• Archives of Plastic Surgery
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• 제37권3호
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• pp.285-288
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• 2010

Purpose: Dermatofibrosarcoma protuberans is a rare tumor, accounting for 0.1% of all malignant tumors. Although metastasis is very uncommon, local recurrence occur frequently. Dermatofibrosarcoma protuberans occurring in children is even more rare; this is the first case report of congenital dermatofibrosarcoma protuberans in Korea. Methods: The patient is a 14-month-old male infant with a lesion that was first thought to be a birthmark. The lesion grew larger, and a punch biopsy revealed dermatofibrosarcoma protuberans. A wide local excision was performed with a 2 cm peripheral resection margin beyond the gross tumor lesion. Deep fascia and a portion of muscle underneath the central part of the lesion were also taken. The surgical defect was covered by a split-thickness skin graft. Results: There has been no clinical sign of recurrence over one year after the surgery. Conclusion: A patient with congenital dermatofibrosarcoma protuberans detected at an early stage underwent a wide local excision of the tumor after accurate diagnosis was carried out by biopsy and immunohistochemical studies. There was no clinical evidence of tumor recurrence during over a 1-year follow-up.

• Archives of Plastic Surgery
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• 제38권5호
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• pp.609-615
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• 2011

Purpose: Dermatofibrosarcoma protuberans is a relatively rare tumor that originates from the dermis and subcutaneous tissue. It is generally known that this tumor easily recurs but can be successfully treated with a wide excision. Therefore, this study was conducted to investigate postoperative outcomes and risk factors for recurrence in patients with dermatofibrosarcoma protuberans who were treated at a single institution for 20 years. Methods: We retrospectively reviewed the medical records of 35 patients who had underwent surgery between June 1992, and September 2010. The patients were assessed in terms of predilection site and size of the tumor, the incidence according to sex, discrepancy between biopsy results and histopathological diagnosis of the surgical specimen, additional treatment after recurrence, recurrence rate and the time interval to recurrence. Results: In multivariate analysis, the depth and site of the tumor were significant risk factors for tumor recurrence. The recurrence rate was significantly higher in tumors occurring in the upper extremity than those occurring in other regions ($p$=0.0348). In addition, the recurrence rate was significantly higher in tumors with involvement of the fascia and the deeper structures ($p$=0.0324, odds ratio=6, relative risk=1.588). Since dermatofibrosarcoma protuberans has strong invasiveness, its tissue involvement is difficult to evaluate accurately. Conclusion: The results of this study shows that involvement of the fascia and the deeper structures and occurrence in the upper extremity were associated with tumor recurrence. Therefore, clinicians should be aware of these risk factors to achieve better treatment outcomes.

• Journal of Korean Neurosurgical Society
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• 제39권2호
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• pp.148-151
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• 2006

Dermatofibrosarcoma protuberans[DFSP] is an uncommon fibrohistiocytic tumor characterized by aggressive local invasion and consequent local recurrence rather than distant metastasis. We present a case of a 51-year-old male patient with a typical cutaneous DFSP, which showed local recurrences and distant metastasis to the central nervous system[CNS] and the lung after 15 years from the initial diagnosis. CNS metastatic lesions recurred in 5 months after surgical resection of the first CNS lesion. These metastatic tumors were treated by open surgery and gamma-knife radiosurgery. Despite continuous medication of oral chemotherapeutic agent, the patient died during 1-year follow-up period.

• Journal of Korean Neurosurgical Society
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• 제37권3호
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• pp.241-243
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• 2005

We report a case of recurrent dermatofibrosarcoma in a 30-years-old woman who had undergone operations three times during 60 months and had received post-operative radiotherapy. On neurological examination, no neurological deficits were noticed. In brain magnetic resonance image(MRI), there was right parieto-occipital scalp mass with high signal in T2-weighted image, low signal in T1-weighted image with homogeneous enhancement. The removal was done including about 2cm uninvolved margins and pathologic examination of the lesion revealed dermatofibrosarcoma protuberans(DFSP). The prognostic factors of local recurrence may be related to surgical margins for resection; the length from the grossly intact margins, and the microscopically controlled excision in margins.

• Investigative Magnetic Resonance Imaging
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• 제10권2호
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• pp.121-125
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• 2006

두피의 융기성 피부섬유육종이 국소 재발한 48세 남자의 예를 보고하고자 한다. MR영상 소견상 두피의 피부와 피하층에 T1 강조영상에서 저신호강도를 T2 강조영상에서 중등도 고신호강도를 지닌 종괴가 있었고, 조영증강 영상에서 강하게 조영증강되는 종괴는 피부와 피하 및 모상층(galea)으로 파급되는 소견을 보였다. 두피의 융기성 피부섬유 육종은 매우 드문 병변이나 침습적인 종양으로서, MR 영상에서 주변 조직으로의 병변 파급을 진단하고 초기에 광범위 국소절제술을 시행하는 것이 재발을 방지하는 데 중요하다.

