Dermatofibrossarcoma na glabela: Uma localização incomum / Dermatofibrosarcoma in glabella: An uncommon location

O dermatofibrossarcoma é um câncer raro que apresenta padrão de crescimento lento e invasão tecidual agressiva. O tratamento para esta condição envolve intervenção cirúrgica com o objetivo de obter margens livres. Neste caso particular, temos um homem de 60 anos que apresentava uma massa na região da glabela que foi extirpada duas vezes sob anestesia local em outro serviço. Os resultados de ambas as biópsias indicaram dermatofibroma. No entanto, o paciente apresentou nova recidiva, que durante a análise imuno-histoquímica da excisão inicial revelou tratar-se de dermatofibrossarcoma. Como resultado, uma ampla excisão foi realizada até que margens negativas fossem obtidas ao exame de congelação. Além disso, um retalho frontal foi empregado no procedimento para reconstrução do defeito cirúrgico. O resultado do tratamento foi considerado bem-sucedido, sem complicações.

Dermatofibrosarcoma is a rare cancer with a slow growth pattern and aggressive tissue invasion. The treatment for this condition involves surgical intervention to achieve clear margins. In this particular case, we have a 60-year-old man who had a mass in the glabella area that was excised twice under local anesthesia at a different facility. The results of both biopsies indicated dermatofibroma. However, the patient experienced a new recurrence, and during the immunohistochemistry analysis of the initial excision, it was revealed to be dermatofibrosarcoma. As a result, a broad excision was performed until negative margins were obtained based on frozen sections. Additionally, a frontal flap was employed to reconstruct the surgical defect. The outcome of the treatment was deemed successful, without complications.

Recurrent dermatofibrosarcoma protuberans of the breast: A case report and review of literature

Dermatofibrosarcoma protuberans (DFSP) is a rare and low-grade sarcoma of fibroblast origin with a tendency to invade and recur locally. The most common sites of origin of DFSP are the head, neck, and extremities. However, DFSP breast has also been reported. It infiltrates surrounding subcutaneous tissue and fascia, with an incidence of <2% for lung metastases. Surgery being the main modality of the treatment, literature has shown that radiation therapy also plays an important role to improve local control in case of recurrent tumors. In this article, we will be discussing one such rare case of DFSP in a recurrent setting and the role of radiation therapy.

Mohs micrographic surgery in rare cutaneous tumors: a retrospective study at a Brazilian tertiary university hospital

Abstract Background Mohs micrographic surgery is an established technique in the treatment of cutaneous neoplasms. It offers higher cure rates and the main indications are non-melanoma malignant skin tumors. Few studies have been performed on the treatment of rare tumors through this technique. Objective To study rare skin tumors and rare variants of basal cell carcinoma and squamous cell carcinoma submitted to Mohs micrographic surgery in a tertiary service in relation to frequency, disease-free evolution, and applicability of this surgical procedure for this group of tumors. Methods This was a retrospective observational study including rare skin tumors and less common variants of basal cell carcinoma and squamous cell carcinoma treated using Mohs micrographic surgery, between October 2008 and April 2021. Results During the study period, 437 tumors were treated using Mohs micrographic surgery, and 22 (5%) rare skin tumors were selected. The tumors comprised three dermatofibrosarcomas protuberans, two atypical fibroxanthomas, two spiradenomas, two hypercellular fibrohistiocytomas, one primary cutaneous adenocarcinoma, one trichoblastoma, one porocarcinoma, one chondroid syringoma, one cutaneous angiosarcoma, one Merkel cell carcinoma, and one sebaceous carcinoma. Six other cases of rare basal cell carcinoma variants with trichoepitheliomatous differentiation, metatypical basal cell carcinoma, and clear cell squamous cell carcinoma were included. There were no cases of recurrence after an average of six years of follow-up. Study limitations This is a retrospective study on rare neoplasms carried out in a single referral center, and this surgical technique isn't widely available in the public service. Conclusion This retrospective case series showed that Mohs micrographic surgery is an appropriate treatment for rare skin tumors. They corresponded to 5% of the tumors treated by the technique during a 12-year-period, with no recurrences identified.

