An unusual clinical presentation of myxoid dermatofibrosarcoma protuberans with a prominent vasculature: A potential pitfall in the diagnosis of myxoid soft tissue tumors

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor that arises primarily on the trunk and extremities but seldom on the scalp. Several variants of DFSP have been described, including myxoid DFSP. Although typical DFSP may have focally myxoid areas, myxoid DFSP, in which most of the stroma is myxoid, is rare and can pose diagnostic challenges. Here, we report a case of myxoid DFSP with an unusual clinical presentation that could have been mistaken for a lipoma. Additionally, the myxoid DFSP displayed prominent vasculature in a myxoid stroma, which could have been mistaken for a myxoid liposarcoma.     

119��¡͹��Ƥ����ά�����ٴ�����

目的 进一步提高对隆凸性皮肤纤维肉瘤（DFSP）的认识，探讨正确和规范手术治疗的重要性。方法对119例隆凸性皮肤纤维肉瘤，进行临床和病理资料的回顾性分析。结果 119例中有111例（占93.3% )在入院前被误诊为良性肿瘤而行局部切除术，经补充广泛切除，病理检查最终明确局部有肿瘤残留者57例，占51.4%，其中40例（占70.2%)在查体或B超检查时未能发现残留灶；12例（占10.1%)出现纤维肉瘤样改变（DFSP-FS），其中10例见于局部切除或广泛切除后复发的病例（复发次数1-13次不等）。结论 为避免误诊，临床医师有必要进一步提高对DFSP的认识；在局部切除术后，一经病理检查确诊为DFSP，必须施行补充广泛切除以避免肿瘤残留；为减少复发，宜重视首次手术的正确治疗和广泛切除手术的规范治疗。     

Immunohistochemical Analysis of CXCR4 Expression in Fibrohistiocytic Tumors

Functional chemokine receptors are expressed in many malignant tumors. These receptors promote tumor growth and metastasis in response to endogenous chemokines. We analyzed the expression of CXCR4, CCR6 and CCR7 in fibrohistiocytic tumors, including dermatofibrosarcoma protuberance (DFSP), malignant fibrous histiocytoma (MFH), dermatofibroma (DF) using immunohistochemistry. We also investigated the relationship between CXCR4 and CD34, the latter of which is an immunohistochemical marker for DFSP. We observed a higher expression of CXCR4 in DFSP and MFH as compared with DF. Interestingly, a significantly higher expression of CXCR4 was detected in relapsed DFSP than in non-relapsed DFSP, but no significant differences were detected between non-relapsed DFSP and DFSP with CD34 immunostaining. Moreover, MFH had strong immunoreactivity for CXCR4, CCR6 and CCR7. These findings suggest that the assessment of CXCR4 immunoreactivity in fibrohistiocytic tumors is a useful tool for predicting tumor aggressiveness.     

隆突性皮肤纤维肉瘤85例临床分析

目的：探讨隆突性皮肤纤维肉瘤的临床特点和治疗方法。方法：回顾性分析85例隆突性皮肤纤维肉瘤临床诊治过程。结果：85例隆突性皮肤纤维肉瘤行扩大切除术45例，扩大切除加植皮或转移皮瓣修补术34例，术前放疗2例，术后合并放疗10例，全组病例复发率为64．7％，5例死于肿瘤转移，结论：隆突性皮肤纤维肉瘤是皮肤低度恶性肿瘤，复发率高，首次治疗时彻底切除极为关键，外科手术是其主要的治疗方法，治疗作为辅助治疗有一定疗效。     

Intracranial extension of a dermatofibrosarcoma protuberans of the scalp: a case report with brief review of literature

Intracranial extension of dermatofibrosarcoma protuberans occurring in the scalp and metastasis to the brain from a distant DFSP are rare phenomena. However, because of the possibility of such an occurrence, wide excision of the tumor with 2.5–3 cm margins and close monitoring of patients is suggested.     

隆突性皮肤纤维肉瘤诊断与治疗

目的：探讨隆突性皮肤纤维肉瘤（DFSP）的诊断和治疗方法。方法：对28例经病理证实不同部位隆突性皮肤纤维肉瘤患者的诊治情况进行回顾性分析。躯干部16例（57％）,四肢部8例（28．5％）,头颈部4例（14．2）。5例行局部切除术,23例扩大切除术＋植皮或皮瓣转移修复术。16例在术后行放射治疗,平均照射剂量60Gy（50～70Gy）。结果：切除28例标本,CD34阳性26例,阴性2例。术后随访1～5年（平均2．3年）,失访2例。复发9例,平均复发时间2．6年（8个月～5年）。28例均未见有远处转移,无死亡。结论：隆突性皮肤纤维肉瘤是一种低度恶性肿瘤,行扩大切除术＋辅助放疗是治疗隆突性皮肤纤维肉瘤的主要治疗方法,手术范围大小及术后辅助放疗与否是影响隆突性皮肤纤维肉瘤的独立预后因素,可有效提高患者生存率及生存质量。     

隆突性皮肤纤维肉瘤16例临床分析

目的 探讨隆突性皮肤纤维肉瘤(DFSP)的临床特点及治疗。方法 收集我院经组织病理检查确诊的DFSP16例，并对其进行分析。结果 本组病例术前诊断为DFSP3例，误诊7例，术后复发6例，复发2次以上2例，转移1例。结论 DFSP是一种好发于真皮的低度恶性软组织肿瘤，局部手术切除后复发率较高，宜采用扩大切除术治疗。     

Dermatofibrosarcoma protuberans-derived fibrosarcoma: clinical history, biological profile and sensitivity to imatinib

Dermatofibrosarcoma Protuberans (DFSP) carries a translocation resulting in the COL1A1/PDGFB fusion-gene, responsible for platelet derived growth factor beta receptor (PDGFRB) activation. Fibrosarcomatous (FS) transformation in DFSP rarely occur. The fusion-gene and PDGFRB expression/activation pattern and imatinib role in DFSP-derived FS is less defined. We reviewed all consecutive patients operated for localized DFSP at our institution from 1994 to 2009, selecting cases with FS component. We also reviewed patients treated with imatinib for advanced FS-DFSP over the same period. When cryopreserved material was available, biochemical/molecular analyses were performed. Of 275 DFSPs, 13 (4.7%) showed a FS component. Fifteen percent of these patients developed metastases, one to the brain. Four patients with DFSP-derived FS received imatinib, with a Response Evaluation Criteria in Solid Tumor Partial Response. Response was followed by early secondary progression in two. One died for brain metastases. Three patients underwent surgery after imatinib. The fusion-gene was detected in all cases in both the classical and FS component, before and after imatinib. PDGFRB expression/activation was confirmed in all cases. mTOR was switched-off, despite the phosphorylation of its effectors. However, a strong phosphorylation of S6 and 4EBP1 was restricted to the FS component. In conclusion, DFSP-derived FS maintains the fusion-gene, being sensitive to imatinib. However, responses are short-lasting. Secondary resistance to imatinib is not related to PDGFRB.