Humans ; Child ; Dermatofibrosarcoma/surgery* ; Skin Neoplasms/surgery* ; Oncogene Proteins, Fusion/genetics*

Dermatofibrosarcoma/surgery* ; Ear Auricle ; Ear, External ; Humans ; Skin Neoplasms/surgery*

Dermatofibrosarcoma protuberans (DFSP) is a slow growing low-grade cutaneous sarcoma. Local recurrence after excision is common due to the poorly defined periphery that renders histological control of surgical margin difficult, Mohs micrographic surgery is the optimal method for treatment. A 41 years old male patient, who had a previous history of DFSP, came to our dermatology clinic for evaluation of an asymptomatic firm flesh-colored nodule on the forehead. Total excision biopsy was done and the mass was histologically proved as DFSP. Wide excision with reconstruction was performed and showed no sign of recurrence till 18-month follow up. Local recurrence is known to be common for DFSP but a new visible lesion distant from the initial site may be confused as a de novo lesion or a benign neoplasm especially in scalp area, and thus interrupt early detection of DFSP. Herein, we report a case of recurrent DFSP of scalp which recurred distant from the original lesion.
Biopsy ; Dermatofibrosarcoma* ; Dermatology ; Follow-Up Studies ; Forehead ; Humans ; Male ; Methods ; Mohs Surgery ; Recurrence ; Sarcoma ; Scalp*

No abstract available.
Dermatofibrosarcoma* ; Mohs Surgery*

PURPOSE: Dermatofibrosarcoma protuberans (DFSP) carries a translocation resulting in the collagen type I alpha 1 (COL1A1)-platelet-derived growth factor beta (PDGFB) fusion gene, which is responsible for PDGFB activation. The purpose of this study is to evaluate the clinicopathological, genetic, and therapeutic features of DFSP in Korean patients. MATERIALS AND METHODS: Clinicopathological features of 37 patients with DFSP were reviewed. Multiplex reverse transcriptase-polymerase chain reaction (PCR) was carried out in 16 patients using formalin-fixed, paraffin-embedded tissues and specific primers for COL1A1 and PDGFB. RESULTS: The mean age of 37 patients was 37.4 years old. The most common tumor location was the trunk. All patients were treated primarily with surgery: 34 (91.7%) cases with Mohs micrographic surgery (MMS) and 3 (8.3%) cases with wide local excision. The median follow-up time was 33.7 months. Two patients, one in each treatment group, demonstrated local recurrence during the follow-up period. The COL1A1-PDGFB fusion gene was expressed in 14 (87.5%) cases, demonstrated by reverse transcriptase PCR analysis. No association was found among the different COL1A1-PDGFB fusion transcripts, the various histological subtypes and clinical features. CONCLUSION: Our results support the effectiveness of MMS in treating DFSP. The COL1A1-PDGFB fusion transcript was observed in 87.5% of patients. Therefore, COL1A1-PDGFB is a useful and accurate tool in diagnosing DFSP in Koreans.
Adolescent ; Adult ; Asian Continental Ancestry Group/*genetics ; Collagen Type I/*genetics ; DNA Primers ; Dermatofibrosarcoma/ethnology/*genetics/*pathology/surgery ; Female ; Humans ; Male ; Middle Aged ; Mohs Surgery ; Multiplex Polymerase Chain Reaction ; Neoplasm Recurrence, Local ; Oncogene Proteins, Fusion/*genetics ; Proto-Oncogene Proteins c-sis/*genetics ; Republic of Korea ; Reverse Transcriptase Polymerase Chain Reaction ; Skin Neoplasms/ethnology/*genetics/*pathology/surgery ; Treatment Outcome

PURPOSE: Dermatofibrosarcoma protuberans (DFSP) carries a translocation resulting in the collagen type I alpha 1 (COL1A1)-platelet-derived growth factor beta (PDGFB) fusion gene, which is responsible for PDGFB activation. The purpose of this study is to evaluate the clinicopathological, genetic, and therapeutic features of DFSP in Korean patients. MATERIALS AND METHODS: Clinicopathological features of 37 patients with DFSP were reviewed. Multiplex reverse transcriptase-polymerase chain reaction (PCR) was carried out in 16 patients using formalin-fixed, paraffin-embedded tissues and specific primers for COL1A1 and PDGFB. RESULTS: The mean age of 37 patients was 37.4 years old. The most common tumor location was the trunk. All patients were treated primarily with surgery: 34 (91.7%) cases with Mohs micrographic surgery (MMS) and 3 (8.3%) cases with wide local excision. The median follow-up time was 33.7 months. Two patients, one in each treatment group, demonstrated local recurrence during the follow-up period. The COL1A1-PDGFB fusion gene was expressed in 14 (87.5%) cases, demonstrated by reverse transcriptase PCR analysis. No association was found among the different COL1A1-PDGFB fusion transcripts, the various histological subtypes and clinical features. CONCLUSION: Our results support the effectiveness of MMS in treating DFSP. The COL1A1-PDGFB fusion transcript was observed in 87.5% of patients. Therefore, COL1A1-PDGFB is a useful and accurate tool in diagnosing DFSP in Koreans.
Adolescent ; Adult ; Asian Continental Ancestry Group/*genetics ; Collagen Type I/*genetics ; DNA Primers ; Dermatofibrosarcoma/ethnology/*genetics/*pathology/surgery ; Female ; Humans ; Male ; Middle Aged ; Mohs Surgery ; Multiplex Polymerase Chain Reaction ; Neoplasm Recurrence, Local ; Oncogene Proteins, Fusion/*genetics ; Proto-Oncogene Proteins c-sis/*genetics ; Republic of Korea ; Reverse Transcriptase Polymerase Chain Reaction ; Skin Neoplasms/ethnology/*genetics/*pathology/surgery ; Treatment Outcome

