Histiocitoma fibroso maligno: estudio hisotpatologico e inmunohistoquímico / Malignant Fibrous Histiocytoma: Histopathological and Immunohistochemical Study

El histiocitoma fibroso maligno (MFH) es el tumor de tejido blando más común en adultos. Generalmente se considera que surge de las células mesenquimales primitivas que muestran diferenciación histiocítica y fibroblástica parcial. Las observaciones inmunohistoquímicas sugieren que la expresión de marcadores del músculo liso en el llamado MFH es el resultado de la diferenciación miofibroblástica. El presente estudio tiene como objetivo correlacionarse entre el subtipo histipatológico y los parámetros clínicos, calificar los casos de MFH dependiendo de los criterios histopatológicos para la clasificación, y examinar los casos inmunohistoquímicamente para la diferenciación miofibroblástica utilizando marcadores musculares lisos en casos de MFH como una ayuda para un diagnóstico preciso para un diagnóstico preciso. . Este estudio incluye 26 muestras de tejidos blandos diagnosticados como MFH recolectados de laboratorios histopatológicos privados y gubernamentales en Basrah durante el período de enero de 2000 a octubre de 2005. 4 casos adicionales (un leiomioma, dos fibromas y un fibrosarcoma se tomaron como control positivo y negativo. Los casos de MFH (77%) estaban en el grupo de edad de 45 a 60 años. La edad media fue de 53.5 años con una relación hombre / mujer de 1.3: 1. Diecinueve casos (73%) se ubicaron en las extremidades principalmente en las extremidades inferiores. Diecisiete años. Los casos (65.4%) fueron primarios. Veintidós (84.8%) eran de subtipo pleomórfico, dos eran mixoides y 2 eran inflamatorios. Todos los casos recurrentes se consideraban como el Grado III, de los diecisiete casos principales eran de grado III, por lo que veinte y veinte Tres casos (88.5%) fueron de grado III, los 3 casos restantes fueron de grado II. No se registró tumor de grado I. La mayoría de los casos de subtipo pleomórficos (95.5%) fueron de grado III. Ambos casos de subtipo mixoide fueron de grado Ii. Desmin expr Se encontró la en 3 casos (11.5%), ninguno para la actina del músculo liso o la proteína S-100. No hubo correlación entre la expresión de desmin y el sitio tumoral, subtipo o grado, así como, con la edad y el sexo de los pacientes. Se encontró una fuerte asociación entre la expresión de desmin y los tumores recurrentes del 33,3%

Malignant fibrous histiocytoma (MFH) is the most common soft tissue tumor in adult. It is generally regarded as arising from primitive mesenchymal cells that show partial histiocytic and fibroblastic differentiation. Immunohistochemical observations suggest that the expression of smooth muscle markers in the so called MFH is a result of myofibroblastic differentiation. The present study is aimed to correlate between histipathological subtype and clinical parameters, to grade the MFH cases depending on the histopathological criteria for grading, and to examine the cases immunohistochemically for myofibroblastic differentiation using smooth muscle markers in cases of MFH as an aid for accurate diagnosis. This study including 26 soft tissue specimens diagnosed as MFH collected from private and governmental histopathological laboratories in Basrah during the period from January 2000 to October 2005. Additional 4 cases (one leiomyoma, two fibromas and one fibrosarcoma were taken as control positive and negative. Twenty cases of MFH (77%) were in the age group 45-60 years. The mean age was 53.5 year with male to female ratio of 1.3: 1. Nineteen cases (73%) were located in the extremities mainly the lower limbs. Seventeen cases (65.4%) were primary. Twenty two (84.8%) were of pleomorphic subtype, two were myxoid and 2 were inflammatory. All the recurrent cases were regarded as grade III, from the seventeen primary cases fourteen were of grade III, so twenty three cases (88.5%) were of grade III, the remaining 3 cases were of grade II. No grade I tumor was recorded. The majority of pleomorphic subtype cases (95.5%) were of grade III. Both cases of myxoid subtype were of grade II. Desmin expression was found in only 3 cases (11.5%), none for smooth muscle actin or S-100 protein. There was no correlation between desmin expression and tumor site, subtype or grade, as well as, with age and sex of the patients. A strong association between desmin expression and recurrent tumors 33.3% was found.

