Tumor in the plantar region: dermatofibrosarcoma protuberans in an infrequent topography

Abstract Dermatofibrosarcoma protuberans is a rare mesenchymal tumor; it is locally aggressive and presents high rates of local recurrence. It may present as a nodular or plaque vegetating lesion. It mainly affects the trunk and proximal limbs, being rare in the distal extremities. Biopsy and immunohistochemistry help confirm the diagnosis. The authors report a case of dermatofibrosarcoma protuberans with plantar region involvement, a rare presentation. To the best of the authors' knowledge, only 11 cases of involvement of the feet were described in the international literature.

Dermatofibrosarcoma protuberans pigmentado: descripción de un caso pediátrico / Pigmented Dermatofibrosarcoma Protuberans: description of a pediatric case

Resumen: Introducción: El tumor de Bednar es un sarcoma de bajo grado, infrecuente, considerado como la variante pigmen tada del dermatofibrosarcoma protuberans (DFSP). Objetivo: describir las características clínicas, histopatológicas, el tratamiento y la evolución de un caso pediátrico de esta infrecuente neoplasia. Caso Clínico: escolar de 9 años que consultó por una pápula indurada asintomática, de dos años de evolución en el dorso del cuarto dedo del pie izquierdo. La biopsia incisional de la lesión fue com patibles con un DFSP pigmentado. El estudio inmunohistoquímico mostró positividad intensa para CD34 en toda la lesión, con factor XIIIa negativo. Se complementó el estudio de la pieza histológica con citogenética molecular FISH para el gen PDGFB (22q13.1) el cual reflejó un patrón anómalo en las células tumorales, no así en los melanocitos ni en la piel peritumoral. Se realizó cirugía micrográfica de Mohs diferida con cobertura mediante sustituto dérmico, sin recidiva ni recurrencia tumoral a los 5 años de seguimiento. Conclusiones: El DFSP pigmentado es un sarcoma de bajo grado, que muy infrecuentemente se presenta en pacientes pediátricos. Las variantes clásica y pigmentada deben ser sospechadas ante una lesión papulonodular única, de crecimiento lento y progresivo, con presencia de células fusiformes con patrón estoriforme en la biopsia y con estudio inmunohistoquímico positi vo para CD34. Es una entidad con buen pronóstico, con escaso riesgo de recurrencia y metástasis, si se logra la realización de una extirpación completa.

Abstract: Introduction: Bednar tumor is a rare low-grade sarcoma considered the pigmented variant of dermatofibrosarco ma protuberans (DFSP). Objective: To describe the clinical and histopathological characteristics, treatment and evolution of this rare neoplasm. Clinical Case: A 9-year old female presented with a 2-year history of an indurated, asymptomatic papule on the back of her fourth left toe. The incisio nal biopsy was compatible with pigmented DFSP. The immunohistochemical study showed intense positivity for CD34 throughout the lesion, with negative factor XIIIa. We complemented the study with molecular cytogenetics (FISH) for PDGFB gene (22q13.1) which showed an abnormal pattern in tumor cells, but not in the melanocytes or the peritumoral skin. Delayed Mohs surgery and skin substitute dressing were performed without neoplastic recurrence at 5 years of follow up. Conclu sion: Pigmented DFSP is a low-grade sarcoma that is very rare in pediatric patients. The classical and pigmented variants should be suspected in the presence of a single papulonodular lesion of slow and progressive growth, with presence of spindle cells with storiform pattern in the biopsy and positive immunohistochemical study for CD34. It is an entity with good prognosis, with little risk of recurren ce and metastasis, if complete excision is achieved.

Estudio Observacional: Dermatofibrosarcoma Protuberans, Serie de casos / Observational Study: Dermatofibrosarcoma Protuberans, Case Series

Introducción: El dermatofibrosarcoma protuberans(DFSP), es uno de los tumores cutáneos menos frecuentes, se clasifica dentro de los sarcomas fibrohistociticos y está caracterizado por un crecimiento lento y progresivo. Métodos: Se realizó una serie de casos retrospectiva de los pacientes con DFSP atendidos en el Centro de la Piel (CEPI) entre 2010 y 2019, se tomó en cuenta la información demográfica, características clínicas, tratamiento y su evolución. Resultados: Seis pacientes fueron diagnosticados y tratados por DFSP en el período evaluado, existió un predominio de mujeres (4 mujeres, 2 hombres) con una media de edad de 58.2 (DE =19,6), tres de ellos presentaron lesiones en forma de placa indurada y los restantes presentaron nódulo único, la localización de las lesiones fueron en hombro, cuello e hipogastrio. El tratamiento más utilizado fue la escisión amplia, ninguno de los pacientes presentó recidivas al primer y quinto año del seguimiento. Conclusión: Es necesario el reconocimiento de las características clínicas típicas de DFSP ya que podría pasar como una tumoración benigna de piel, por lo cual se debe apoyar con el estudio histopatológico frente a la sospecha de este tipo de lesión

