Undifferentiated pleomorphic sarcoma: prognostic factors in 42 extremity cases / Sarcoma pleomórfico indiferenciado: fatores de prognóstico em 42 casos em extremidades

ABSTRACT Introduction: Soft tissue undifferentiated pleomorphic sarcoma (UPS) in extremities is considered a rare neoplasm, corresponding to 5% of soft tissue sarcomas (STS) today. The objective was to evaluate prognostic factors related to death, local recurrence (LR), and impact on survival rates. Methods: A retrospective study including 42 patients with UPS in extremities treated surgically in a single center. Comparisons were made between demographic data, characteristics of the neoplasia, and treatment. Between the variables with statistical significance, logistic regression analysis was used. Survival rates were evaluated using Kaplan-Meier plots. To compare the effect of variables on survival rates, the Log-Rank test was used. Results: Age group of patients was from 25 to 85 years (mean 58 years), with a mean follow-up of 29.6 months. The variables with the highest effect on survival rates were sizes larger than 15 cm (T4) with p = 0.01, presence of metastatic lesions, and prognostic stage IV according to the American Joint Committee of Cancer (AJCC) with p < 0.001. The mean survival was 25.9 months. Metastasis and stage IV of AJCC were associated with a reduction in patient survival (17.8 months) with Log-Rank test p < 0.001. Conclusion: The main factors of poor prognosis related to mortality and reduction of survival of UPS in extremities were metastatic lesions and stage IV of AJCC. Level of Evidence III, Retrospective Study.

RESUMO O sarcoma pleomórfico indiferenciado (SPI) de tecidos moles em extremidades, neoplasia rara, correspondente a 5% dos sarcomas de tecidos moles (STM). Objetivo: Avaliar fatores de prognóstico relacionados a óbito, recorrência local (RL) e impacto na sobrevida geral. Métodos: Estudo retrospectivo de 42 pacientes com SPI tratados cirurgicamente em centro único. Foram comparados dados demográficos, características da neoplasia e de tratamento; e para as variáveis com significância estatística, foi realizada análise de regressão logística. A sobrevida foi avaliada através de gráficos de Kaplan-Meier; e os efeito das variáveis sobre a sobrevida, por meio do teste de log-rank. Resultados: Os pacientes tinham idades entre 25 e 85 anos (média de 58 anos), com seguimento ambulatorial médio de 29,6 meses. As variáveis com maior relação com o desfecho óbito foram tamanho maior que 15 cm (T4), com p = 0,01, metástases e estágio IV de prognóstico da American Joint Committee on Cancer (AJCC), com p < 0,001. A sobrevida média dos pacientes foi de 25,9 meses. Presença de metástase e estágio IV da AJCC foram associados à redução na sobrevida dos pacientes (17,8 meses; p < 0,001). Conclusão: Os principais fatores de mau prognóstico relacionados ao óbito e à redução da sobrevida dos pacientes com SPI foram doença metastática e estágio IV da AJCC. Nível de Evidência III, Estudo Retrospectivo.

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.

Histiocitoma fibroso maligno no tornozelo: relato de caso / Malignant fibrous histiocytoma in ankle: case report

Histiocitoma fibroso maligno (MFH) ou sarcoma pleomórfico indiferenciado (UPS) é um sarcoma moderadamente agressivo, capaz de invadir estruturas adjacentes. Trata-se de neoplasia mesenquimal que predomina em homens entre a sexta e sétima décadas de vida. Localiza-se, principalmente, nos membros inferiores, podendo acometer cabeça e pescoço, tronco e retroperitônio, com tendência à recorrência e à metástase local. O presente relato tem como objetivo apresentar um caso de MFH no tornozelo de uma mulher de 49 anos, com invasão óssea adjacente, que evoluiu com amputação transtibial. São abordados aspectos clínicos, radiológicos, histopatológicos e terapêuticos, salientando-se a importância do diagnóstico precoce.