Dermatofibrosarcoma protuberans with unusual presentation in vulva / Dermatofibrosarcoma protuberans con presentación inusual en vulva

Abstract Gynecological sarcomas are uncommon and their location in the vulva and vagina has an incidence of 5% of all malignant neoplasms of the female genital tract. We present the case of a 54-year-old patient with a diagnosis of dermatofibrosarcoma protuberans in the vulva, an infrequent pathology, with less than 60 cases reported worldwide in this anatomical location. Clinically it has a locally aggressive behavior, due to the proliferation of spindle cells with pleomorphism and frequent figures of mitosis that infiltrate the reticular dermis and subcutaneous cellular tissue, giving rise to tumor lesions of variable size and with high rates of local recurrence. The treatment of first choice is surgical excision of the tumor with Mohs micrographic surgery or other surgical techniques for complete evaluation of the circumferential and deep peripheral margin. However, the identification of carcinogenesis mechanis ms where the chromosomal translocation t (17; 22) (q22; q13) is recognized, forming the COL1A1-PDGFB fusion gene, which participates in stimulating tumor cell proliferation, allowing treatment with tyrosine kinase inhibitors such as imatinib for neoadjuvant therapy of surgically unresectable tumors and local recurrences.

Resumen Los sarcomas ginecológicos son infrecuentes y la localización de estos en vulva y vagina tienen una incidencia del 5% de todas las neoplasias malignas del tracto genital femenino. Presentamos el caso de una paciente de 54 años con diagnóstico de dermatofibrosarcoma protuberans en vulva, una patología infrecuente, con menos de 60 casos reportados a nivel mundial en esta localización anatómica. Clínicamente tiene un comportamiento localmente agresivo, debido a la proliferación de células fusiformes con pleomorfismo y frecuentes figuras de mitosis que infiltran la dermis reticular y tejido celular subcutáneo, dando origen a lesiones tumorales de tamaño variable y con altas tasas de recurrencia local. El tratamiento en primera elección es la escisión quirúrgica del tumor con cirugía micrográfica de Mohs u otras técnicas quirúrgicas para evaluación completa del margen periférico circunferencial y profundo. Sin embargo, la identificación de mecanismos de carcinogénesis donde se reconoce la translocación cromosómica t (17; 22) (q22; q13), formando al gen de fusión COL1A1-PDGFB, el cual participa estimulando la proliferación celular tumoral, ha permitido la utilización de los inhibidores de la tirosina quinasa como el imatinib para la realización de terapia neoadyuvante en casos de tumores irresecables quirúrgicamente y en recurrencias locales.

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.

Large anterior abdominal wall fibrosarcoma in a known case of recurrent dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans is a rare soft tissue sarcoma of low to intermediate malignant potential. Incidence is estimated to be 0.8 to 4.5 cases per million persons per year. The tumor is highly aggressive locally and is known to recur. Punch biopsy or excisional biopsy (in case of small tumors) are diagnostic. Computer tomography (CT) and magnetic resonance imaging (MRI) are useful in determining the size and extent of the tumor’s treatment is primarily surgical, with a wide local excision with at 2 cm margin. However, local recurrence after apparently adequate surgical excision is common. Mohs micrographic surgery has been recommended as it enables maximum preservation of tissue. When surgery is insufficient, imatinib mesylate is shown as a safe and effective treatment in dermatofibrosarcoma protuberans (DFSP), especially in cases of locally advanced or metastatic disease. Here, we report a case of a giant fibrosarcoma on the anterior abdominal wall, measuring 27×18×9 cm, which occurred in a recurrent dermatofibrosarcoma protuberans. Diagnosis was done by histopathological examination (HPE) of the previously excised tumor and CT was done. The tumor was excised with a 3 cm margin and extensive reconstruction of the anterior abdominal wall defect was done using synthetic mesh, myocutaneous flaps and split skin grafting. Keeping in view the recurrent nature of our case, large tumor size, DFSP-fibrosarcomatous (FS) transformation and close negative margins in the HPE of the postoperative specimen, the patient was planned for adjuvant radiotherapy.