Introduction: Dermatofibrosarcoma protuberans (DFSP) is one of the rare tumors found in the fibrohistocytic sarcomas and it is characterized by slow and progressive growth. Methods:A retrospective study of six cases treated at the Skin Center (CEPI) between 2010 and 2019 was carried out. We have take into account demographic information, characteristics, treatment and evolution of the lesions. Results:Six patients were diagnosed and treated by DFSP. There was a predominance of women (4 women, 2 men) with a mean age of 58.2 (SD = 19.6), three of them presented lesions in the form of indurated plaque and the rest presented a unique nodule. The locations of the lesions were the shoulder, neck and hypogastrium. The most commonly used treatment was wide excision, none of the patients presented recurrences at the first and fifth year of follow-up. Conclusion:Recognition of the typical clinical characteristics of DFSP is necessary since it could be mistaken as a benign tumor of the skin. The diagnosis must be always done by histopathological confirmation

Dermatofibrosarcoma protuberans: lo diagnosticamos en la edad pediátrica / Dermatofibrosarcoma protuberans: we diagnose it in the pediatric age

El dermatofibrosarcoma protuberans (DFSP) es un tumor cutáneo, de baja frecuencia, fibrohistiocítico, infiltrante, de lento crecimiento, de agresividad local, de malignidad intermedia; con escasas probabilidades de metástasis pero con alto índice de recurrencia local. El diagnóstico debe sospecharse y confirmarse con histología e inmunohistoquímica. El tratamiento de elección es con cirugía convencional y/o cirugía micrográfica de Mohs, con márgenes de 2-4 cm. Se considera que la prevalencia del DFSP en la edad pediátrica es baja, debido al escaso índice de sospecha. En el presente trabajo compartimos cinco casos de DFSP en la edad pediátrica, estudiados en el Hospital General de Niños Pedro de Elizalde. (AU)

Dermatofibrosarcoma protuberans (DFSP) is a cutaneous, low frequency, fibrohistiocytic, infiltrating, slow growing, local aggressiveness, intermediate malignancy tumor; with little chance of metastasis but with a high rate of local recurrence. The diagnosis should be suspected and confirmed with histology and immunohistochemistry. The treatment of choice is with conventional surgery and / or Mohs micrographic surgery, with margins of 2-4 cm. The prevalence of DFSP in pediatric age is considered to be low, due to the low index of suspicion. In this paper we share five cases of DFSP in the pediatric age, studied at the Pedro de Elizalde Children's General Hospital. (AU)

Dermatofibrosarcoma protuberans / Dermatofibrosarcoma protuberans

Resumen El dermatofibrosarcoma protuberans (DFSP) es un sarcoma cutáneo de baja incidencia y lento crecimiento que afecta principalmente a personas de entre 20 a 39 años y es más frecuente en afrodescendientes. La localización anatómica más común es el tronco, seguido de las extremidades superiores. La etiología no está clara, pero la mayoría de los casos presentan rearreglos genéticos que involucran los genes COL1A1 y PDGFB. El riesgo de metástasis es bajo, pero tiene tendencia a la recurrencia local, especialmente en: los tumores resecados con márgenes insuficientes, los tumores profundos y los tumores que presentan cambio fibrosarcomatoso. La primera línea de manejo para la enfermedad local es la cirugía con la resección local amplia o la cirugía micrográfica de Mohs (CMM), que ha mostrado recurrencias consistentemente bajas. La radioterapia se ha usado para tumores irresecables o como adyuvancia. En los casos de enfermedad metastásica el manejo de elección es el imatinib.

Abstract Dermatofibrosarcoma protuberans (DFSP) is a low incidence and slow growing cutaneous sarcoma that mainly affects individuals between 20 and 39 years old and is more frequent in black race. The most common anatomical site is the trunk, followed by the upper extremities. The etiology is unclear, but the most cases present genetic rearrangements involving the COL1A1 and PDGFB genes. The risk of metastasis is low, but it tends to local recurrence, especially when section margins were insufficient, in deep tumors or those with fibrosarcomatous change. The first line of treatment for local disease is surgery; wide local resection or Mohs micrographic surgery (MMS). The latter technique has shown consistently low recurrence rate. Radiotherapy has been used for unresectable cases or as adjuvant therapy. Imatinib is useful for metastatic disease.

