Pleomorphic sarcoma of maxillary sinus: Clinical report of a patient initially diagnosed with denture-induced fibrous hyperplasia.

Undifferentiated pleomorphic sarcoma is a high-grade soft-tissue malignant tumor that is rare in the head and neck region. A 74-year-old woman displayed a large nodular lesion in the maxillary alveolar mucosa, which was initially identified as denture-related fibrous hyperplasia. Although her prosthodontist has adjusted the maxillary complete denture to relieve pressure on the lesion, it increased in size over time. Computed tomography images of the maxillary sinus showed an extensive destructive lesion. A biopsy was performed, and microscopic examination revealed a poorly differentiated malignancy with numerous spindle cells. Immunohistochemistry reactions were negative for CD45, CD30, CD68, CD34, cytokeratin, S100, desmin, and smooth muscle actin. These findings led to the diagnosis of an undifferentiated pleomorphic sarcoma of the maxillary sinus.

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.

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.

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.

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)

A Rare Case of Malignant Fibrous Histiocytoma (Undifferentiated High-Grade Pleomorphic Sarcoma) of Malar Region.

Malignant fibrous histiocytoma is a sarcoma with rare occurrence in the oral and maxillofacial region; surgery is the most reliable treatment. Inadequate resection of the sarcoma on the oral and maxillofacial region is associated with a high incidence of local recurrence and a poor prognosis. Only few patients of malignant fibrous histiocytoma of the malar region have been previously reported in the literature. The authors report a new patient of malignant fibrous histiocytoma on the right malar region that treated a complete tumor surgical excision without lymph node dissection. Examination of the resected specimen revealed that the tumor was completely removed.

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.

Histiocitoma fibroso maligno em um cão relato de caso / Malignant fibrous histiocytom a in a dog: a case report

Um canino macho, de aproximadamente cinco anos de idade, da raça Pinscher, foi diagnosticado comum histiocitoma fibroso maligno nos cornetos nasais. A neoplasia é do tipo mesenquimal maligno, que sedesenvolve nos tecidos moles e abrange a derme e o tecido subcutâneo. O caso foi analisado com auxíliode exames radiográficos da cabeça, além do citopatológico, para maior precisão no prognóstico clínico.Diante da situação foi indicada a remoção cirúrgica do histiocitoma, e sendo esta uma das formas maisraras de neoplasias, se torna relevante a sua apresentação aos clínicos médicos veterinários.(AU)

A male dog, about five years old, Pinscher, was diagnosed with a malignant fibrous histiocytomaof the nasal turbinates. The tumor is malignant mesenchymal type, which develops in the soft tissueand covers the dermis and subcutaneous tissue. The casewas analyzed with the aid of X-rayexaminations of the head, in addition to cytology,for greater accuracy in clinical prognosis. Giventhe situation was nominated forsurgical removal of histiocytoma, and this being one of the rarestforms of cancer, it becomes relevant to their clinical presentation to veterinarians.(AU)

Histiocitoma fibroso maligno em um cão – relato de caso / Malignant fibrous histiocytom a in a dog: a case report

Um canino macho, de aproximadamente cinco anos de idade, da raça Pinscher, foi diagnosticado comum histiocitoma fibroso maligno nos cornetos nasais. A neoplasia é do tipo mesenquimal maligno, que sedesenvolve nos tecidos moles e abrange a derme e o tecido subcutâneo. O caso foi analisado com auxíliode exames radiográficos da cabeça, além do citopatológico, para maior precisão no prognóstico clínico.Diante da situação foi indicada a remoção cirúrgica do histiocitoma, e sendo esta uma das formas maisraras de neoplasias, se torna relevante a sua apresentação aos clínicos médicos veterinários.

A male dog, about five years old, Pinscher, was diagnosed with a malignant fibrous histiocytomaof the nasal turbinates. The tumor is malignant mesenchymal type, which develops in the soft tissueand covers the dermis and subcutaneous tissue. The casewas analyzed with the aid of X-rayexaminations of the head, in addition to cytology,for greater accuracy in clinical prognosis. Giventhe situation was nominated forsurgical removal of histiocytoma, and this being one of the rarestforms of cancer, it becomes relevant to their clinical presentation to veterinarians.

Pleomorphic malignant histiocytoma of pulmonary arteries presenting as pulmonary aneurysms.

Pulmonary aneurysms and primary neoplasms of the great vessels are very rare entities; pulmonary aneurysms are commonly associated with congenital heart diseases, and less frequently in atherosclerosis, medial cystic necrosis, trauma, infection, and inflammatory processes. Many patients have pulmonary hypertension, most frequently resulting from pulmonary artery sarcomas mimicking pulmonary thromboembolism. Symptoms are vague. In 30% of cases, rupture and death occur, related to pulmonary aneurysms. We present the case of a patient with a diagnosis of pulmonary artery pleomorphic malignant histiocytoma that presented as a right pulmonary aneurysm thrombosis and a contained rupture of a left pulmonary aneurysm.

