• Archives of Plastic Surgery
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• v.37 no.3
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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.

• Journal of Korean Neurosurgical Society
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• v.37 no.3
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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.

• Archives of Plastic Surgery
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• v.38 no.5
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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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• v.39 no.2
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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.

• Investigative Magnetic Resonance Imaging
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• v.10 no.2
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• pp.121-125
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• 2006

A 48-year-old man presented with a dermatofibrosarcoma protuberans (DFSP) of the scalp associated with local recurrence. Axial T1- and T2-weighted images demonstrated a well-circumscribed hypointense and intermediate hyperintense mass in the skin and subcutaneous layer of the scalp, respectively. Contrast-enhanced T1-weighted images showed the strongly enhanced mass invasion to the skin, subcutaneous layer and adjacent galeal layer. Scalp DFSP is very uncommon but is an aggressive tumor, so MR imaging diagnosis of the extent of the lesion to underlying structures, and initial wide local resection is important to prevent recurrence.

• Archives of Plastic Surgery
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• v.36 no.4
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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.

• Korean Journal of Head & Neck Oncology
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• v.24 no.2
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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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• v.61 no.6
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• pp.573-577
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• 2006

Dermatofibrosarcoma protuberans (DFPS) is a locally aggressive skin tumor with a very low incidence in the general population. This tumor has a remarkable tendency to recur, However, a metastasis is rare. We report a case of DFPS with a pulmonary metastasis in 28-year-old man. The pulmonary metastasis developed 5 years after a complete resection of the primary skin tumor. We reviewed the clinical manifestations and treatment of DFPS, and highlight the need for a long-term follow-up examination for metastases after a wide excision of these lesions.

• Korean Journal of Head & Neck Oncology
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• v.40 no.1
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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.

• The Journal of the Korean bone and joint tumor society
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• v.6 no.1
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• pp.17-21
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• 2000

Purpose : Dermatofibrosarcoma protuberans(DFSP) is a rare tumor of the skin with a strong tendency of infiltration to surrounding tissues. Inadequate surgical intervention brings about frequent recurrence and poor prognosis. We attempted to find a guideline for adequate treatment for DFSP. Materials and Methods : Fourteen cases who had been treated in our department since Mar. 1993 and followed up for more than 12 months postoperatively were reviewed. Including nine cases who were transferred from other hospital after recurrence, thirteen cases underwent wide resection. One case was treated by intralesional resection followed by chemotherapy (CYVADIC) due to neurovascular abutment to the mass in the inguinal area. Results : The nine cases who were transferred due to recurrences experienced recurrence in average 1.3(1-2) times and the average period until first local recurrence from primary operation was 11.8(2-24) months. The thirteen cases with wide surgical margin showed no recurrence at the final follow up. One case treated by intralesional resection and chemotherapy showed multiple recurrence and died of the disease due to lung metastasis. Conclusions : From these data, we could find that primary wide resection can be the way of reducing recurrence and metastasis, and the follow up period for the detection of recurrence should be at least two years.