Mixofibrosarcoma mandibular de bajo grado en un adulto / Low grade mandibular myxofibrosarcoma in an adult

Se describe el caso clínico de un paciente de 61 años de edad, con antecedente de buena salud, atendido en el St. Paul's Milennium Medical Hospital de Etiopía por presentar, desde hacía 2 años, una lesión en la encía marginal, con abombamiento de ambas corticales, que se extendía del primer premolar inferior derecho al izquierdo, por lo cual se decidió ingresarlo en el Servicio de Cirugía Maxilofacial para su estudio y tratamiento. Se trataba de un tumor poco frecuente y de gran tamaño, con amplia diseminación ganglionar hacia los niveles IV y V de ambas partes del cuello, de manera que se realizó el procedimiento quirúrgico. La evolución fue favorable en el posoperatorio inmediato y egresó de la institución 7 días después, pero continuó con radioterapia. Y seguimiento por consulta externa durante 24 meses después.

The clinical case of a 61-year-old patient, with a history of good health, treated at St. Paul's Milennium Medical Hospital in Ethiopia for presenting, for 2 years, a lesion in the marginal gum, with bulging of the gums, is described. both cortices, which extended from the lower right to the left first premolar, for which it was decided to admit him to the Maxillofacial Surgery Service for study and treatment. It was a rare and large tumor, with extensive lymph node dissemination to levels IV and V in both parts of the neck, so the surgical procedure was performed. The evolution was favorable in the immediate postoperative period and he was discharged from the institution 7 days later, but continued with radiotherapy. And follow-up by outpatient service for 24 months afterwards.

Fibrohistiocitoma maligno de face: uma apresentação rara / Malignant fibrohistiocytoma of face: a rare presentation

Contexto: O fibrohistiocitoma maligno é um sarcoma de tecidos moles muito agressivo, com rara apresentação limitada à pele e tecido subcutâneo em face. O diagnóstico é anatomopatológico com auxílio da imuno-histoquímica. Descrição do caso: Este artigo relata o caso de um paciente com diagnóstico de fibrohistiocitoma maligno restrito à face com boa resposta terapêutica após exérese cirúrgica. Discussão: Tendo em vista a raridade dessa afecção, dificuldade diagnóstica devido ao quadro inespecífico e com rápida evolução, é importante lembrar desse possível diagnóstico e atuar precocemente. Conclusões: O diagnóstico precoce interfere de forma significativa na evolução do quadro, sendo necessária a manutenção do acompanhamento oncológico e dermatológico com o intuito de detectar precocemente recidivas locais e metástases a distância.

Tumor Fibro-histiocítico Plexiforme de Vulva: Relato de Caso / Vulva Plexiform Fibrohistiocytic Tumor: Case Report / Tumor Fibrohistiocítico Plexiforme de la Vulva: Reporte de Caso

Introdução: O tumor fibro-histiocítico plexiforme é uma condição clínica rara, com cerca de 150 casos descritos na literatura. Relato do caso: Paciente, 23 anos, sexo feminino, portadora de lesão nodular em região vulvar sem características de malignidade em exames de ultrassonografia. Realizou acompanhamento clínico, tendo apresentado aumento do tamanho da lesão inicial, sendo optado então por realização de ressecção cirúrgica da lesão. Em estudo histopatológico, evidenciou-se lesão compatível com tumor fibro-histiocítico plexiforme, com margens cirúrgicas livres. No seguimento, a paciente foi encaminhada para avaliação oncológica com a intenção de realizar exames complementares de rastreio e descartar diagnósticos morfologicamente semelhantes. Diante dos exames complementares confirmando o diagnóstico inicial e sem evidência de doença metastática após excisão cirúrgica completa com margens livres, foi iniciado seguimento clínico. Conclusão: Relatar o caso é de extrema importância para divulgar a apresentação clínica, etapas diagnósticas, tratamento proposto e compartilhar informações acerca de evolução clínica apresentada.

