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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.

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.

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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.

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Abstract Congenital and self-healing Hashimoto-Pritzker reticulohistiocytosis is the benign variant of the Langerhans cell histiocytosis (LCH) group. It is characterized by multiple skin lesions (congenital or appearing during the first days after birth), without systemic manifestations and spontaneous resolution in days to months. The authors report the case of a boy with a single congenital leg skin lesion, a rare disease variant. Through histopathology, a dense skin infiltration of S100 protein-, CD1a-, CD207-immunomarked cells was found. KI67 index was high (62%). A complete spontaneous resolution occurred 07 days after the biopsy (25 days after birth). Monolesional disease, distal limb lesion, absence of lesions in the mucous membrane or seborrheic area, and less than 25 percent of LCs with Birbeck granules were said to be possible clues for a favorable prognosis in LCs histiocytosis. But, as a precautionary measure, the child will be followed up until at least 2 years of age.

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Congenital and self-healing Hashimoto-Pritzker reticulohistiocytosis is the benign variant of the Langerhans cell histiocytosis (LCH) group. It is characterized by multiple skin lesions (congenital or appearing during the first days after birth), without systemic manifestations and spontaneous resolution in days to months. The authors report the case of a boy with a single congenital leg skin lesion, a rare disease variant. Through histopathology, a dense skin infiltration of S100 protein-, CD1a-, CD207-immunomarked cells was found. KI67 index was high (62%). A complete spontaneous resolution occurred 07 days after the biopsy (25 days after birth). Monolesional disease, distal limb lesion, absence of lesions in the mucous membrane or seborrheic area, and less than 25 percent of LCs with Birbeck granules were said to be possible clues for a favorable prognosis in LCs histiocytosis. But, as a precautionary measure, the child will be followed up until at least 2 years of age.

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Benign fibrous histiocytoma (BFH) is a common mesenchymal lesion on the skin where it is better known as dermatofibroma. It mainly affects areas of the integument exposed to the sun. It can occur in the mucous membranes, being quite unusual in the oral cavity. This article presents a rare case of benign oral fibrous histiocytoma in childhood. An eleven-year-old male patient sought the stomatology clinic of the dentistry course at a reference university in northeastern Brazil, complaining of an asymptomatic increase in volume on the cheek, with an evolution of approximately two years. On intraoral examination, a lesion in the cheek mucosa was observed, measuring approximately 4 cm. The lesion had a nodular, circumscribed, reddish appearance, showing ulcerated areas covered by a yellowish pseudomembrane on its surface. The clinical diagnostic hypothesis was pleomorphic adenoma. Excisional biopsy was performed and, after morphological and immunohistochemical evaluation, the diagnosis of BFH was reached. Although it is a lesion with an excellent prognosis, BFH must be carefully analyzed for the differential diagnosis with malignant neoplasms that may have a mild appearance, such as fibrosarcoma or dermatofibrosarcoma. In addition, other benign lesions such as desmoplastic fibroblastoma, myofibroma, myopericytoma and nodular fasciitis may, in some histological sections, mimic the microscopic appearance of benign fibrous histiocytoma. In this reported case, immunohistochemical evaluation was essential to conclude the diagnosis of BFH(AU)

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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.

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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

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Abstract Dermatofibroma is a dermal fibrohistiocytic neoplasm. The Langerhans cells are the immunocompetent cells of the epidermis, and they represent the first defense barrier of the immune system towards the environment. The objective was to immunohistologically compare the densities of S100-positive Langerhans cells in the healthy peritumoral epidermis against those in the epidermis overlying dermatofibroma (20 cases), using antibodies against the S100 molecule (the immunophenotypic hallmark of Langerhans cells). The control group (normal, healthy skin) included ten healthy age and sex-matched individuals who underwent skin biopsies for benign skin lesions. A significantly high density of Langerhans cells was observed both in the epidermis of the healthy skin (6.00 ± 0.29) and the peritumoral epidermis (6.44 ± 0.41) vs. those in the epidermis overlying the tumor (1.44 ± 0.33, p < 0.05). The quantitative deficit of Langerhans cells in the epidermis overlying dermatofibroma may be a possible factor in its development.

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Undifferentiated pleomorphic sarcoma (UPS) is a high-grade neoplasm that is usually located in the extremities and retroperitoneum. In the past, UPS was considered the most common soft tissue sarcoma in adults; due to improvements in diagnostic techniques, most cases have been reclassified as other lineage-specific tumors. Gnathic bones are rarely affected, and the clinicopathological characteristics of this neoplasm when diagnosed in the jaw remain to be better described. In this report, we present a rare case of mandibular UPS affecting an 88-year-old female who demonstrated a painful swelling on the right side of the mandible that was accompanied by a pathological fracture. Microscopic examination revealed a pleomorphic spindlecell neoplasm with mitotic figures and necrosis. The patient underwent surgery and adjuvant radiotherapy but experienced metastasis after 12 months of follow-up and died. Diagnosis of UPS is challenging, and oral pathologists must be aware of this entity when dealing with aggressive undifferentiated neoplasms.