Dermatofibrosarcoma Protuberans. Presentación de un caso / Dermatofibrosarcoma protuberant. Presentation of a case

RESUMEN El dermatofibrosarcoma protuberante es un raro tumor fibrohistiocitario de la piel y grado intermedio de malignidad. Localmente tiene un comportamiento agresivo, se extiende al tejido celular subcutáneo y músculo subyacente. Su crecimiento es lento e indoloro con una alta frecuencia de recurrencia posterior a la intervención quirúrgica. Se presentó una paciente operada en el Servicio de Cirugía General del "Hospital Universitario Dr. Mario Muñoz Monroy" de Colón, Matanzas. Diagnosticada con dermatofibrosarcoma protuberans se presentó con un tumor de partes blandas, localizado en parrilla costal izquierdo, recidivante sobre una lesión resecada anteriormente hacia 8 meses. El tumor de superficie multinodular e irregular, de aproximadamente 3 a 4 cm de diámetro, presentaba zonas de color azuladas y otras más oscuras con un área enrojecida circundante, consistencia dura, y no adherida a planos profundos. Con el resultado anatomopatológico se ratificó el diagnóstico. Nuevamente se aplicó tratamiento quirúrgico con amplios márgenes libres de tumor. Las metástasis son raras y pocos frecuentes, pero localmente pueden infiltrar, grasa, fascia, músculos y hasta estructuras óseas. Cuando se presentan ocurren generalmente en pulmón y ganglios linfáticos regionales, en estos casos la supervivencia es muy poca.

ABSTRACT Dermatofibrosarcoma protuberant (DFSP) is a rare fibrohistiocytic skin tumor of intermediate level of malignance. Locally it has an aggressive behavior extending to the subcutaneous cellular tissue and underlying muscle. Its grow is slow and painless with a high recurrence frequency after surgery. The authors present the case of a female patient operated in the Service of General Surgery of the University Hospital ¨Dr. Mario Muñoz Monroy¨ of Colon, Matanzas. Diagnosed with dermatofibrosarcoma protuberant, she arrived to the consultation with a tumor of soft tissues located in left rib cage, recidivist on a lesion resected 8 months before. It had a multinodular and irregular surface, of around 3-4 cm diameter, with bluish and dark blue areas and a reddened surrounding area of hard consistence, not adhered to deep planes. The diagnosis was ratified by the neuropathologic result. The surgical treatment was applied again with wide free-tumors margins. Metastases are rare and few frequent, but locally they can infiltrate fat, fascia, muscles and even bone structures. When they present they occur mainly in lung and regional lymphatic ganglia; in these cases survival is scarce.

Dermatofibrosarcoma protuberans vulvar: tres casos clínicos de localización inusual / Dermatofibrosarcoma protuberans vulvar: three clinical cases of localization unusual

RESUMEN El dermatofibrosarcoma protuberans (DFSP) es un sarcoma fusocelular de grado intermedio de malignidad con máxima incidencia en adultos entre 20 y 40 años y de localización habitualmente troncular (cabeza, cuello extremidades superiores). Se presentan tres casos de DFSP de localización excepcional a nivel vulvar. Las pacientes fueron tratadas con vulvectomía respetando márgenes de seguridad pero, debido a la idiosincrasia del tumor, presentaron recidivas locales que precisaron de una nueva cirugía. En su posterior seguimiento no presentaron recaídas y se encuentran libres de enfermedad.

ABSTRACT Dermatofibrosarcoma protuberans (DFSP) is an intermediate grade spindle-cell sarcoma with a highest incidence in adults between 20 and 40 years old and a trunk location (head, neck and arms). We introduce three case reports of vulvar DFSP considered unusual because of their location. The patients were conducted a free-margin vulvectomy but, due to the nature of the tumor, local reappearances required a second surgery. In the subsequent follow-up they did not suffer from any tumor relapse and they are currently disease-free.

Fibrosarcomatous dermatofibrosarcoma protuberans from scar following trauma

Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue neoplasm of low metastatic potential notable for its progressive growth and high rate of local recurrence after surgical excision. Fibrosarcomatous transformation of DFSP (FS-DFSP) is a rare variant characterized by higher rates of local recurrence and metastasis. Trauma has been hypothesized as a potential risk factor for the development of DFSP, although clear evidence has been lacking. In this study, we report a case of FS-DFSP that was found arising from a previously stable scar following a traumatic injury. A 49-year-old male was diagnosed with keloid scars following a motor vehicle accident where he sustained trauma. 12 years later, a large tumor developed immediately after a second traumatic event to the primary scar. Pathology of the excisional biopsy specimen demonstrated FS-DFSP with focal areas consistent with keloid and hypertrophic scar. This observation demonstrates the development of DFSP from underlying scar following a clear history of trauma. Furthermore, it suggests trauma as a possible trigger for the fibrosarcomatous transformation of DFSP.