Introduction: Plexiform fibrohistiocytic tumor is a rare clinical condition, with about 150 cases described in the literature. Case report: 23-years-old, female patient with nodular lesion in vulva without malignancy characteristics on ultrasound image. She underwent clinical follow-up and after an increase in the size of the lesion, surgical resection was opted. Histopathological findings suggested plexiform fibrohistiocytic tumor, with free surgical margins. In follow up, she was referred to oncological evaluation for screening complementary exams and rule out morphologically similar diagnoses. After the complementary exams confirming the initial diagnosis of plexiform fibrohistiocytic tumor and without evidence of metastatic disease after complete surgical excision with free margins, the patient is in clinical follow-up. Conclusion: Reporting the case is extremely important to disseminate the clinical presentation, diagnostic steps, proposed treatment and share information about the clinical evolution presented.

Introducción: El tumor fibrohistiocítico plexiforme es una condición clínica poco frecuente, con unos 150 casos descritos en la literatura. Reporte del caso: Paciente de 23, sexo feminino, años con lesión nodular en una región vulvar sin características malignas en los exámenes de ultrasonido. Se sometió a un seguimiento clínico, habiendo presentado un aumento en el tamaño de la lesión inicial, y se decidió realizar la resección quirúrgica de la lesión. En un estudio histopatológico, se evidenció una lesión compatible con tumor fibrohistiocítico plexiforme con márgenes quirúrgicos libres. Fue referida para la evaluación oncológica, realizó pruebas complementarias para el cribado y para descartar diagnósticos morfológicamente similares. En vista de las pruebas complementarias que confirman el diagnóstico inicial y sin evidencia de enfermedad metastásica después de una escisión quirúrgica completa con márgenes libres, se inició el seguimiento clínico. Conclusión: Informar del caso es extremadamente importante para difundir la presentación clínica, etapas diagnósticas, tratamiento propuesto y compartir información sobre la evolución clínica presentada.

Rapidly progressive malignant fibrous histiocytoma of right atrium: a rare case report

Abstract We are going to present a case of malignant fibrous histiocytoma in the right atrium, which is a very rare entity. The patient had a right atrial mass, which prolapsed through the tricuspid valve into the right ventricle, causing functional tricuspid valve stenosis. The tumor was completely resected and the patient had an uneventful postoperative period. Histopathological examination reported malignant fibrous histiocytoma. The patient presented to the emergency department five weeks after discharge with dyspnea and palpitation. Echocardiography and magnetic resonance imaging revealed recurrent right atrial tumor mass. His clinical status has worsened, with syncope and acute renal failure. On the repeated echocardiography, suspected tumor recurrence was observed in left atrium, which probably caused systemic embolization. Considering the aggressive nature of the tumor and systemic involvement, our Heart Council decided to provide palliative treatment by nonsurgical management. His status deteriorated for the next few days and the patient succumbed to a cardiac arrest on the 4th day.

Fibrohistiocitoma maligno gigante de face: tratamento reparador microcirúrgico utilizando retalho miocutâneo transverso do reto do abdome - Relato de caso / Giant malignant fibrous histiocytoma of the face: case report of microsurgical repair using a transverse rectus abdominis myocutaneous flap

Introdução: A ressecção de tumores invasivos de cabeça e pescoço pode resultar em defeitos extensos e complexos exigindo reparação imediata. Uma das opções de reparação é a transferência, utilizando técnica de microcirurgia vascular, do retalho musculocutâneo do reto abdominal pediculado nos vasos epigástricos inferiores profundos (TRAM). O presente estudo tem como objetivo registrar um procedimento utilizado no tratamento reparador microcirúrgico de fibrohistiocitoma maligno gigante de face com retalho TRAM. Relato de Caso: Paciente procurou atendimento médico devido a lesão tumoral gigante em hemiface direita. Foi realizada a tomografia computadorizada do crânio revelando volumoso processo expansivo de aspecto vegetante com limites mal definidos. Após os procedimentos básicos no pré-operatório, realizou-se a exérese do tumor que acometia músculos masseter e temporal direito, glândula parótida, assoalho orbitário à direita e osso malar. Posteriormente, retirou-se o retalho microcirúrgico do músculo reto do abdome em conjunto com a artéria epigástrica inferior profunda através de incisão cirúrgica da área hipogástrica. Em seguida, dissecção da artéria e veia facial utilizando microscópio e anastomoses venosa e arterial. Quanto à evolução retalho apresentou-se íntegro, com boa perfusão, sem sinais de infecção. Conclusões: A reconstrução facial microcirúrgica oferece liberdade ao cirurgião de cabeça e pescoço para realizar grandes ressecções tumorais.

