤细胞CD 34染色阳性，而FS-DFSP区仅15/33例CD 34阳性。41例患者中34例（平均90个月，中位数36个月）随访显示20例（58%）局部复发（5例患者复发2次或2次以上），5例肺转移。5例患者（14.7%）中观察到1例骨和1例软组织）。至今死于肿瘤2例（5.8%）。FS-DFSP中坏死、高有丝分裂率（每10个高倍视野中有丝分裂> 10个）和多形性区域的存在往往与不良临床结局相关，但未检测到统计学显著相关性。DFSP中的纤维肉瘤性变化代表了DFSP中肿瘤进展的一种形式，并且与比普通DFSP显著更具侵袭性的临床病程相关，表明在此类病例中可能需要强化治疗。
The fibrosarcomatous variant of dermatofibrosarcoma protuberans (FS-DFSP) represents an uncommon form of DFSP, in which the prognostic influence of the fibrosarcomatous component is still debated. We analyzed the clinicopathologic and immunohistochemical features in a series of 41 patients. Patient age ranged from 8 to 87 years (median, 48 years, and 19 patients were female. Twenty five lesions were seen on the trunk, 6 on the upper limbs, and 4 on the lower limbs, and five neoplasms were located in the head/neck region; in one case, exact anatomic sire was unknown. Twenty seven tumors involved purely dermal and subcutaneous tissues, in 10 casts, deeper structures were also involved, 1 case arose in the breast, and, in 3 cases, it was impossible to define exact depth of the lesion. Preoperative duration ranged from 1 month to 60 years (median, 3 years). Twenty six tumors were excised locally with clear margins, 7 were treated by wide excision, 3 by incomplete excision, and, in 4 patients, the lesion was shelled our. In one case, exact treatment was unknown. in addition, radiotherapy was administered in three cases and chemotherapy in one case. Histologically, the lesions showed areas of typical, low-grade DFSP adjacent to fibrosarcomatous areas. Ln four cases, a previously ordinary DFSP recurred as pure fibrosarcoma, in two cases, local recurrence of FS-DFSP showed features of ordinary DFSP. Fibrosarcomatous change was more common in the primary (de novo) lesions than in recurrent lesions (3.6:1). Proportion of fibrosarcoma varied between < 30% in 6 cases to > 70% of tumor tissue in 21 cases. An abrupt transition between both components was seen in 19 cases. The fibrosarcomatous component showed focal necrosis in seven cases and showed a higher mitotic rate in comparison with ordinary DFSP areas (mean, 13.4 versus 2.3 mitoses in 10 high-power fields). Additional histologic features included progression to pleomorphic sarcoma in 2 recurrent cases, melanin-pigmented cells (Bednar FS-DFSP) in 1 case, focal myxoid change in 13 cases, plaque or keloidlike hyalinization in 3 cases, and myoid bundles and nodules in 9 cases. Immunohistochemically, tumor cells in DFSP areas stained positively for CD34, whereas, in FS-DFSP areas, only 15 out 33 cases were positive for CD34. Follow-up in 34 of 41 patients (mean, 90 months; median, 36 months) revealed local recurrence in 20 patients (58%) (recurrence occurred in 5 patients on two or more occasions), Metastases (5 lung. 1 bone, and 1 soft tissue) were seen in 5 patients (14.7%). and 2 patients have died of tumor to date (5.8%). Necrosis, high mitotic rate (> 10 mitoses per 10 high-power fields), and presence of pleomorphic areas in FS-DFSP tended to be related with poor clinical outcome, but no statistically significant association was detected. Fibrosarcomatous change in DFSP represents a form of tumor progression in DFSP and is associated with a significantly more aggressive clinical course than in ordinary DFSP, indicating a possible need for treatment intensification in such cases.