Dermatofibrosarcoma protuberance: a recrudescent dermal malignancy

Dermatofibrosarcoma protruberens is a rare soft tissue tumour of low malignant potential, commonly seen on the trunk and extremities. It is a slow growing sarcoma, with a tendency for recurrence. Rarely, it may be a high-grade tumour. It responds well to wide local excision. Radiotherapy is beneficial for margin positive and high-grade tumours. We present a case of a 22 years old male with a dermatofibrosarcoma protruberens presenting as a swelling over the left inguinal region since, 4 years, that was excised with a 2cm margin. Patient was followed up for six months without any recurrence.

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.

Dermatofibrossarcoma protuberans de apresentação clínica atípica: relato de um caso / Atypical presentation of dermatofibrosarcoma protuberans: Case report

Introdução: O dermatofibrossarcoma protuberans (DFSP) é um sarcoma localmente agressivo que apresenta recidiva local em até 60% dos casos e raras metástases pulmonares. Métodos: Paciente masculino, de 41 anos, apresenta tumoração amolecida à palpação, localizada no hálux direito há dois anos. Histopatologia com células fusiformes em arranjo estoriforme. A imuno-histoquímica foi focalmente positiva para o CD34. Discussão: Algumas variantes clínicas são descritas. As principais são: lesões nodulares confluentes formando uma placa, muitas vezes com aspecto clínico semelhante ao de queloide; lesão tumoral; placa atrófica. Conclusão: Casos de DFSP simulando cisto subcutâneo foram encontrados na literatura, porém trata-se de uma apresentação clínica inusitada de DFSP.

Introduction: Dermatofibrosarcoma protuberans (DFSP) is a local aggressive sarcoma that presents 60% of recurrences. Rarely it presents lung metastasis too. Methods: 41 years old man presented a soft tumor in his right hallux for two years. Histopathology has shown spindle-shaped cells arranged in a storiform manner, and immunohistochemistry was CD34 positive. Discussion: The literature describes some clinical types of DFSP. The most common are: confluent nodules forming a plaque, sometimes similar to keloids, tumoral lesions, and atrophic plaques. Conclusion: DFSP cases simulating a subcutaneous cyst have been described in the literature; nevertheless, it is an unusual presentation of this tumor.

Dermatofibrossarcoma protuberante mimetizando queloide / Dermatofibrosarcoma protuberans simulating keloid

O dermatofibrossarcoma protuberante é um sarcoma localmente agressivo, de malignidade intermediária, que predomina na faixa de adultos jovens à meia-idade. Lesões congênitas ou na infância são raras, mas foram relatadas. Nos estágios precoces, pode ser mal diagnosticado e ser incompletamente excisado, o que aumenta o risco de recorrências. Relata-se o caso de paciente do sexo masculino, de 24 anos, com história de mácula hipercrômica em região abdominal desde o nascimento que evoluiu para tumoração após duas abordagens cirúrgicas. Biópsia e imuno-histoquímica confirmaram o diagnóstico de dermatofibrossarcoma protuberante, e a lesão foi excisada com margem de 3cm

Dermatofibrosarcoma protuberans is a locally aggressive sarcoma of intermediate malignancy that predominates in the range of young adults to middle age. Congenital or childhood lesions are rare but have been reported. It can be misdiagnosed and incompletely excised in the early stages, which increases the risk of recurrence. The present article reports the case of a 24-year-old male patient presenting a hyperchromic macula in the abdominal region from birth that evolved into a tumor after several surgical approaches. Biopsy and immunohistochemistry confirmed the diagnosis of dermatofibrosarcoma protuberans, and the lesion was excised with a 3 cm margin

Wounds after recision in recurrence of dermatofibrosarcoma protuberan in the left shoulder and chest repaired with anteromedial thigh perforator flap instead of anterolateral thigh perforator flap: a case report / 中华烧伤杂志

On October 23, 2017, a 52-year-old male patient with 3 recurrences of dermatofibrosarcoma protuberans in the left shoulder and chest was admitted to the Department of Burns and Plastic Surgery of Dali Bai Autonomous Prefecture People′s Hospital. Dermatofibrosarcoma protuberans on the skin were completely resected, leaving wound defect of 10 cm×10 cm. The wound was planned to be repaired by the transplantation of right anterolateral thigh perforator free flap. However, the anterolateral thigh perforator branch was absent during flap removal, and only one small perforating branch was found. Moreover, it was difficult to separate. Therefore, this flap cutting was given up. The anteromedial thigh perforator was explored through the same incision, and a thicker perforator was found, which was supplied by an independent iatrogenic artery. The length and diameter of the vascular pedicle matched with the blood vessels in the receiving site. An anteromedial thigh perforator flap (10 cm×10 cm) was cut to repair the defect. The postoperative 9-month follow-up revealed that the color, texture, and thickness of the flap were good, the two-point discrimination distance was 30 mm, and the linear scar remained at the donor site of right thigh.