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Abstract Multinucleate cell angiohistiocytoma is a rare, benign vascular proliferation of unknown etiology. It occurs mainly in middle-aged women and usually affects the acral regions; the lesions appear as discrete, grouped, and asymptomatic violaceous papules. Histopathology shows proliferation and dilated small vessels in the papillary dermis, fibrous stroma with thickened collagen bundles, and multinucleated giant cells. To date, there are approximately 140 cases described in the indexed literature. This report presents the case of a 62-year-old woman with a typical clinical condition, who chose not undergo treatment, considering the benign character of her illness. The clinical and immunohistological aspects of this unusual dermatological entity are emphasized.

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Dermatofibroma is a dermal fibrohistiocytic neoplasm. The Langerhans cells are the immunocompetent cells of the epidermis, and they represent the first defense barrier of the immune system towards the environment. The objective was to immunohistologically compare the densities of S100-positive Langerhans cells in the healthy peritumoral epidermis against those in the epidermis overlying dermatofibroma (20 cases), using antibodies against the S100 molecule (the immunophenotypic hallmark of Langerhans cells). The control group (normal, healthy skin) included ten healthy age and sex-matched individuals who underwent skin biopsies for benign skin lesions. A significantly high density of Langerhans cells was observed both in the epidermis of the healthy skin (6.00 ±â€¯0.29) and the peritumoral epidermis (6.44 ±â€¯0.41) vs. those in the epidermis overlying the tumor (1.44 ±â€¯0.33, p < 0.05). The quantitative deficit of Langerhans cells in the epidermis overlying dermatofibroma may be a possible factor in its development.

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O histiocitoma cutâneo canino é um tumor benigno de células redondas, sendo uma das neoplasias cutâneas mais comuns visto em cães jovens. Este trabalho, tem o objetivo de relatar um caso de um canino jovem diagnosticado com histiocitoma. Após um exame clínico inicial o animal foi encaminhado para o exame de citologia aspirativa por agulha fina, o qual evidenciou alterações características de um histiocitoma. A causa etiológica é de origem desconhecida. Não foi realizado procedimento cirúrgico para exérese do tumor, e sim, instituído tratamento conservador, havendo remissão total do tumor após duas semanas.

Canine cutaneus histiocytoma is a benign round cell tumor, being one of the most common cutaneous neoplasms seen in young dogs. This paper aims to report a case of a canine diagnosed with histiocytoma. After an initial clinical examination the animal was referred to the fine needle aspiration cytology, which showed characteristics changes of a histiocytoma. The etiological cause is of unknown origin. No surgical procedure was performed for tumor excision, but conservative treatment was institued with total tumor remission after two weeks.

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O histiocitoma cutâneo canino é um tumor benigno de células redondas, sendo uma das neoplasias cutâneas mais comuns visto em cães jovens. Este trabalho, tem o objetivo de relatar um caso de um canino jovem diagnosticado com histiocitoma. Após um exame clínico inicial o animal foi encaminhado para o exame de citologia aspirativa por agulha fina, o qual evidenciou alterações características de um histiocitoma. A causa etiológica é de origem desconhecida. Não foi realizado procedimento cirúrgico para exérese do tumor, e sim, instituído tratamento conservador, havendo remissão total do tumor após duas semanas.(AU)

Canine cutaneus histiocytoma is a benign round cell tumor, being one of the most common cutaneous neoplasms seen in young dogs. This paper aims to report a case of a canine diagnosed with histiocytoma. After an initial clinical examination the animal was referred to the fine needle aspiration cytology, which showed characteristics changes of a histiocytoma. The etiological cause is of unknown origin. No surgical procedure was performed for tumor excision, but conservative treatment was institued with total tumor remission after two weeks.(AU)

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Multinucleate cell angiohistiocytoma is a rare, benign vascular proliferation of unknown etiology. It occurs mainly in middle-aged women and usually affects the acral regions; the lesions appear as discrete, grouped, and asymptomatic violaceous papules. Histopathology shows proliferation and dilated small vessels in the papillary dermis, fibrous stroma with thickened collagen bundles, and multinucleated giant cells. To date, there are approximately 140 cases described in the indexed literature. This report presents the case of a 62-year-old woman with a typical clinical condition, who chose not undergo treatment, considering the benign character of her illness. The clinical and immunohistological aspects of this unusual dermatological entity are emphasized.

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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

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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.

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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.

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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.

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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.

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Abstract: Dermatofibroma is a proliferation of spindle cells located in the dermis. We used scanning electron microscopy to examine two histologically confirmed lesions and observed preserved collagen bundles in the perilesional area. In the lesional area, the collagen was denser, without formation of bundles. Higher magnification showed collagen with mesh-like appearance similar to stretched tufts of cotton. Very high magnification evidenced the tufts of cotton and spindle cells measuring 2 to 12 microns.

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