Malignant fibrous histiocytoma (MFH) or undifferentiated pleomorphic sarcoma (UPS) is a sarcoma capable of invading adjacent structures. It is a mesenchymal neoplasia that predominates in men between the sixth and seventh decades of life. It is located mainly in the lower limbs and may affect the head and neck, trunk, and retroperitoneum, presenting a tendency to recurrence and local metastasis. This report aims to present a case of MFH in the ankle of a 49-year-old woman with an adjacent bone invasion, which evolved with transtibial amputation. Clinical, radiological, histopathological, and therapeutic aspects were addressed, highlighting the importance of early diagnosis

Dermatoscopia do dermatofibroma aneurismático: relato de dois casos / Dermoscopy of aneurysmal dermatofibroma: report of two cases

O dermatofibroma (DF) aneurismático é um tumor benigno raro, de origem na derme, considerado uma variante de dermatofibroma. É prevalente nos membros inferiores de mulheres acima de 30 anos. Sua etiologia é desconhecida, e o exame histopatológico confirma o diagnóstico. Este trabalho apresenta dois casos de DF aneurismático, em pacientes jovens, do sexo masculino, e com lesões semelhantes: nódulo hipercrômico único, de aproximadamente 1,5cm e crescimento progressivo. O objetivo deste relato é demonstrar uma variante pouco frequente de dermatofibroma e ressaltar o possível diagnóstico diferencial com outros tumores por meio do exame dermatoscópico.

Aneurysmal dermatofibroma (DF) is a rare benign tumor originating in the dermis, considered a variant of dermatofibroma. It is more prevalent in women over 30 years of age, in the lower limbs. Its etiology is unknown and histopathological examination confirms the diagnosis. This study reports two cases of aneurysmal DF in young patients, both men, presenting similar lesions: single hyperchromic nodule, with approximately 1.5 cm and progressive growth. This report aims to demonstrate an uncommon variant of dermatofibroma and to highlight the possible differential diagnosis with other tumors through dermoscopic examination

First report of hemosiderotic adenodermatofibroma in Brazil / Primeiro relato de adenodermatofibroma hemossiderótico no Brasil

ABSTRACT Hemosiderotic adenodermatofibroma is a recently recognized lesion, characterized by a dermal nodule with cystic structures of an apocrine gland, surrounded by a dermatofibroma-like stroma with hemosiderotic macrophages. We present the first case report of this entity in Brazil together with representative images, in addition to a review on the subject and discussion about the apocrine origin of this lesion.

RESUMEN El dermatoadenofibroma hemosiderótico es una lesión recientemente descrita, caracterizada por un nódulo dérmico con estructuras quísticas de una glándula apocrina, rodeado por un estroma del tipo dermatofibroma con macrófagos hemosideróticos. Presentamos el primer reporte de caso de esa entidad en Brasil, junto a imágenes representativas, además de una revisión del tema y discusiones acerca del origen apocrina de esa lesión.

RESUMO Adenodermatofibroma hemossiderótico é uma lesão recentemente conhecida, caracterizada por um nódulo dérmico com estruturas císticas de uma glândula apócrina, circundado por um estroma do tipo dermatofibroma com macrófagos hemossideróticos. Apresentamos o primeiro relato de caso dessa entidade no Brasil junto a imagens representativas, além de uma revisão do assunto e discussões sobre a origem apócrina dessa lesão.

Fibriohistiocitoma en fosa infratemporal derecha. Reporte de un caso / Right infratemporal fossa Fibriohistiocytoma. Case Report

RESUMEN Fundamento: El fibriohistiocitoma benigno es infrecuente que se presente en la adolescencia y menos con la ubicación topográfica inusual en fosa infratemporal derecha. Objetivo: Ilustrar la presentación de un fibriohistiocitoma benigno en fosa infratemporal derecha en un paciente masculino de 17 años de edad. Presentación de caso: Se reportó un caso en un paciente masculino de 17 años de edad, con un fibriohistiocitoma que tiene un comportamiento biológico benigno y se localiza en fosa infratemporal derecha; bajo el músculo temporal, en el servicio de Cirugía Maxilofacial de Sancti Spíritus. Conclusiones: El fibrohistiocitoma puede aparecer en cualquier parte del cuerpo es poco frecuente en cabeza y cuello, menos aún en fosa infratemporal, como se reportó en este caso.