OBJECTIVE: To Summary the experience of surgical treatment for many times in 1 case of maxillofacial huge dermatofibrosarcoma protuberans. METHOD: The patient was diagnosed with dermatofibrosarcoma protuberans according to the pathological examination. From 2004 to 2011, the patient underwent extensive surgical resection, Mohs micrographic surgery(MMS) and free flap grafting method as surgical treatment for many times, and was closely followed-up after operation. RESULT: The tumor was completely removed in each operation, with wound skin graft survived. But relapse occured repeatedly, recurrence interval was from 7 months up to five years and eight months,recurrence interval gradually extended, all of the recurrence occurred at Mohs microsurgery cut edge. CONCLUSION Eexpanded resection is the preferred treatment for the early stage maxillofacial huge dermatofibrosarcoma protuberans. For advanced stage tumor, expanded resection should be combined with Mohs microsurgery. Free flap grafting was suggested in primary repair insteading of myocutaneous flap. At the same time, postoperative assistant radiotherapy, strengthen follow-up and early intervention were suggested.
Dermatofibrosarcoma ; surgery ; Facial Neoplasms ; surgery ; Free Tissue Flaps ; Humans ; Mohs Surgery ; Myocutaneous Flap ; Neoplasm Recurrence, Local ; surgery ; Reoperation ; Skin Neoplasms ; Skin Transplantation

Adult ; Dermatofibrosarcoma ; diagnosis ; surgery ; Humans ; Male ; Middle Aged ; Reconstructive Surgical Procedures ; methods ; Skin Neoplasms ; diagnosis ; surgery ; Thoracic Wall ; Young Adult

Skin neoplasms can result in large skin defects after surgical removal. Skin grafting has been used commonly to repair large defects, but it is associated with esthetic and functional problems. Herein, we report three cases with application of the keystone island flap in large skin defects of the trunk and extremities. A 53-year old male with recurred dermatofibrosarcoma protuberans on his thigh was repaired by the keystone island flap after Mohs micrographic surgery, and a 52-year old male with malignant melanoma on his back was successfully reconstructed by the keystone island flap after wide excision. Lastly, a 59-year old male diagnosed with dermatofibrosarcoma protuberans on the inguinal area was closed by the keystone island flap with commendable cosmetic outcome. Due to the ease of performance, excellent cosmetic and functional results, we recommend the keystone island flap for the reconstruction of large skin defects on the trunk and extremities.
Dermatofibrosarcoma ; Extremities ; Humans ; Male ; Melanoma ; Mohs Surgery ; Skin Neoplasms ; Skin Transplantation ; Skin* ; Thigh

BACKGROUND: Dermatofibrosarcoma protuberans is a mesenchymal tumor of the skin of intermediate-grade which is a rare condition. The slow growing and aggressive invasion on local tissues are characteristic features of dermatofibrosarcoma protuberans. The treatment for dermatofibrosarcoma protuberans is mainly a surgical excision such as a wide excision and Mohs micrographic surgery. OBJECTIVE: The aim of this study was to compare the result of wide excision and Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans at a single institution in Korea. METHODS: A retrospective review was done for 24 patients diagnosed with dermatofibrosarcoma protuberans and treated surgically from 1999 to 2010 at Chonbuk National University Hospital. Patient demographics, tumor features, surgical features, and recurrence during the follow-up period were evaluated. RESULTS: 13 patients were treated with wide excision, and 11 with Mohs micrographic surgery. There was no metastasis for all the cases. Mean operation time for the wide excision group was 83 minutes whereas 182 minutes for the Mohs micrographic surgery group, and it was a statistically significant difference. However, no significant difference was observed in post-operative defect size, advanced surgical repair and local recurrence in our study. CONCLUSION: We suggest that wide excision and Mohs micrographic surgery are both successful modalities for the surgical treatment of dermatofibrosarcoma protuberans. Hence, individualized patient and tumor characteristics should be concerned when determining the surgical options for dermatofibrosarcoma protuberans.
Demography ; Dermatofibrosarcoma ; Follow-Up Studies ; Humans ; Mohs Surgery ; Neoplasm Metastasis ; Recurrence ; Retrospective Studies ; Skin