Clinicophathologic Analysis of 28 Cases of Dermatofibrosarcoma Protuberans
-
摘要:
目的探讨隆突性皮肤纤维肉瘤的临床病理特征。 方法对28例隆突性皮肤纤维肉瘤从临床表现、病理形态及免疫组化等方面进行观察分析。 结果28例隆突性皮肤纤维肉瘤好发于中青年男性，临床多表现为皮肤固定的结节状肿物，手术切除不完全易局部复发。病理组织学表现为梭形细胞排列成典型的车辐状或席纹状。免疫组化所有病例Vimentin及CD34均阳性，S-100均阴性。其中2例局部伴高级别肉瘤转化，转化区域CD34表达减弱或不均匀。 结论CD34有助于DFSP与其他皮肤梭形细胞肿瘤的鉴别，当CD34表达减弱时需注意观察是否局部伴高级别肉瘤转化。 Abstract:Objective To study the clinicophathologic characteristics of dermatofibrosarcoma protuberans (DFSP). Methods Clinical pathological features and immunohistochemical findings were observed in 28 cases of DFSP. Results The majority of the patients were young to middle-aged men. The tumor usually presented as a firm nodule, with a high frequency of recurrence after incomplete excision. The spindle-shaped cells were typically arranged in a storiform or a cartwheel growth pattern. In this study, all cases of DFSPs tended to stain positively for Vimentin and CD34 and negatively for S-100. DFSP with high-grade sarcomatous transformation was observed in 2 cases, in which CD34 was weak. Conclusion CD34 is helpful for us to distinguish DFSP from other cutaneous spindle cell tumors. When CD34 is weak or uneven in some areas, attention should be paid to observing if the DFSP contains a high-grade sarcomatous component. -
[1] Darier J, Ferrand M. Dermatofibromas progressifs et recidivants oufibrosarcomes de la peau[J]. Ann Dermatol Syph, 1924(9): 545-6. [2] 王坚, 朱雄增. 软组织肿瘤病理学[M]. 北京: 人民卫生出版社, 2008:178-83. [3] Manganoni AM, Pavoni L, Gualdi G, et al. Dermatofibrosarcomaprotuberans in an adolescent: a case report and review of theliterature[J]. Pediatr Hematol Oncol, 2013, 35(5): 383-7. doi: 10.1097/MPH.0b013e3182843edc [4] John R, Andrew L, Sharon W. Goldblum enzinger and weiss's Softtissue tumors,sixth edition[M]. china: elsevier press, 2014: 397. [5] Llombart B, Serra-Guillen C, Monteagudo CA, et al.Dermatofibrosarcoma protuberans: a comprehensive review andupdate on diagnosis and management[J]. Semin Diagn Pathol,2013, 30(1): 13-28. doi: 10.1053/j.semdp.2012.01.002 [6] Llombart B, Monteagudo C, Sanmartin OA, et al. Dermatofibrosarcomaprotuberans: A clinicopathological, immunohistochemical, genetic(COL1A1-PDGFB), and therapeutic study of low-grade versushigh-grade (fibrosarcomatous) tumors[J]. J Am Acad Dermatol,2011, 65(3): 564-75. doi: 10.1016/j.jaad.2010.06.020 [7] Abbott JJ, Oliveira AM, Nascimento AG. The prognosticsignificance of fibrosarcomatous transformation indermatofibrosarcoma protuberans[J]. Am J Surg Pathol, 2006, 30(4): 436-43. doi: 10.1097/00000478-200604000-00002 [8] Swaby MG, Evans HL, Fletcher CD, et al. Dermatofibrosarcomaprotuberans with unusual sarcomatous transformation: a series of 4cases with molecular confirmation[J]. Am J Dermatopathol, 2011,33(4): 354-60. doi: 10.1097/DAD.0b013e3182014631 -
下载:
点击查看大图
图(4)
计量
- 文章访问数: 1409
- HTML全文浏览量: 372
- PDF下载量: 21
- 被引次数: 0
