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

OBJECTIVETo study the clinicopathologic characteristics of cellular fibrous histiocytoma (CFH) with emphasis on diagnosis and differential diagnosis.

METHODSClinical and pathologic features were reviewed in 27 cases of CFH (encountered during the period from 2008 to 2012) along with outcome analysis. Immunophenotyping was performed with EnVision method.

RESULTSThe patients included 13 males and 14 females. The age at presentation ranged from 15 to 61 years (mean, 34 years; median, 32 years). The tumor occurred in the extremities (n = 14), head and neck (n = 7), and trunk (n = 6). Histologically, the tumors were located in the dermis. Some cases showed wedge like extension into the subcutaneous adipose tissue. On high power, they consisted of dense fibroblasts and myofibroblasts. Other cell components such as psammoma-like histiocytes, hemosiderin-containing macrophages or touton-type giant cells were rare. The spindled tumor cells were arranged mostly in intersecting fascicles. Focal storiform architecture was not uncommon. In addition, a few cases showed prominent hemangiopericytoma-like pattern. There was no prominent cellular atypia but increased mitotic figures were not difficult to find. Two cases exhibited necrosis. By immunohistochemistry, the tumor cells showed variable expression of alpha smooth muscle actin. CD34 positive cells were present in some cases, but were distributed mostly in the periphery or bottom of the lesions. They were all negative for desmin, h-caldesmon, S-100 protein and cytokeratin. Follow-up in 19 cases revealed local recurrences in 5 cases and bilateral pulmonary metastases in 1 case after repeated recurrences.

CONCLUSIONSCFH is a cellular form of benign fibrous histiocytoma which has a risk for local recurrence after incomplete excision. Distant metastasis can occur in rare examples. However, there were no morphological parameters predicting the risk of recurrence or metastasis. Increased awareness of the clinocopathological features and immunophenotypes of CFH is helpful in avoiding misdiagnosing the disease as malignant tumors, especially dermatofibrosarcoma protuberans.

Actins ; metabolism ; Adolescent ; Adult ; Antigens, CD34 ; metabolism ; Dermatofibrosarcoma ; metabolism ; pathology ; Diagnosis, Differential ; Extremities ; Female ; Follow-Up Studies ; Head and Neck Neoplasms ; metabolism ; pathology ; surgery ; Histiocytoma, Benign Fibrous ; metabolism ; pathology ; secondary ; surgery ; Humans ; Lung Neoplasms ; secondary ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Retrospective Studies ; Skin Neoplasms ; metabolism ; pathology ; surgery ; Young Adult

Adult ; Antigens, CD ; metabolism ; Antigens, CD34 ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Dermatofibrosarcoma ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Female ; Fibroma ; metabolism ; pathology ; Humans ; Lipoma ; pathology ; Neurofibroma ; metabolism ; pathology ; Receptors, Cell Surface ; metabolism ; Skin Neoplasms ; metabolism ; pathology ; surgery