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      Dermatofibrosarcoma protuberans: Presentación de un caso y revisión de la literatura Translated title: Dermatofibrosarcome protubérant: Présentation d´un cas et mise en revue de la littérature Translated title: Dermatofibrosarcoma protuberans: Case report and literature review

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          Abstract

          Se presenta un paciente con un dermatofibrosarcoma protuberans en el muslo izquierdo, raro sarcoma de las partes blandas. Se expone el cuadro clínico y la terapéutica efectuada. Se revisa la literatura.

          Translated abstract

          Un patient atteint d´un dermatofibrosarcome protubérant sur la cuisse gauche, un rare sarcome des parties molles, est présenté. Le tableau clinique et la thérapeutique effectuée sont exposés. La litérature est mise en revue.

          Translated abstract

          A patient with a dermatofibrosarcoma protuberans on the left thigh, a rare sarcoma of the soft parts, is presented. The clinical picture and the therapeutics used are exposed. Literature is also reviewed.

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          Most cited references17

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          A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans.

          There is growing evidence that Mohs micrographic surgery (MMS) is the treatment of choice for dermatofibrosarcoma protuberans (DFSP). This study retrospectively compared the recurrence rates of DFSP after MMS with those after wide surgical excision; results at the Mayo Clinic and in the world literature were evaluated. In addition, preoperative tumor sizes and postoperative defect sizes after MMS were compared to determine whether MMS conserved more normal tissue than wide surgical excision. The medical records of 84 patients with DFSP who had been treated at the Mayo Clinic were reviewed. They were categorized into two treatment groups: MMS and surgical excision. Fifteen patients with DFSP who underwent MMS had follow-up data available; one of these patients had local recurrence (recurrence rate, 6.6%; average duration of follow-up, 40 months). Thirty-nine patients had wide excision; four of these patients had local recurrences and one had pulmonary metastases (recurrence rate, 10%; average duration of follow-up, 36 months). A review of the world literature revealed neither local recurrences nor metastases in the 11 studies in which DFSP was treated with MMS. Overall, including our results, the average recurrence rate of DFSP after MMS was 0.6% (range, 0% to 6.6%) and the total recurrence rate was 1.6% (1 of 64). Including our series, DFSP was treated with wide excision in 15 studies; the average recurrence rate was 18% (range, 0% to 60%) and the total recurrence rate was 20% (100 of 489). In eight published studies, DFSP was surgically resected with undefined or conservative excisional margins; the average recurrence rate was 43% (range, 26% to 60%) and the total recurrence rate was 44% (140 of 317). A surgical margin of 2.5 cm to deep fascia was required for complete clearance of all tumors treated with MMS. Twenty-two percent of tumors were removed with a 0.5 cm margin, 50% with a 1.0 cm margin, 67% with a 1.5 cm margin, and 89% with a 2.0 cm margin. On the basis of our results and data compiled from the literature, MMS may be the treatment of choice for DFSP because of its high cure rate and maximal conservation of tissue.
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            Radiation in management of patients with dermatofibrosarcoma protuberans.

            The preferred treatment of dermatofibrosarcoma protuberans (DFSP) is wide resection, namely, margins > or = 3 cm beyond the evident disease and histologically negative margins. We assess the success achieved by radiation combined with surgery for positive/close margins or by radiation alone for those tumors that are not resectable for technical/medical reasons. The literature on this point is virtually nonexistent. The outcome of treatment of 18 patients with DFSP by radiation alone (n = 3) and radiation and surgery (n = 15) at the Massachusetts General Hospital was assessed. All of the lesions at the time of the treatment by radiation alone or combined with surgery were less than 10 cm. This was the maximum dimension. The actual tumor volume was much less than indicated by this maximum dimension, as the tumors were usually relatively flat. The 10-year actuarial local control rate was determined to be 88%. Local control was realized in the three patients treated by radiation alone, with follow-up periods of > or = 9 years. Among 15 patients treated by radiation and surgery, there have been three local failures; the 10-year actuarial local control rate was 84%. The three local failures occurred in 12 patients whose surgical margins were positive. One of these three local failures developed in the group of two patients whose lesions were scored as grade II. Radiation in well-tolerated dose schedules is an effective option in the management of patients with DFSP. This appears to be true for radiation alone or postoperatively for margin-positive disease (primary or recurrent).
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              Gain of 17q24-qter detected by comparative genomic hybridization in malignant tumors from patients with von Recklinghausen's neurofibromatosis.

              The genetic changes leading to the development of malignant peripheral nerve sheath tumors (MPNSTs) are largely unknown. The few tumors that have been investigated cytogenetically had highly complex karyotypes and no consistent rearrangements, and the attempts to pinpoint consistent DNA-level changes have met with only limited success. We used comparative genomic hybridization to analyze seven MPNSTs and one dermatofibrosarcoma protuberans from eight patients with von Recklinghausen's disease (neurofibromatosis type 1), as well as three sporadic MPNSTs. Gains and losses of DNA sequences were found in all tumors, with an average of four losses (range, 0-14) and two gains (range, 0-5) per tumor. Two striking observations were made: (a) an increase in copy number of the distal part of the long arm of chromosome 17, with the smallest region of overlap 17q24-qter, was seen in five of seven MPNSTs and in the only dermatofibrosarcoma protuberans, all of which were from patients with neurofibromatosis, whereas none of the three sporadic MPNSTs had this alteration; and (b) loss of 13q, with the smallest region of overlap 13q14-q21, was found in 6 of 10 MPNSTs. The consistent involvement of these two chromosomal regions probably reflects two different pathogenetic mechanisms for MPNSTs.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                ort
                Revista Cubana de Ortopedia y Traumatología
                Rev Cubana Ortop Traumatol
                Editorial Ciencias Médicas (Ciudad de la Habana )
                1561-3100
                December 1998
                : 12
                : 1-2
                : 84-88
                Affiliations
                [1 ] Complejo Científico Ortopédico Internacional Frank País Cuba
                Article
                S0864-215X1998000100017
                cc7eaeb7-3e4b-4274-85a0-4f8e54c5df8e

                http://creativecommons.org/licenses/by/4.0/

                History
                Product

                SciELO Cuba

                Self URI (journal page): http://scielo.sld.cu/scielo.php?script=sci_serial&pid=0864-215X&lng=en
                Categories
                ORTHOPEDICS

                Orthopedics
                DERMATOFIBROSARCOME,NÉOPLASMES CUTANÉS,DERMATOFIBROSARCOMA,SKIN NEOPLASMS,NEOPLASMAS CUTANEOS

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