Margens cirúrgicas no dermatofibrossarcoma protuberans: relato de caso e revisão da literatura / Surgical margins of dermatofibrosarcoma protuberans: case report and literature review

Introdução: O dermatofibrossarcoma protuberante (DFSP) é um tumor fibrohistiocítico de origem dérmica raro, que acomete a pele, representando 1% dos sarcomas de partes moles e menos de 0,1% de todas malignidades. A principal característica deste tipo de tumor é a sua elevada taxa de recidiva local após excisão cirúrgica. Decidir a margem cirúrgica adequada para a ressecção completa é um desafio. Relato de caso: 24 anos, apresenta lesão tumoral vegetante avermelhada no ombro esquerdo, assintomática. Possui história familiar positiva de DFSP em membro inferior. O laudo histopatológico definitivo confirmou Dermatofibrossarcoma protuberans. Discussão: A característica histológica do tumor é a presença de projeções tipo tentáculos de células neoplásicas na periferia que se estendem através do tecido subcutâneo até a fáscia muscular. Certamente, o fator de maior importância para o controle local é a obtenção de margens cirúrgicas livres. A excisão através da cirurgia micrográfica de Mohs é uma ótima opção em regiões onde a excisão ampla não é desejável, como na face. A margem padrão estabelicida em grande parte da literatura é de 3 cm com ressecção até a fáscia muscular, podendo ser diminuida para 2 cm em locais em que a excisão ampla prejudique a conformação do local, como na face. (AU)

Dermatofibrosarcoma protuberans (DFSP) is a rare dermal fibrohistiocytic tumor that affects the skin, accounting for 1% of soft tissue sarcomas and representing less than0.1% of all malignancies. The main characteristic of this type of tumor is a high rate of local recurrence after surgical excision. Deciding the proper surgical margin for complete resection is a challenge. Case report: 24 year-old, shows a reddish vegetative asymptomatic tumor on the left shoulder. She has a positive family history of DFSP in the lower limb. The definitive histopathological report confirmed Dermatofibrossarcoma protuberans. Discussion: The histological feature of the tumor is the presence of tentacle type projections of neoplastic cells in the periphery extending through the subcutaneous tissue to the muscular fascia. Of course, the most important factor for local control is the achievement of free surgical margins. Excision through Mohs micrographic surgery is a great option in regions where wide excision is not desirable, as in the face. The standard margin in most pf the literature is 3 cm resected to the muscular fascia, and it can be reduced to 2 cm in places where the extensive excision impairs the conformation of the site, as in the face. (AU)

An unusual variant of atrophic dermatofibrosarcoma protuberans

Abstract: Dermatofibrosarcoma protuberans is an uncommon neoplasm that is most often seen in young adults. The most common clinical presentation is the protruding form; however, other subtypes are known, such as the atrophic. In 2012 there were only 33 reports of this variant in the literature. Many cases of Dermatofibrosarcoma protuberans in children are only discovered in adulthood because they were not diagnosed early. Due the high morbidity, we raise the need for attention from the dermatologist to recognize uncommon neoplasms in the clinical practice. We report a case of a 15-year-old patient diagnosed with atrophic Dermatofibrosarcoma protuberans on the back.

Expansão prévia no tratamento de dermatofibrossarcoma protuberante envolvendo o osso frontal / Preceding expansion in the treatment of dermatofibrosarcoma protuberans involving the frontal bone