ABSTRACT Background: Benign fibriohistiocytoma is uncommon to occur in adolescence and less with the unusual topographic location in the right infratemporal fossa. Objective: To illustrate the presentation of a benign fibriohistiocytoma in the right infratemporal fossa in a 17-year-old male patient. Case presentation: A case was reported in a 17-year-old male patient, with a fibriohistiocytoma that has a benign biological behavior and is located in the right infratemporal fossa; under the temporal muscle, in the Maxillofacial Surgery service of Sancti Spíritus. Conclusions: Fibrohistiocytoma can appear in any part of the body, it is rare in the head and neck, even less in the infratemporal fossa, as reported in this case.

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)

Histiocitoma fibroso maligno pleomórfico estoriforme del brazo izquierdo / Malignant pleomorphic storiform fibrous histiocytoma of the left arm

Fundamento: el histiocitoma fibroso maligno es el sarcoma de partes blandas más frecuente en la adultez. Se encuentra en su mayoría en las extremidades en un 70-75 % y los miembros inferiores son los más afectados con el 60 %. Objetivo: presentar un caso de histiocitoma fibroso maligno pleomórfico estoriforme del brazo izquierdo que recibió tratamiento quirúrgico en el Hospital Universitario Manuel Ascunce Domenech de Camagüey en el 2016. Caso clínico: paciente masculino de 60 años, con aumento de volumen de la cara posterolateral proximal del brazo izquierdo, redondeado y acompañado de circulación colateral. Se palpó tumoración de 20 cm de diámetro, de superficie lisa, firme, fija a planos profundos y dolorosa a la palpación con auscultación negativa. La radiografía simple de húmero izquierdo informó una radiopacidad homogénea de las partes blandas circundantes del extremo proximal del húmero, sin reacción perióstica ni lesión ósea. La ecografía de partes blandas demostró imagen ecogénica de 200 x 170 mm, que se interponía entre los músculos deltoides y tríceps braquial, con seudocápsula bien definida y que respetaba el húmero. Se realizó biopsia por aspiración que informó una neoplasia maligna mesenquimátosa, posible histiocitoma maligno. Se decidió tratamiento quirúrgico consistente en excéresis del tumor con márgenes oncológicos. La biopsia definitiva luego de la inmunohistoquímica, informó un histiocitoma fibroso maligno pleomórfico estoriforme. Se remite para tratamiento oncológico con radioterapia, presentó una recidiva local por lo que se decidió amputación interescapulotorácica. Conclusiones: el histiocitoma fibroso maligno es el tumor maligno de partes blandas más frecuente del adulto. Presenta diferentes variantes histológicas por lo que la inmunohistoquímica es clave para el diagnóstico de certeza. La cirugía y la radioterapia son la mejor combinación terapéutica. La edad, la profundidad de la lesión, la variante histológica, la localización, la ausencia de metástasis y el tamaño son los factores pronósticos más importantes.