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)

Pitfall management of dermatofibrosarcoma protuberans on right leg

Dermatofibrosarcoma protuberans (DFSP) is a rare, locally invasive soft tissue sarcoma. The local recurrence rate is high, in some studies upwards of 60%, likely reflecting a failure to remove occult extensions of tumor. Surgical excision has been the treatment of choice for the resection of DFSP. Any pitfall on management therapy of DFSP not only increase the recurrence rate but also add new problems to patients with DFSP. 58-year-old male patient, came with a local recurrent of DFSP on his right leg. Then performed excision with margin one centimetre, and closing defect using pedicle sural flap, durante surgery turned pedicle on the flap is too short and because of poor soft tissue handling pedicle was injured. It consulted into the vascular division for evaluated and treatment pedicle. Postoperative evaluation, the flap experiences bluish due to vascularization compromised. It was decided to expose the flap and the defect was covered with skin graft. 2 months postoperative evaluation found local recurrent on skin graft area. Excision margins between 2 to 5 cm can reduce the recurrence rate. Proper planning in designing flaps to cover defects and the ability of good soft tissue handling is required to avoid new problems on management DFSP.

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.

Presentación de un caso con dermatofibrosarcoma protuberante / Dermatofibrosarcoma protuberans case report

RESUMEN Paciente masculino de 60 años, con lesión en la piel de 1 mes y medio de evolución, localizada en el muslo derecho, de aspecto tumoral, que ocasionalmente sangraba y picaba, pero no resolvía con los tratamientos médicos indicados. En el Servicio de Dermatología del Hospital Clínico-Quirúrgico de Holguín, se diagnosticó como un dermatofibrosarcoma protuberante; por lo que se traslada al Servicio de Oncología del Hospital Vladimir Ilich Lenin, de Holguín, para cirugía; la cual se realizó con un amplio margen oncológico y una recuperación satisfactoria. Durante 3 años no se ha reportado recidivancia u otra complicación, a pesar de ser un tumor altamente invasivo localmente, con notable incidencia de recidivancia.

ABSTRACT A 60-year-old male patient with a skin lesion of 45 days of evolution, located on the right thigh, with a tumorlike appearance, which occasionally bled and itched and did not resolve with the indicated medical treatments. It was diagnosed as a protuberant dermatofibrosarcoma in the Dermatology Service of the Clinical-Surgical Hospital of Holguín, and it was transferred to the Oncology Service of the Vladimir Ilich Lenin Hospital in Holguín for surgery, with of oncology wide range and a satisfactory recovery during 3 years. Although the tumor is highly invasive locally, with a high incidence of recurrence no other complications have been reported.

PTEN Mutation Identified in Patient Diagnosed with Simultaneous Multiple Cancers / Journal of the Korean Cancer Association, 대한암학회지

PTEN hamartoma tumor syndrome is a spectrum of disorders characterized by unique phenotypic features including multiple hamartomas caused by mutations of the tumor suppressor gene PTEN. Cowden syndrome and Bannayan–Riley–Ruvalcaba syndrome are representative diseases, and both have several common clinical features and differences. Because PTEN mutations are associated with an increased risk of malignancy including breast, thyroid, endometrial, and renal cancers, cancer surveillance is an important element of disease management. We report a germline mutation of the PTEN (c.723dupT, exon 7) identified in a young woman with a simultaneous occurrence of breast cancer, dermatofibrosarcoma protuberans, and follicular neoplasm. This case suggests that it is critical for clinicians to recognize the phenotypic features associated with these syndromes to accurately diagnose them and provide preventive care.

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.