Department of Dermatology, Instituto Valenciano de Oncología, Valencia, Spain; Department of Pathology, Hospital Clínico Universitario, Valencia, Spain.
Department of Pathology, Hospital Clínico Universitario, Valencia, Spain.
J Am Acad Dermatol. 2011 Sep;65(3):564-575. doi: 10.1016/j.jaad.2010.06.020. Epub 2011 May 12.
Dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous tumor, usually low grade, except for the fibrosarcomatous variant (DFSP-FS).
We sought to compare the clinicopathological, immunohistochemical, genetic, and therapeutic features between DFSP and DFSP-FS.
The clinicopathological features were reviewed in 63 DFSP and 12 DFSP-FS. Immunohistochemistry and multiplex reverse transcriptase-polymerase chain reaction were carried out using formalin-fixed, paraffin-embedded tissue, using specific primers for collagen type I alpha 1 (COL1A1) and platelet-derived growth factor beta (PDGFB).
DFSP-FS was associated with tumor history longer than 5 years (P = .009), tumor size greater than 4 cm (P = .001), more stages of modified Mohs micrographic surgery (P = .005), expansive subcutaneous infiltration (P = .005), muscular invasion (P = .0001), absence of CD34 staining (P = .018), p53 positivity (P = .006), and increased proliferative activity (P = .004) compared with DFSP. The COL1A1-PDGFB fusion transcript was found in 100% DFSP-FS and 72% DFSP. No association was found between the different COL1A1-PDGFB fusion transcripts and the different histologic subtypes. Wide local excision (2 cm) was performed in 47% of cases and modified Mohs micrographic surgery in 53%. After a mean follow-up of 73 months (range 21-235), 6 patients had local recurrence (5 DFSP, 1 DFSP-FS) and one died of disease (DFSP-FS). The only factor related to local recurrence was the type of surgery (17% wide local excision vs 0% modified Mohs micrographic surgery) (P = .006).
Our study is retrospective. Prospective studies are necessary to confirm our results.
DFSP-FS reflects tumor progression in DFSP, with larger size, particular invasive patterns, p53 expression, and increased proliferative activity. However, as in low-grade DFSP, appropriate surgery permits a tumor-free excision. COL1A1-PDGFB is a useful tool for diagnosis of DFSP and particularly for DFSP-FS.
隆突性皮肤纤维肉瘤（DFSP）是一种罕见的皮肤肿瘤，通常为低级别，纤维肉瘤型（DFSP-FS）除外。
我们旨在比较 DFSP 和 DFSP-FS 的临床病理、免疫组化、遗传和治疗特征。
回顾性分析 63 例 DFSP 和 12 例 DFSP-FS 的临床病理特征。使用福尔马林固定、石蜡包埋组织，使用 COL1A1 和 PDGFB 特异性引物进行免疫组化和多重逆转录-聚合酶链反应。
DFSP-FS 与肿瘤病史超过 5 年（P =.009）、肿瘤大小大于 4cm（P =.001）、改良Mohs 显微镜手术阶段更多（P =.005）、扩张性皮下浸润（P =.005）、肌肉侵犯（P =.0001）、CD34 染色缺失（P =.018）、p53 阳性（P =.006）和增殖活性增加（P =.004）相关。100%的 DFSP-FS 和 72%的 DFSP 中发现 COL1A1-PDGFB 融合转录本。不同 COL1A1-PDGFB 融合转录本与不同组织学亚型之间无关联。47%的病例行广泛局部切除术（2cm），53%行改良 Mohs 显微镜手术。平均随访 73 个月（21-235 个月）后，6 例患者发生局部复发（5 例 DFSP，1 例 DFSP-FS），1 例患者死亡（DFSP-FS）。与局部复发相关的唯一因素是手术类型（17%广泛局部切除术 vs 0%改良 Mohs 显微镜手术）（P =.006）。
本研究为回顾性研究。需要前瞻性研究来证实我们的结果。
DFSP-FS 反映了 DFSP 中的肿瘤进展，具有更大的体积、特殊的侵袭模式、p53 表达和增殖活性增加。然而，与低级别 DFSP 一样，适当的手术可实现肿瘤无残留切除。COL1A1-PDGFB 是诊断 DFSP 特别是 DFSP-FS 的有用工具。