Background: malignant fibrous histiocytoma is the most frequent soft tissue sarcoma in adulthood. It is seen in extremities in about 70-75 % and lower limbs are the most affected ones with 60 %. Objective: to present a case of malignant storiform pleomorphic histiocitoma of the left arm who underwent surgical treatment at universitary hospital Manuel Ascunce Domenech of Camagüey in 2016. Clinical case: a 60 year old male patient with a round increasing volume in posterolateral proximal left arm with collateral circulation. It was palpated a 20 cm diameter smooth, firm, fix to deep planes and painful mass with negative auscultation. Simple left humerus radiography informed a homogeneous radiopacity in surrounding soft tissues, no periosteal reaction or bone damage. Ecography of soft tissues: hypoecoic image of 200 x 170 mm between triceps and deltoid muscles reaction, with well defined pseudocapsule and no bone affection. An aspiration biopsy was done which inform a malignant mesenchimal neoplasia as a possible malignant histiocytoma. Surgical treatment was carried out to remove the tumor with oncologic margins. Final biopsy after immunohystochemical staining revealed a malignant fibrous pleomorphic storiform histiocytoma. The patient was sent to oncologic treatment with radiotherapy and a local recurrence was diagnosed, that is why a four quarter amputation was achieved. Conclusions: malignant fibrous histiocytoma is the most frequent soft tissue sarcoma in adulthood. There are different histological varieties, that is why immunohystochemical staining is a key tool for accurate diagnosis. Surgery and radiotherapy are the best therapeutic combination. Age, tumor depth, histological variety, location, absence of metastasis and size are the most important prognosis factors.

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.

Benign fibrous histiocytoma of the tongue: a case report / Histiocitoma fibroso benigno em língua: relato de caso

The fibrous histiocytoma is a soft tissue neoplasm that affects the dermis and the subcutaneous tissue, rarely is found in the oral cavity and perioral regions, and is originated from the proliferation of fibroblasts and histiocytes. The objective of this paper is to report a case of Benign Fibrous Histiocytoma in a 30-year-old male patient, complaining of a painless nodule in the tongue for about six months. With diagnostic clinical hypotheses of Fibrous Hyperplasia, Neurofibroma, Traumatic Neuroma, Fibrous Histiocytoma, Granular Cell Tumor or Ectomesenchymal Chondromyxoid Tumor a excisional biopsy was performed. The histopathological examination revealed a non-encapsulated proliferation of spindle cells with some giant multinucleated cells in the periphery of the lesion. Immunohistochemical reactions were performed, staining only for vimentin in the spindle cells and for CD68 in the multinucleated giant cells. According to these characteristics, the final diagnosis was Benign Fibrous Histiocytoma. The correct diagnosis of spindle shaped cell neoplasia must be performed with the aid of histopathological analysis and immunohistochemistry, mainly because the morphological similarities with other benign and malignant lesions. (AU)

O Histiocitoma Fibroso é uma neoplasia de partes moles que acomete a derme e o tecido subcutâneo, raramente é encontrado na cavidade oral e regiões periorais, e tem origem a partir da proliferação de fibroblastos ou histiócitos. O objetivo deste artigo é relatar um caso de Histiocitoma Fibroso Benigno em um paciente masculino, 30 anos de idade, com um nódulo indolor, bem delimitado, com duração de cerca de seis meses, localizado no dorso anterior da língua. Com as hipóteses clínicas diagnósticas de Hiperplasia Fibrosa, Neurofibroma, Neuroma Traumático, Histiocitoma Fibroso, Tumor de Células Granulares e Tumor Condromixoide Ectomesenquimal uma biópsia foi realizada sob anestesia local e a lesão foi fixada em formol a 10% e enviada para análise histopatológica. O exame histopatológico revelou uma proliferação não-encapsulada de células fusiformes com algumas células gigantes multinucleadas na periferia da lesão. A marcação imunohistoquímica foi positiva para CD68 nas células gigantes multinucleadas e para vimentina nas células fusiformes. O diagnóstico final foi de Histiocitoma Fibroso Benigno. Para um diagnóstico correto, este deve ser feito correlacionando características clínicas, análise histopatológica e imunohistoquímica devido à similaridade microscópica do Histiocitoma Fibroso com outras lesões com aspecto fusocelular, assim como similaridade clínica com outras lesões benignas e malignas.(AU)

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.