Introduction: The resection of invasive tumors of the head and neck can result in extensive and complex defects requiring immediate repair. One repair option is the transfer of a transverse rectus abdominis myocutaneous (TRAM) flap pedicled on deep inferior epigastric vessels using vascular microsurgery. This study aimed to register a procedure used in the microsurgical treatment of giant malignant fibrous histiocytoma of the face using a TRAM flap. Case Report: A male patient sought medical care for a giant tumoral lesion in the right hemiface. Computed tomography of the skull revealed a voluminous expansive process of vegetating aspect with poorly defined borders. The excision of the tumor affected the right masseter and temporalis muscles, parotid gland, and right orbital and malar bones. Subsequently, microsurgical withdrawal of the TRAM flap was performed with the deep inferior epigastric artery through a surgical incision in the hypogastric area. Dissection of the facial artery and vein under microscopy and venous and arterial anastomoses followed. The flap was intact with good perfusion and no signs of infection. Conclusions: Microsurgical facial reconstruction allows head and neck surgeons to resect large tumors.

Undifferentiated pleomorphic sarcoma of the orbital region: a case report / Sarcoma pleomórfico indiferenciado da região orbitária: relato de caso

ABSTRACT Undifferentiated pleomorphic sarcoma (UPS) is an extremely rare tumor that occurs in the head and neck region. Here, we report a unique case of a primary undifferentiated pleomorphic sarcoma in the orbital region. A 35-year-old woman presented with a progressive proptosis and periocular edema for 1 year. She had no previous history of surgery, skin malignancy, or radiation. Imaging tests showed an extraconal mass, not involving the muscles. The tumor was surgically removed and adjuvant radiotherapy was required after histological examination, which showed an undifferentiated pleomorphic sarcoma of the orbit. There was no recurrence after 1 year of follow-up. Though rare, undifferentiated pleomorphic sarcoma should be included in the differential diagnoses of orbital tumors.

RESUMO O sarcoma pleomórfico indiferenciado (SPI) é um tumor extremamente raro na região da cabeça e pescoço. Relatamos um caso de um sarcoma pleomórfico indiferenciado primário na região orbital. Uma mulher de 35 anos apresentou proptose progressiva e edema periocular há um ano. Ela não tinha histórico prévio de cirurgia, malignidade da pele ou radiação. Exames de imagem mostraram uma massa extraconal, poupando os músculos. O tumor foi removido cirurgicamente e foi necessária radioterapia adjuvante após o resultado histopatológico. O exame histológico demonstrou um sarcoma pleomórfico indiferenciado da órbita. Não houve recidiva após 1 ano de seguimento. Apesar de raro, o sarcoma pleomórfico indiferenciado deve ser incluído no diagnostico diferencial de qualquer tumor originado na órbita.

Histiocitoma fibroso maligno metastásico en estómago / Metastatic malignant fibrous histiocytoma in the stomach

El histiocitoma fibroso maligno es el sarcoma de partes blandas más frecuente de la adultez tardía. Se desarrolla habitualmente en los miembros inferiores y en el retroperitoneo. Su pronóstico es malo, la recidiva local y las metástasis a distancia. Se presenta el caso de un paciente con metástasis en el estómago de un histiocitoma fibroso maligno. El objetivo de esta publicación es la presentación de una forma poco común de diseminación metastásica del histiocitoma fibroso maligno(AU)

Malignant fibrous histiocytoma is the most common soft tissue sarcoma of late adult life. It usually develops in the lower extremities and in the retroperitonium. Prognosis is fairly poor; local relapse and distant metastasis are the common natural history. The is presented of a patient with stomach metastasis of malignant fibrous histiocytoma. This paper is aimed at presenting a rare way of metastatic dissemination of the malignant fibrous histiocytoma(AU)

