隆突性皮肤纤维肉瘤的诊断治疗与研究进展

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2003～2009年,在福建省肿瘤医院接受治疗且资料完整的49例隆突性皮肤纤维肉瘤临床诊治过程。结果 49例隆突性皮肤纤维肉瘤中,行扩大切除术45例,扩大切除加植皮或转移皮瓣修补术18例,术前放疗1例。全组病例复发率为8.16%,2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,复发率较高,首次治疗时彻底切除为关键。外科手术是其主要的治疗方法。     

Adequate surgical margins for dermatofibrosarcoma protuberans – A multi-centre analysis

BackgroundDermatofibrosarcoma protuberans (DFSP) is a locally aggressive tumour. Adequate margins have a positive impact on recurrence rates. The aim of this study is to assess how adequate margins are achieved and secondly which additional treatment modalities might be necessary to achieve adequate margins.Material & methodsPatients with DFSP treated between 1991 and 2016 at three tertiary centres were included. Patient- and tumour characteristics were obtained from a prospectively held database and patient files.ResultsA total of 279 patients with a median age of 39 (Interquartile range [IQ], 31–50) years and a median follow-up of 50 (IQ, 18–96) months were included. When DFSP was preoperatively confirmed by biopsy and resected with an oncological operation in a tertiary centre, in 86% was had clear pathological margins after one excision. Wider resection margins were significantly correlated with more reconstructions (p = 0.002). A substantial discrepancy between the primary surgical macroscopic and the pathological margins was found with a median difference of 22 (range, 10–46) mm (Fig. 1). There was no significant influence of the width of the pathological clear margins (if > 1 mm) and the recurrence rate (p = 0.710).ConclusionThe wider the resection margins, the more likely it is to obtain clear pathological margins, but the more likely patients will need any form of reconstruction after resection. The aim of the primary excision should be wide surgical resection, where the width of the margin should be balanced against the need for reconstructions and surgical morbidity.     

FNA diagnosis of giant cell fibroblastoma: A case report of an unusual pediatric soft tissue tumor

Giant cell fibroblastoma (GCF) is a rare pediatric soft tissue tumor, which exists on a spectrum with dermatofibrosarcoma protuberans (DFSP). Histologic features are well established for these entities; however, cytologic findings have not been well characterized. We report for the first time a case of GCF, confirmed by cytogenetics, with mixed DFSP features. In this case of an 8‐month‐old boy, a fine needle aspiration specimen showed a low‐grade spindle cell tumor, with oval to spindled cells dispersed singly and in patternless groups, and with occasional giant cells. Subsequent histologic features were consistent with GCF, which is an uncommon, CD34 positive, soft tissue neoplasm with a distinct molecular aberration. This case emphasizes the differential diagnosis in pediatric soft tissue tumors and stresses the unique features of GCF. Diagn. Cytopathol. 2015;43:325–328. © 2014 Wiley Periodicals, Inc.     

Guidelines of the French Society of Otorhinolaryngology (SFORL), short version. Extension assessment and principles of resection in cutaneous head and neck tumors

Cutaneous head and neck tumors mainly comprise malignant melanoma, squamous cell carcinoma, trichoblastic carcinoma, Merkel cell carcinoma, adnexal carcinoma, dermatofibrosarcoma protuberans, sclerodermiform basalioma and angiosarcoma. Adapted management requires an experienced team with good knowledge of the various parameters relating to health status, histology, location and extension: risk factors for aggression, extension assessment, resection margin requirements, indications for specific procedures, such as lateral temporal bone resection, orbital exenteration, resection of the calvarium and meningeal envelopes, neck dissection and muscle resection.