• 대한두경부종양학회지
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• 제24권2호
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• pp.203-206
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• 2008

Objectives：DFSP(Dermatofibrosarcoma protuberans) is an uncommon, slowly growing, locally invasive malignant tumor that usually presents as a painless, often long-standing mass arising in the dermis of skin. It occurs most frequently on the trunk and proximal parts of the limbs, less commonly in the head and neck region and has a frequent tendency to recur after surgical excision. Clinically, the initial appearance of the tumor similar to that of benign tumor such as keloid and dermatofibroma. Therefore, accurate clinical diagnosis and adequate surgical excision are important. Materials and Methods：We experienced 6 patients of DFSP in head and neck during the recent 6 years, 5 male and 1 female patients. The age of the patients ranged from 31 to 66. As reconstructive methods, the authors used cervicofacial flap, trapezius musculocutaneous flap, TRAM flap, anterolateral thigh free flap and skin graft. Results：The patients were followed up after operation from 24 to 79 months and all remained free of disease except one case, who occurred at forehead area. Conclusion：We present the experience of 6 cases of DFSP occurred in head and neck. We obtained satisfactory results with appropriate diagnosis and treatment which wide excision with surgical margins 3-5cm. We also present an operative plan of this locally aggressive and highly recurrent tumor.

• Tuberculosis and Respiratory Diseases
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• 제61권6호
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• pp.573-577
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• 2006

저자들은 융기성 피부섬유육종을 완전 절제한 환자에서 5년 후에 원발부위에 재발없이 폐에 단독으로 전이된 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Archives of Plastic Surgery
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• 제36권4호
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• pp.417-421
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• 2009

Purpose: Dermatofibrosarcoma protuberans(DFSP) is a moderate - degree malignant tumor of soft tissue from dermis to fat layer with high recurrences(11% to 73%) due to its local infiltrative characteristic. Many debates and controversies in deciding accurate surgical margin were presented before, but references about depth of invasion and appropriate surgical excision level were not properly made out. Therefore, we tried to identify the degree of tissue invasion of DFSP. Methods: Twenty patients, including 8 patients with recurrent lesions, over last 10 years were reviewed retrospectively. Different surgical margins were applied according to the location and based on histopathologic result, we have defined as a 'deep tissue invasion' if there were infiltration of tumor cell into fascia or underlying muscle layer was present. All invaded tissue including dermis, fat, fascia and muscle were excised until no tumor cell was found during intraoperative frozen section biopsy. And comparative analysis of deep tissue invasion according to age, primary site, duration of disease and recurrence was done. Results: Thirteen patients(65%) showed deep tissue invasion and incidence was found to be increasing with age(over 30 years old). All patients with DFSP on head and neck region revealed deep tissue invasion followed by trunk(54%) and lower extremities(50%). There was no relationship between duration of disease and deep tissue invasion. Conclusions: It is clear that many cases of DFSP had a deep tissue invasion. And high prevalence of deep tissue invasion with age, primary site was intimately associated. So, underlying deep tissue must be completely examined and excised sufficiently throughout the operation for clear resection of DFSP with no recurrences, especially when age is over 30s and on head and neck region.

• 대한두경부종양학회지
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• 제40권1호
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• pp.49-53
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• 2024

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma, with an incidence of about 0.8% to 5% per million people per year, accounting for 1% of soft tissue sarcomas. In its early stage, DFSP is typically found as a violet or pinkish macule or patch, and it can develop into a palpable mass with ulceration or bleeding. The standard treatment for DFSP is wide local excision of the tumor with a 2- to 3-cm negative margin, and radiation therapy or chemotherapy can be conducted with surgical treatment. A 35-year-old man had a palpable mass on the left side of his occipital scalp without color change, ulceration, or bleeding, which typically are present in malignancy. A magnetic resonance imaging (MRI) scan showed a 3-cm homogenous enhanced mass without adhesion between the scalp and the mass. Unexpectedly, a biopsy revealed the round mass to be DFSP. A wide excision and rotation of the scalp flap were performed. The patient recovered without any complications and received adjuvant radiotherapy at a dose of 60 Gray (Gy) for six weeks. There was no recurrence through six months of follow-up. Here we report this unique case of DFSP with atypical presentation.

• 대한골관절종양학회지
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• 제6권1호
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• pp.17-21
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• 2000

목적 : 융기성 피부섬유육종은 피부에 생기는 드문 종양으로서 부적절한 절제가 잦은 재발과 예후에 영향을 미치는 것으로 보고되고 있다. 저자들이 경험한 환자를 대상으로 치료와 예후의 관계를 연구하여 적절한 치료법을 제시하고자 하였다. 대상 및 방법 : 1990년 3월부터 본원 정형외과에서 치료한 융기성 피부섬유육종 환자중 추시 기간이 최소 12개월 이상인 14례를 대상으로 치료 방법 및 최종 추시까지의 상태를 점검하였다. 전례 모두 병리학적 진단하에 치료되었으며, 재발 후 전원된 9례를 포함한 13례에서 광범위 절제술이 시행되었다. 1례는 서혜부의 신경, 혈관이 종양과 인접하여 종양내 전절제술을 시행하였고 술후 항암요법을 추가한 경우였다. 결과 : 재발후 전원되었던 9례의 평균 재발 회수는 1.3 (1~2)회였으며, 처음 수술 후 첫번째 재발까지의 기간은 평균 11.8(2~24)개월이었다. 술후 최종 추시까지(평균 추시기간 50.8개월)에서 광범위절제술로 치료한 13례에서는 재발을 볼 수 없었으나, 병소내 전절제술과 항암제치료를 하였던 예에서는 반복된 재발을 보였고, 결국 폐전이로 사망하였다. 결론 : 융기성 피부섬유육종은 반드시 생검을 통한 확진 후 초기에 광범위 절제연 이상으로 절제를 시행하여야 할 것으로 사료된다. 재발은 최소한 2년까지의 추시가 있어야 그 여부를 알 수 있을 것으로 사료된다.