Introdução: Dermatofibrossarcoma protuberante é um tumor cutâneo raro de malignidade intermediária e potencial metastático baixo, apresentando, entretanto, alta taxa de recorrência após tratamento cirúrgico. O tratamento cirúrgico clássico é a ressecção alargada com margens variadas. Métodos: Os pacientes foram submetidos à expansão prévia dos tecidos da região frontal utilizando dois expansores convencionais. Margens laterais de 3 cm de tecido de aparência normal foram tatuadas com tinta nanquim. Os expansores foram incluídos em uma cirurgia anterior, através de incisões cutâneas distantes dessas marcas, em um plano cirúrgico logo acima do periósteo, preservando a área de ressecção alargada previamente delimitada. Os tecidos laterais foram expandidos e, posteriormente, os pacientes foram submetidos à ressecção do tumor. A margem profunda incluiu da lâmina externa do osso frontal. O período médio de expansão foi de 45 dias. Resultados: Foram relatados três casos de dermatofibrossarcoma protuberante da região frontal com envolvimento da lâmina externa do osso frontal. Os retalhos expandidos fecharam facilmente os defeitos. O acompanhamento pós-operatório médio foi de 194 meses, sem nenhuma recorrência tumoral. Conclusão: A expansão tecidual prévia da região frontal no tratamento do dermatofibrossarcoma protuberante, invadindo o osso frontal, usando margens laterais de 3 cm com remoção da lâmina externa, permitiu facilmente o fechamento do defeito, em três pacientes. O tempo de acompanhamento médio de 194 meses sem nenhuma recorrência tumoral mostrou a eficiência do método.

Introduction: Dermatofibrosarcoma protuberans is a rare skin tumor with intermediate malignancy and low metastatic potential, but a high recurrence rate after surgical treatment. Treatment involves extended resection with variable margins. Methods: Patients were managed with preceding expansion of tissues of the forehead using two conventional expanders. The lateral margins of normal-appearing tissue 3-cm apart were tattooed with China ink. In a preceding operation, expanders were inserted through skin incisions at a distance from the marks, by undermining the surgical plane just above the periosteum, while preserving the area of wide resection previously delimited. After the lateral tissues were expanded, the patients underwent surgical resection. The deep margin included the outer table of the frontal bone. The expansion period averaged 45 days. Results: Three cases of dermatofibrosarcoma protuberans of the forehead with involvement of the outer table of the frontal bone are described. The expanded flaps easily closed the defects. The average follow-up was 194 months without tumor recurrence. Conclusion: Preceding expansion of forehead tissue in the treatment of dermatofibrosarcoma protuberans with invasion of frontal bone, using 3-cm lateral margins and removal of the outer table of the frontal bone, allowed easy closure of the defect in 3 different patients. Average follow-up of 194 months with no recurrence of the lesion showed the effectiveness of the method.

Dermatofibrosarcoma protuberante en una paciente adulta / Protuberant dermatofibrosarcoma in a mature patient

Se presenta el caso clínico de una paciente de 56 años de edad, quien acudió al Hospital Provincial Docente Clinicoquirúrgico Saturnino Lora Torres por presentar una gran tumoración localizada en la espalda baja, poco dolorosa y con necrosis tumoral en la región apical. Se le realizó exéresis total del tumor con márgenes de sección amplios. La biopsia del espécimen confirmó la presencia de un dermatofibrosarcoma protuberante. Luego de varios días de hospitalización bajo estricta vigilancia y tratamiento médico se le dio el alta; 6 meses después de la cirugía su evolución ha sido satisfactoria

The case report of a 56 years patient is presented who went to Saturnino Lora Torres Teaching Clinical Surgical Provincial Hospital due to a large tumor located in the low back, not very painful and with tumoral necrosis in the apical region. Total exeresis of the tumor was carried out with wide section margins. The biopsy specimen confirmed the presence of a protuberant dermatofibrosarcoma. After several days of hospitalization under strict surveillance and medical treatment she was discharged; 6 months after the surgery her clinical course has been satisfactory

Dermatofibrosarcoma protuberans: A propósito de un caso / Dermatofibrosarcoma protuberans: About a case

El dermatofibrosarcoma protuberans (DFSP) es un tumor cutáneo raro, localmente agresivo, con tendencia a la recurrencia local pero que rara vez metastatiza. Constituye el sarcoma de origen cutáneo más común. Representa un desafío diagnóstico y terapéutico para los dermatólogos, debido a la alta tasa de recurrencia local que posee. En el siguiente artículo, presentamos el caso de un paciente con este diagnóstico y su posterior manejo (AU)

Dermatofibrosarcoma protuberans (DFSP) is an unusual locally aggressive cutaneous tumor, with tendency to local recurrence but rarely to metastasis. It is the most common cutaneous sarcoma, presenting a diagnostic and therapeutic challenge for dermatologists, due to the high rate of local recurrence. A male patient diagnosed with DFSP and its subsequent management is reported (AU)

Dermatofibrossarcoma protuberante diagnosticado erroneamente como queloide e tratado com acetonido de triancinolona / Dermatofibrosarcoma protuberans misdiagnosed as keloid and treated with triamcinolone acetonide