Congenital multiple clustered dermatofibroma and multiple eruptive dermatofibromas - unusual presentations of a common entity / Dermatofibroma multiplo agrupado congenito e dermatofibromas multiplos eruptivos - apresentacoes singulares de uma entidade comum

Dermatofibroma is one of the most common entities seen in dermatology clinical practice. Several clinical subtypes have nevertheless been described, all of them of uncommon occurrence. The authors present two rare clinical variants of dermatofibromas: congenital multiple clustered dermatofibroma (the presented case is the 4th congenital case to be reported so far) and multiple eruptive dermatofibromas developing in the setting of a Sjögren's syndrome. Since the uncommon subtypes may not be clinically evident, dermatologists should familiarize themselves with their main features and we advise a high level of clinical suspicion in order to reach the correct diagnosis.

O dermatofibroma é uma das entidades mais frequentemente observadas na prática clínica dermatológica. No entanto, além do dermatofibroma comum, vários subtipos clínicos de ocorrência incomum têm sido descritos na literatura. Os autores descrevem duas variantes clínicas raras de dermatofibromas: dermatofibroma múltiplo agrupado congênito (o caso apresentado é o quarto caso congênito reportado até hoje) e dermatofibromas eruptivos múltiplos no contexto de uma Síndrome de Sjögren. Estes diagnósticos menos comuns podem não ser clinicamente evidentes portanto os dermatologistas devem estar familiarizados com estas apresentações, sendo de suma importância um elevado índice de suspeita clínica.

Atrophic dermatofibroma / Dermatofibroma atrofico

Dermatofibroma is a benign fibrohistiocytic tumor, common and easily diagnosed when classical clinicopathologic features are present. The atrophic variant of dermatofibroma is of uncertain origin. This lesion is characterized clinically by a flat or atrophic and depressible surface. Histopathological features show reduction of the thickness of the dermis and elastic fibers. We report a typical case of this uncommon and probably underdiagnosed variant.

O dermatofibroma é um tumor fibrohistiocitário benigno, comum e facilmente diagnosticado quando apresenta os achados clinicopatológicos clássicos. O dermatofibroma atrófico é uma variante específica do dermatofibroma, de origem ainda incerta. Esta é caracterizada clinicamente por lesão plana ou atrófica, depressível à compressão. Ao exame histopatológico, observa-se redução da espessura da derme e redução das fibras elásticas. Relatamos um caso típico desta variante incomum e provavelmente subdiagnosticada.

Dermatofibroma in a black tattoo: report of a case / Dermatofibroma sob pigmento preto de tatuagem: relato de um caso

Tattooing has been associated with a variety of complications including inflammatory and granulomatous reactions, transmission of infections, and neoplasms. We report a case of a 24-year-old male who presented with a 2-month history of an erythematous nodule involving a newly made tattoo on the right leg. An excisional biopsy was performed and the histopathological evaluation was consistent with dermatofibroma. Only three cases of dermatofibroma associated with tatooing were reported in litetature. We report an additional case and review the literature regarding cutaneous reactions to tattoos.

Tatuagens têm sido associadas com uma variedade de complicações incluindo reações inflamatórias e granulomatosas, transmissão de infecções e neoplasias. Relatamos um caso de homem com 24 anos de idade que apresentava há dois meses nódulo eritematoso sob pigmento preto de uma tatuagem na coxa direita. A biópsia excisional foi realizada e a avaliação histológica foi consistente com dermatofibroma. Apenas três casos da associação dermatofibroma e tatuagem foram relatados na literatura. Nós reportamos um caso adicional e revisamos a literatura sobre reações cutâneas em tatuagens.