Malignant fibrous histiocytoma in a patient presenting with urinary system symptoms

Summary Malignant fibrous histiocytoma is a rare tumor. It is most commonly seen in individuals between the fifth and seventh decades of life, in extremities, and less frequently in the retroperitoneum. Although its etiology is not clearly known, radiotherapy, chemical agents, previous history of surgery, trauma and fracture, and Hodgkin lymphoma have been blamed. Leiomyosarcoma, liposarcoma and rhabdomyosarcoma should be taken into account in differential diagnosis. It is seen on computed tomography as a mass lesion with irregular borders and density similar to that of the surrounding muscle tissue. Necrotic and hemorrhagic components in the mass are characterized as heterogeneous low density areas. Fluid-fluid levels can be detected by computed tomography and magnetic resonance imaging.

Histiocitoma fibroso maligno cerebral en paciente pediátrico: Presentación de un caso y revisión de la literatura / Malignant Fibrous Histiocytoma of the Brain in Pediatric Patient: A Case Report and Literature Review

Los tumores cerebrales son la segunda causa de neoplasias malignas en niños y los sarcomas primarios en el sistema nervioso central son extremadamente raros. Por esta razón, presentamos los resultados del estudio de un caso en una menor de edad y la revisión realizada sobre la fisiopatología, manifestaciones clínicas y hallazgos en las imágenes de resonancia magnética cerebral de esta patología. Se trata de una niña de 7 años de edad que ingresa al servicio de urgencias con cefalea, sintomatología sugerente de hipertensión endocraneana y disminución de la fuerza del hemicuerpo derecho, por lo que se le practica resonancia magnética cerebral (RM) con medio de contraste, con hallazgo de masa extraaxial temporoparietal izquierda, que desplaza la línea media. La paciente es valorada por neurocirugía y remitida a craneotomía frontoparietal izquierda, con resección completa del tumor. El informe de patología muestra sarcoma fusocelular de alto grado, compatible con histiocitoma fibroso maligno.

Brain tumours are the second cause of malignant neoplasms in children while primary sarcomas in the central nervous system (SNC), are extremely rare. For this reason, we present the results of the study of a case of a girl and revision on the pathophysiology, clinical manifestations, and findings on brain MRI of this pathology. We report the case of a sevenyear- old patient admitted to the emergency department with headache and symptoms suggestive of intracranial hypertension, and decreased strength of the right side of the body. In brain MRI, we found a left temporoparietal extra-axial mass, displacing the midline. The patient was examined by the neurosurgeon who performed a left frontoparietal craniotomy, with complete tumor resection. The pathology study showed high-grade spindle cell sarcoma, classified as malignant fibrous histiocytoma.

Neoplasmas em suínos: 37 casos / Neoplasia in swine: 37 cases

Neoplasmas em suínos são raros. Esse trabalho descreve os neoplasmas encontrados em suínos na rotina diagnóstica de um laboratório de patologia veterinária localizado na Região Central do Rio Grande do Sul. Durante um período de 49 anos, 2.266 casos de várias afecções em suínos foram encontrados, dos quais 37 (1,6%) eram neoplasmas. Em ordem decrescente de frequência, os seguintes neoplasmas foram encontrados: Linfoma (11/37), nefroblastoma (11/37), melanoma (8/37) e papiloma (2/37). Adicionalmente, um caso de cada um dos seguintes tumores foi observado: Adenoma hepatocelular, carcinoma hepatocelular, colangiocarcinoma, histiocitoma fibroso maligno e sarcoma granulocítico. O aspecto macroscópico e histológico desses tumores é descrito e a sua epidemiologia é comparada com os dados disponíveis na literatura para neoplasia suína.