INTRODUÇÃO: Dermatofibrossarcoma protuberante é um tumor de pele raro e de malignidade intermediária, com baixo potencial metastático, mas altas taxas de recorrência após tratamento cirúrgico. Por apresentar eventual semelhança clínica com cicatrizes hipertróficas e queloides, o diagnóstico correto mostra-se fundamental para o sucesso do tratamento. O objetivo do presente trabalho é fazer um alerta e relatar quatro casos de dermatofibrossarcoma protuberante erroneamente diagnosticados como queloide e tratados alhures com infiltração de acetonido de triancinolona. MÉTODO: Entre novembro de 1983 e janeiro de 2008, foram atendidos quatro pacientes com dermatofibrossarcoma protuberante que tinham sido submetidos alhures a infiltrações intralesionais de acetonido de triancinolona, em virtude de diagnóstico errôneo de queloide. Nos quatro casos, foram realizadas excisões cirúrgicas radicais, com remoção de 3 cm de tecido sadio nas margens laterais, incluindo-se, na margem profunda, uma estrutura anatômica não infiltrada pelo tumor. Os pacientes receberam avaliação médica periódica em longo prazo. RESULTADOS: Os pacientes foram acompanhados por uma média de 159 meses. Três pacientes (75%) permaneceram vivos, sem sinais de doença em atividade. Um paciente (25%) faleceu devido à doença, após tentativa de remover o avançado tumor recorrente, por meio de extensa cirurgia craniofacial. A recidiva ocorreu sete anos após a operação radical. CONCLUSÃO: Dermatofibrossarcoma protuberante deve ser considerado no diagnóstico diferencial dos queloides. A infiltração intralesional de acetonido de triancinolona só deverá ser realizada após diagnóstico de certeza, que pode demandar exame anatomopatológico prévio. Um exame clínico cuidadoso e o conhecimento da lesão favorecem um diagnóstico preciso e, portanto, um tratamento adequado.

INTRODUCTION: Dermatofibrosarcoma protuberans is a rare skin tumor with intermediate malignancy, low metastatic potential, and high recurrence rates after surgical treatment. Owing to a possible clinical resemblance with hypertrophic scars and keloids, the correct diagnosis is fundamental for treatment success. The objective of the present work is to report on four cases of dermatofibrosarcoma protuberans misdiagnosed as keloid and treated elsewhere with infiltration of triamcinolone acetonide. METHOD: Between November 1983 and January 2008, four patients with dermatofibrosarcoma protuberans who had undergone intralesional infiltration with triamcinolone acetonide elsewhere were treated because of an erroneous diagnosis of keloid. Radical surgical excision was performed, and 3 cm of healthy tissue was removed from the side margins, including the deep margin, an anatomical structure not infiltrated by the tumor. The patients underwent long-term periodic medical evaluations. RESULTS: The patients were followed-up for an average of 159 months. Three patients (75%) are still alive without signs of disease at the time of this report. One patient (25%) died of the disease after an attempt to remove the advanced recurrent tumor using extensive craniofacial surgery. Recurrence occurred 7 years after the radical operation. CONCLUSION: Dermatofibrosarcoma protuberans must be considered in the differential diagnosis of keloids. Intralesional infiltration with triamcinolone acetonide should only be performed after diagnostic confirmation , which may require pathological examination. A careful clinical examination and knowledge of the lesion favor a precise diagnosis and an appropriate treatment.

Dermatofibrosarcoma protuberans, análisis clínico e histopatológico de los últimos 14 años en población consultante del Hospital Clínico Universidad de Chile / Dermatofibrosarcoma protuberans, clinical analysis and histopathological of the last 14 years in population consultant of the University Hospital of Chile

Introducción: El Dermatofibrosarcoma Protuberans (DFSP) es un sarcoma cutáneo infrecuente y poco descrito en la literatura regional. En este trabajo se describió y analizó las características clínicas e histopatológicas de los DFSP tratados en nuestro centro. Métodos: Estudio retrospectivo de todos los casos de DFSP primarios, confirmados con biopsia entre los años 2002 y 2016 en el Servicio de Anatomía Patológica del Hospital Clínico Universidad de Chile (HCUCh). Resultados: La muestra contó con 18 casos, 67% de sexo femenino, la edad promedio de diagnóstico fue 45,5 años. El 28% de los DFSP se localizaron en tronco, 22% extremidades inferiores, 22% extremidades superiores, 22% región inguinogenital y 6% cervical. Un 22% de las biopsias fueron enviadas con diagnóstico clínico presuntivo de DFSP. En la histopatología, todos los DFSP estaban compuestos por células fusiformes, distribuidas en patrón estoriforme que infiltraban hasta el tejido adiposo subcutáneo. La inmunohistoquímica se realizó en 7 casos, siendo positivo para CD34 en todas estas muestras y negativo para el factor XIIIa. En el 50% de los casos se observó compromiso de márgenes quirúrgicos. Conclusiones: Nuestros resultados fueron similares a publicaciones internacionales. Se observó mayor frecuencia en mujeres y diagnóstico clínico más tardío. Se constató un bajo índice de sospecha clínica de DFSP, confundiéndose con patologías benignas, lo que podría incidir en la frecuencia de márgenes positivos del estudio. Este trabajo constituye uno de los reportes de series de casos más extensos desarrollado a nivel nacional.