Caracterización de pacientes con sarcomas de partes blandas. Estudio de 12 años / Characterization of patients with soft tissue sarcomas. A study of 12 years

Se realizó un estudio longitudinal, descriptivo y transversal de 89 pacientes con sarcomas de partes blandas, atendidos en el Hospital Oncológico Provincial Docente "Conrado Benítez García" de Santiago de Cuba, desde enero del 2000 hasta diciembre del 2011, luego de ser remitidos fundamentalmente de otros centros asistenciales (donde fueron diagnosticados y operados), para ser valorados y tratados por un equipo multidisciplinario, con vistas a caracterizarles y así aplicar diferentes modalidades diagnósticas y terapéuticas en ellos. En la casuística se obtuvo una mayor frecuencia de la enfermedad en edades menores, así como del fibrosarcoma y el histiocitoma fibroso como variedades hísticas, y de la localización en los miembros inferiores. Se destacó que el diagnóstico temprano es determinante, al igual que el tratamiento de elección consistente en la resección no mutilante, combinada con el tratamiento adyuvante o no. La mayoría de los pacientes fueron reintervenidos y se diagnosticaron en etapa II de la afección, lo que empeoró el pronóstico y además indicó la existencia de procedimientos incompletos en el proceso terapéutico, que conspiran a dañar la sobrevida de los pacientes.

A longitudinal, descriptive and cross-sectional study was conducted in 89 patients with soft tissue sarcomas, attended in "Conrado Benítez García" Provincial Teaching Oncology Hospital of Santiago de Cuba, from January 2000 to December 2011, after being referred primarily from other care centers (where they were diagnosed with and operated), to be assessed and treated by a multidisciplinary team, with the purpose of characterizing them and thus applying different diagnostic and therapeutic modalities in them. A higher frequency of the disease at younger ages, of fibrosarcoma and fibrous histiocytoma as tissular varieties, and location in the lower limbs was found in the case material. It was stressed that early diagnosis is crucial, as well as the treatment of choice consisting in non-mutilating resection combined with adjuvant treatment or not. Most patients were reoperated and diagnosed in stage II of the disease, worsening the prognosis and indicating the existence of incomplete procedures in the therapeutic process that conspire to harm the survival of patients.

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...

Histiocitoma fibroso maligno / Malignant fibrous histiocytoma

Introducción: los sarcomas son tumores malignos de partes blandas que representan 0,7 porciento de los tumores malignos en general, con una tasa de 3 por cada 100 000. El histiocitoma fibroso maligno resulta un tumor de alto grado incluido en el grupo de los sarcomas de partes blandas. Objetivo: exponer las características citohistológicas, el tratamiento y la evolución de este tumor en un caso estudiado y tratado en el Hospital General Provincial Docente Roberto Rodríguez Fernández de Ciego de Ávila. Descripción: se presentó el caso de un varón de 60 años con una gran masa de partes blandas en el muslo izquierdo, de crecimiento progresivo. La masa se trató quirúrgicamente con exéresis en bloque, previos estudios analíticos, arteriografía femoral y ecografía. Conclusiones: el diagnóstico anatomopatológico fue de histiocitoma fibroso maligno variedad pleomórfica; fue remitido al servicio de oncología donde lo trataron con radioterapia. A los 6 meses se encontraba libre de la enfermedad. Se realizó una revisión de la literatura y se analizó su presentación clínica, también los hallazgos histológicos, las pruebas diagnósticas de imagen y el procedimiento terapéutico

Introduction: the sarcomas are soft tissue malignant tumors accounting for the 0.7 percent of malignant tumors in general with a rate of 3 by 100 000. The malignant fibrous histiocytoma is a high grade tumor included in the group of soft tissue tumors. Objective: to expose the cytohistologic features, treatment and course of this tumor in study case and treated in Roberto Rodríguez Fernández Teaching Provincial Hospital of Ciego de Avila municipality. Description: this is the case of a male patient aged 60 presenting with an increasing large soft tissue mass in the left thigh. The mass was operated on with block exeresis, analytical previous studies, femoral arteriography and echography. Conclusions: the anatomic and pathologic diagnosis was a malignant fibrous histiocytoma pleomorphous type; the patient was referred to Oncology service where was treated with radiotherapy. At 6 months he was free of disease. A review of literature was performed analyzing its clinical presentation also, its histological findings, the imaging diagnostic test and the therapeutical procedure