Neoplasms in swine are rare. This paper describes neoplasms found in swine in the diagnostic routine of a veterinary pathology laboratory in the Central Region of Rio Grande do Sul, Brazil during a 49-year period, during which 2,266 cases of the various affections in swine were diagnosed. Of those 37 cases (1.6%) were neoplasms. In decreasing order of prevalence, the following neoplasms were found: lymphoma (11 out of 37 cases), nephroblastoma (11/37), melanoma (8/37), and papilloma (2/37). Adenoma hepatocelular, carcinoma hepatocelular, cholangiocarcinoma, malignant fibrous histiocytoma, granulocytic sarcoma were each found in one case out of the 37 cases. The gross and histological aspects of these tumors are described and their epidemiology is compared with the data available in the literature for neoplasia in swine.

Fibrohistiocitoma maligno cutáneo con metástasis satélites / Malígnant fibrous histiocytoma with satellite metastasis

El fibrohistiocitoma maligno es un tumor subcutáneo de larga evolución y localizado principalmente en extremidades. Se presenta el caso de una paciente de 77 años con una tumoracián nodular de superficie ulcerada con múltiples pápulas perilesionales en su pierna derecha. Se realizó la confirmación histopatológica y de inmunohistoquimica compatible con Fibrohistiacitoma maligno asociado a metástasis cutáneas satélites.

Malignant fibrous histiocytoma is a subcutaneous tumor of long evolution, more frequently located in the extremities. We present the case of a 77 year- old female with a nodular ulcerated tumor with multiple perilesional papules on her right lower leg. Histopathology and immunohistochemistry were consistent with cutaneous malignant fibrous histiocytoma associated to satellite metastasis.

Fibrohistiocitoma maligno del pie con metástasis a piel, pleura y pulmones en un paciente varón / Malignant fibrous histiocytoma of the foot with skin, pleural and lung metastasis

El fibrohistiocitoma maligno está  considerado dentro del grupo de sarcomas de partes blandas, de los cuales el más común. Tiene una gran agresividad, un pronóstico infausto y una supervivencia corta. En la mayoría de los casos se encuentra localizado en miembros inferiores y afecta tejidos profundos, como músculo estriado, tejido nervioso, vascular, conectivo, restos embrionarios, etc. Raramente se encuentra en piel, siendo las metástasis a pulmones la más frecuente.Se presenta el caso de un paciente varón de 42 años, con diagnóstico histológico de fibrohistiocitoma maligno en miembros inferiores en primer dedo del pie izquierdo, el cual fue intervenido quirúrgicamente. El paciente, por decisión propia, no siguió ninún tratamiento ni control médico siguiendo la enfermedad en su evolución natural. Dicho paciente se hospitaliza por presentar gran dificultad respiratoria, dolor torácico, baja de peso, derrame pleural propio de la metastasis a nivel pulmonar, confirmém dose mediante el estudio histopatológico de la biopsia practicada y de igual manera demostrán dose metástasis a nivel cutánea. Se resalta las características clínicas, la evolución de la enfermedad no sujeta a control y sus complicaciones. De infrecuente diagnóstico en nuestra institución: Hospital Arzobispo Loayza.

The malignant fibrous histiocytoma in the most common soft-tissue sarcoma. It is a very aggressive tumor that has a bad prognosis and short survival time and is most commonly located in the legs. The malignant fibrous histiocytoma affects deep tissue, such as striated muscle, nervous, vascular, connective and embryonic tissue. It is very uncommon to find this type of sarcoma in the skin. The most common metastasis is to the lungs.In this article we present the case of a male patient of 42 years of age with the histopathological diagnosis of malignant fibrous histiocytoma of the legs. It was located in the first toe of the left foot and was surgically removed. Because the patient decided not to take any treatment or to attend to his medical follow-up visits, the disease went through its natural history. The patient seeked medical help because of great respiratory difficulty, chest pain, weigth loss and pleural effusion. The last one is a characteristic sing of metastasis to the lung, which was confirmed by the histopathological study. Skin metastasis was also determined by the biopsy of the foot tissue. In this article we highlight the clinical characteristics, the evolution of the disease without any treatment or medical control and its complications. It is an unfrequent diagnosis in our institution: Hospital Nacional Arzobispo Loayza.