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare skin sarcoma barely described in regional literature. This paper described and analyzed the clinical and histopathologic features of DFSP treated in our center. Methods: Retrospective study of all cases of primary DFSP, confirmed with biopsy between 2002 and 2016 in the Department of Pathology of the University of Chile Clinical Hospital (HCUCh). Results: The sample had 18 cases, 67% female, the average age of diagnosis was 45.5 years. The 28% of DFSP were located on the trunk, 22% lower limbs, 22% upper limbs, 22% inguino-genital region and 6% on the cervical region. A 22% of the biopsies were sent with presumptive clinical diagnosis of DFSP. On histopathology, all DFSP were composed of spindle- shaped cells distributed in storiform pattern, infiltrating to the subcutaneous adipose tissue. Immunohistochemistry was performed in 7 cases, being positive for CD34 in all of these samples and negative for factor XIIIa. In 50% of cases surgical margins were positive. Conclusions: Our results were similar to international publications. Higher frequency was observed in females and a delayed clinical diagnosis. There was a low index of clinical suspicion of DFSP, often confused with benign conditions, which could have influenced the high frequency of positive margins found in the study. This paper is one of the most extensive reports of case series developed nationwide.

Dermatofibrosarcoma protuberans: série de 27 casos consecutivos / Dermatofibrosarcoma protuberans: a single series of 27 consecutive cases

INTRODUÇÃO: A dermatofibrosarcoma protuberans (DFSP) é um tumor maligno de baixo grau de partes moles caracterizado por infiltração local agressiva e propenso a recidiva local. Este estudo retrospectivo analisou resultados clínicos, taxas de recidiva e sobrevida após tratamento cirúrgico de DFSP. MÉTODOS: Pacientes submetidos a cirurgia para erradicação completa do tumor, e subsequente seguimento por exame clínico e vigilância ultrassonográfica de locais primários do tumor e linfonodos correspondentes para detectar recidiva local ou distante. A cirurgia, invariavelmente, incluiu grande excisão do tumor, seguida por procedimentos de reconstrução diferente como enxerto de pele em 23 casos (85%), retalho local em 2 pacientes (7.4%), retalho livre em 1 caso (4%), enquanto a principal sutura foi realizada apenas em um caso (4%). RESULTADOS: Foi necessária segunda cirurgia em 9 casos (33%) para atingir margens livres mínimas de 2-3 cm. Outros tratamentos cirúrgicos foram utilizados, como cirurgia micrográfica de Mohs, ou terapias adjuvantes, como radioterapia e quimioterapia. Em nossa série de pacientes o intervalo livre de recidiva variou de 1 a 10 anos, com média de 6 anos. A recidiva local ocorreu em 3 pacientes (11%), e necessitou de outras excisões cirúrgicas extensas. Um total de 27 pacientes não desenvolveu metástase distante durante o seguimento. CONCLUSÃO: A excisão extensa é efetiva para disponibilizar local confiável para controlar a doença, porém somente se limitadas por margens livres de 2-3 cm e confirmadas por patologista. A vigilância clinica e ultrassonografia durante o seguimento permite identificação precoce de eventuais recidivas locais e envolvimento de linfonodos.

INTRODUCTION: Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade malignant tumor of soft tissues characterized by aggressive local infiltration and propensity for local recurrence. This retrospective study analyzed clinical outcomes, recurrence and survival rates after surgical treatment of DFSP. METHODS: Patients underwent surgery to complete eradicate tumor, and subsequently a close follow-up by clinical examination and ultrasounds surveillance of primary tumor site and corresponding lymph nodes, to detect local or distant recurrence. Surgery invariably included wide excision of tumor, followed by different reconstructive procedures as skin grafting in 23 cases (85%), local flap in 2 patients (7.4%), free flap in 1 case (4%), while primary closure was performed only in one case (4%). RESULTS: Second surgery was needed in 9 cases (33%) to achieve minimum free-margins of 2-3cm. Other surgical treatments like Mohs Micrographic Surgery, or adjuvant therapies, like radio- or chemotherapy were not used. Free-recurrence lapse among this series of patients varied from 1 to 10 years, with a medium period of 6 years. Local recurrence occurred in 3 patients (11%), and required a further extended surgical excision. A total of 27 patients did not develop distance metastasis during the follow-up. CONCLUSION: Extended excision is effective to provide a reliable local control of disease, but only if free margins limiting 2-3 cm is confirmed by pathologist. Clinical and ultrasound surveillance during close follow-up provide early detection of eventual local recurrence and of lymph nodes involvement.

Dermatofibrossarcoma protuberans em placa simulando carcinoma basocelular esclerodermiforme / Dermatofibrosarcoma protuberans plate simulating basal cell carcinoma sclerodermiform

Contexto: Descrição de neoplasia maligna cutânea com expressão clínica rara. Descrição do caso: Paciente do sexo feminino, de 41 anos, apresentou-se com placa endurada, acastanhada, infiltrada, de superfície irregular e crescimento progressivo, localizada na face lateral do braçodireito (terço proximal) há quatro anos. À dermatoscopia, tinha rede pigmentar periférica, vasos arboriformes focados e desfocados, crisálidas,coloração rosa de fundo e áreas despigmentadas sem estruturas. Foram elencadas as hipóteses de carcinoma basocelular, dermatofibroma edermatofibrossarcoma protuberans. Biópsia incisional evidenciou neoplasia fusocelular na derme e infiltração do tecido subcutâneo em ?favode mel?. A imunoistoquímica revelou expressão de CD34 nas células neoplásicas. Com esses elementos, foi realizado diagnóstico de dermatofibrossarcoma protuberans. Discussão: O dermatofibrossarcoma protuberans, tumor fibro-histiocítico de malignidade intermediária, é o sarcoma cutâneo mais frequente. O pico de incidência ocorre entre 30 e 40 anos, sendo o tronco o principal local de acometimento. Surge comoplaca endurada, assintomática, aderida à pele, de cor rósea a violácea, com 1 a 5 cm de diâmetro. Após meses até décadas, passa a apresentarmúltiplos nódulos na superfície. Em casos avançados/recorrentes, pode invadir fáscia, músculo e osso. A dermatoscopia é promissora comoauxiliar no diagnóstico precoce desse tumor. Confirmação diagnóstica é estabelecida por meio da associação dos dados clínicos, dermatoscópicose principalmente pela análise histopatológica/imunoistoquímica do tumor. Tratamento clássico é cirúrgico com margens amplas, porém não há consenso estabelecido a respeito de seu tamanho. Apresenta elevadas taxas de recidiva local e metástases são raras. Conclusões: Relata-se tumor maligno raro, cujo diagnóstico precoce aumenta as chances de sucesso terapêutico.

Retalho Duoescapular: uma nova opção na cirurgia reparadora do ombro / Duoscapular flap: a new option in shoulder reconstructive surgery

INTRODUÇÃO: O Dermatofibrossarcoma do Ombro é patologia incomum e seu tratamento demanda extensas ressecções. O sistema escapular é fonte de retalhos bastante utilizados nesta região. MÉTODO: Realizado estudo longitudinal, prospectivo, através da condução de um caso de Dermatofibrossarcoma Protuberans em ombro direito, submetido a ressecção e reconstrução local com Retalho Duoescapular, obtido através da associação dos retalhos escapular e paraescapular. RESULTADOS: Paciente evoluiu sem intercorrências no pós-operatório, não sendo observadas complicações sistêmicas e locais, e limitações funcionais. CONCLUSÃO: O Retalho Duoescapular é nova e relevante opção para reconstrução de feridas extensas, com exposição de estruturas nobres no ombro. Permite fechamento primário da área doadora, sem acrescentar morbidade ao procedimento.

INTRODUCTION: Shoulder cutaneous fibrosarcoma is an unusual pathology that requires extensive resections. The scapula is a source of flaps widely used in this region. METHOD: A longitudinal and prospective study was carried out in a patient with protuberans cutaneous fibrosarcoma on the right shoulder who underwent resection followed by local reconstruction with a Duoscapular Flap (a combination of scapular and parascapular flaps). RESULTS: The patient had no postoperative complications, as systemic and local complications as well as functional limitations were not observed. CONCLUSION: Duoscapular Flap placement is a novel procedure and a relevant choice for the reconstruction of extensive wounds exposing noble structures in the shoulder. It allows the primary closure of the donor area without increasing the morbidity of the procedure.