Sarcoma pleomórfico em úlcera de Marjolin / Pleomorphic sarcoma in Marjolin's ulcer

Úlcera de Marjolin é uma transformação maligna em tecido cutâneo cronicamente inflamado ou traumatizado, que ocorre especialmente após queimaduras. O carcinoma de células escamosas é o tipo histológico mais encontrado nas úlceras de Marjolin, seguido de carcinoma basocelular e melanoma maligno. Sarcomas em úlcera de Marjolin são raros, correspondendo a aproximadamente 5% dessas degenerações malignas. Neste artigo é descrito o caso de paciente do sexo feminino, vítima de queimadura há 42 anos, com grande ulceração em dorso. A biópsia dessa ulceração evidenciou sarcoma pleomórfico de alto grau em úlcera de Marjolin. A paciente foi submetida a ressecção da ulceração e enxerto de pele no local, seguidos de radioterapia e quimioterapia adjuvantes. Em 3 anos de seguimento, a paciente não apresentou recidiva da neoplasia. Úlceras de Marjolin são neoplasias malignas de comportamento agressivo, com alto índice de metástases regionais. A importância de seu entendimento está na necessidade de prevenção das mesmas, com o tratamento adequado dos pacientes queimados, evitando-se a cicatrização por segunda intenção. Sarcomas em úlcera de Marjolin são considerados raros, com poucos casos relatados na literatura, o que demonstra a importância deste relato.

Marjolin's ulcer is a malignant transformation of traumatized or chronically inflamed cutaneous tissue that occurs after burns. The most common histological type of carcinoma found in Marjolin's ulcers is squamous cell carcinoma, followed by basal cell carcinoma and malignant melanoma. Sarcomas in Marjolin's ulcers are rare, representing approximately 5% of these malignant degenerations. In this report, we describe the case of a female patient who was burned 42 years prior, with a large ulceration on her back. Biopsy of the ulceration showed a high-grade pleomorphic sarcoma in the Marjolin's ulcer. The patient underwent resection of the ulceration and a skin graft followed by radiation therapy and adjuvant chemotherapy. In 3 years of follow-up, the patient had no tumor recurrence. Marjolin's ulcers are aggressive and have a high rate of regional metastases. It is important that clinicians develop an understanding of their prevention by properly treating burns. Sarcomas in Marjolin's ulcers are rare and few cases have been reported in the literature, which demonstrates the importance of this report.

Recurrence of atypical fibroxanthoma. Diagnosis and treatment

Introduction: The soft tissue sarcomas (SPM) accounts for only 1% of malignant tumors of the adult population. The SPM is the most frequent malignant fibrous histiocytoma (MFH) that exhibits behavior characterized by the tendency to invasion of adjacent tissue and metastatic spread early. One of its variants is the atypical fibroxanthoma (FA). Objective: To describe a case of probable recurrence of AF underwent surgical treatment and presentation of a literature review. Case report: Patient female, 63 years, presenting with a mass in the face about four inches and a history of prior resection of the lesion in the same topography. The patient underwent surgical resection with a diagnosis of AF. Conclusion: The FA is a rare tumor histological diagnosis difficult. The correct histological diagnosis and patient follow-up are essential...

A fifty-year review of soft tissue sarcomas in Jamaica: 1958-2007 / Cincuenta años de revisión de los sarcomas de tejidos blandos en Jamaica: 1958-2007

OBJECTIVE: To determine the distribution of histologic subtypes of soft tissue sarcomas (STS) in Kingston and St Andrew, Jamaica, according to age and topography. METHODS: From the Jamaica Cancer Registry (JCR) archives, all cases of STS diagnosed between 1958 and 2007 were extracted. For each case, age, gender, histological diagnosis and anatomical site of tumour were recorded. Patients were categorized according to age at diagnosis as: children (0-14 years) and adults (> 14 years), and the distribution of histologic diagnoses with respect to age and anatomical site were analysed. RESULTS: There were 432 cases (67 children, 364 adults, one person of unknown age) of STS recorded in the JCR over the 50-year period (218 males, 214 females). The commonest STS in adults were "sarcoma, not otherwise specified [NOS]" (20.1%), malignant fibrous histiocytoma [MFH] (17.9%), fibrosarcoma (12.4%), liposarcoma (10.7%) and malignant peripheral nerve sheath tumour [MPNST] (10.2%). In children, they were neuroblastoma (38.8%), rhabdomyosarcoma (23.9%), "sarcoma, NOS" (9%), fibrosarcoma (6%) and MFH (6%). In adults, the lower limb was the commonest location, followed by trunk and/or upper limb for MFH, fibrosarcoma and liposarcoma, and head and neck for MPNST. In children, head and neck was the commonest site for rhabdomyosarcoma, head and neck and upper limb for MFH, retroperitoneum for neuroblastoma and trunk for fibrosarcoma. CONCLUSION: A high proportion of soft tissue sarcomas in Jamaica are unclassified and the anatomical distribution of common classified sarcomas shows some differences with the literature. Limited access to immunohistochemistry/molecular diagnostics and increasing core biopsy diagnosis may contribute to these phenomena.

OBJETIVO: Determinar la distribución de subtipos histológicos de sarcomas de tejido blando (STB) en Kingston y Saint Andrew, Jamaica, según la edad y la topografía. MÉTODOS: De los archivos del Registro de Cáncer de Jamaica, se extrajeron todos los casos de STB diagnosticados entre 1958 y 2007. Para cada uno de los casos, se registró la edad, el género, el diagnóstico histológico, y el sitio anatómico del tumor. Los pacientes fueron clasificados de acuerdo con la edad en el momento del diagnóstico, bajo las categorías de niños (0-14 años) y adultos (> 14 años), y se analizó la distribución de diagnósticos histológicos con respecto a la edad y el sitio anatómico. RESULTADOS: Se registraron 432 casos de STB (67 niños, 364 adultos, una persona de edad desconocida) en el JCR en un período de 50 años (218 varones, 214 hembras). Los STB más comunes en los adultos fueron "el sarcoma no especificado [NE]" (20.1%), el histiocitoma fibroso maligno [HFM] (17.9%), el fibrosarcoma (12.4%), el liposarcoma (10.7%), y el tumor maligno de la vaina del nervio periférico [TMVNP] (10.2%). En los niños, se trató de los neuroblastomas (38.8%), los rabdomiosarcomas (23.9%), "los sarcomas NE" (9%), los fibrosarcomas (6%), y los HFM (6%). En los adultos, los miembros inferiores fueron el lugar más común, seguido del tronco y/o los miembros superiores para el HFM, el fibrosarcoma y el liposarcoma; y la cabeza y el cuello para el TMVNP. En los niños, la cabeza y el cuello fueron el sitio más común para el rabdomiosarcoma; la cabeza, el cuello y los miembros superiores para el HFM; el retroperitoneo para el neuroblastoma; y el tronco para el fibrosarcoma. CONCLUSIÓN: Una proporción alta de sarcomas de tejidos blandos en Jamaica no están clasificados, y la distribución anatómica de sarcomas clasificados comunes muestran algunas diferencias con la literatura. El acceso limitado a los diagnósticos moleculares/inmunohistoquímicos, y el aumento de los diagnósticos centrales, pueden contribuir a estos fenómenos.

Case for diagnosis / Caso para diagnóstico

Atypical fibroxanthoma is a rare cutaneous tumor found mainly in elderly people on sun-exposed areas of the body. Histologically, atypical fibroxanthoma is considered a malignant fibrous histiocytoma with bizarre neoplastic cells, marked pleomorphism, hyperchromatic nuclei and abundant mitoses. It must be differentiated from other skin tumors, usually by immunohistochemistry, since its diagnosis is made by exclusion.

O fibroxantoma atípico é um tipo de neoplasia cutânea maligna rara, encontrado principalmente em idosos, em áreas fotoexpostas. Na histologia, o fibroxantoma atípico é uma neoplasia fibro-histiocítica dérmica, de células fusiformes e epitelioides, algumas vezes bizarras, com acentuado pleomorfismo, apresentando núcleos hipercromáticos e mitoses abundantes. Deve ser diferenciado de outros tumores de pele, principalmente através da imunoistoquímica, já que seu diagnóstico é de exclusão.