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...

Intracardiac malignant fibrous histiocytoma. A case report.

BACKGROUND: Primary cardiac tumors are rare forms of cardiac disease. The reported incidence varies between 0.002 and 0.3%; 25% of these tumors are malignant, usually a variant of sarcoma. Malignant fibrous histiocytoma constitutes <3% of primary cardiac tumors. CLINICAL CASE: We review the case of a 53-year-old female who presented with congestive heart failure and pleural effusion. Transthoracic echocardiography and chest computed tomography revealed cardiac tumor involving the left atrium. Tumor was excised surgically and histologically proven to be a malignant fibrous histiocytoma, primarily confined to the heart. During the immediate postoperative evolution, the patient presented left pleural effusion for 2 weeks. The patient was referred to Oncology Service for complementary treatment. CONCLUSION: Malignant fibrous histiocytoma is a rare tumor and, in this case, prognosis is reserved.

A low-grade malignant fibrous histiocytoma presenting as a left maxillary swelling

A 31-year-old male presented with a three month history of a swelling in the left cheek which was gradually increasing in size. The patient also complained of a left nasal obstruction but no discharge or epistaxis. There was no history of smoking or alcohol consumption nor any co-morbid medical conditions. On examination of his left cheek there was a 3 x 2 cm firm, non-tender, non-fluctuant, non-pulsatile mass which seemed to be originating from the left maxillary sinus. Left nasal examination revealed a polypoidal mass arising from the middle meatus. There was also a convex distortion of the left anterior aspect of the hard palate (no erosion of epithelium of palate) and mild proptosis of the left eye.

The usefulness of latissimus dorsi transfer for reconstruction for malignant fibrous histiocytoma in the infraspinatus muscle region: a case report.

Malignant fibrous histiocytoma (MFH) is a type of highly malignant soft tissue sarcoma with a predilection for the extremities of adults. We report a patient with MFH in the infraspinatus muscle for which wide resection including total resection of the infraspinatus muscle was performed, followed by transfer of the latissimus dorsi muscle for shoulder reconstruction in a one-stage operation with good postoperative function.

The usefulness of latissimus dorsi transfer for reconstruction for malignant fibrous histiocytoma in the infraspinatus muscle region: a case report / Utilidad de la transferencia del latissimus dorsi en la reconstrucción por histiocitoma fibroso maligno en la región muscular infraespinosa: reporte de un caso

Malignant fibrous histiocytoma (MFH) is a type of highly malignant soft tissue sarcoma with a predilection for the extremities of adults. We report a patient with MFH in the infraspinatus muscle for which wide resection including total resection of the infraspinatus muscle was performed, followed by transfer of the latissimus dorsi muscle for shoulder reconstruction in a onestage operation with good postoperative function.

El histiocitoma fibroso maligno (HFM) es un tipo de sarcoma del tejido suave, altamente maligno, con predilección por las extremidades de los adultos. Reportamos el caso de un paciente con HFM en el músculo infraespinoso, al cual se le hizo una amplia resección que incluyó la resección total del músculo infraespinoso, seguida de una transferencia del músculo latissimus dorsi para la reconstrucción del hombro, en una operación de una sola etapa con buena función postoperatoria.

Histiocitoma fibroso maligno primario del músculo temporal: presentación de un caso / Primary malignant fibrous histiocytoma temporalis muscle: case report

El Histiocitoma fibroso maligno (HFM) fue descrito por Obrien y Stout en 1964 representando al 1-3 por ciento de los sarcomas del tejido blando de cabeza y cuello. Su histogénesis es incierta y el comportamiento biológico es propenso a la recidiva local y a las metástasis a distancia, preferentemente a ganglios linfáticos regionales, pulmón, hígado y huesos. Se presenta una paciente de 38 años de edad, con una lesión tumoral epicraneal recidivante, de consistencia blanda, fluctuante, no dolorosa, de aproximadamente 5 por 3 cm en región temporal derecha, se le realiza Tomografía computarizada de cráneo e Imagen por resonancia magnética, es intervenida quirúrgicamente resecándose totalmente una tumoración predominantemente quística en intima relación con el músculo temporal y reintervenida quince días después, el Rx de tórax, el ultrasonido abdominal y el survey óseo fueron negativos, el estudio histopatológico arrojó un Histiocitoma fibroso maligno pleomorfico por lo que es enviada al servicio de oncología para tratamiento adyuvante (radioterapia). Actualmente pasado un año se encuentra libre de enfermedad. Concluimos que el HFM primario del músculo temporal es una rara localización entre los sarcomas de partes blandas y aun más entre los de cabeza y cuello, donde el aumento de volumen no doloroso fue la forma clínica de presentación, la tomografía computarizada y principalmente las imágenes por resonancia magnética mostraron la relación músculo tumor, lográndose con el empleo de la cirugía y la radioterapia adyuvante alcanzar buenos resultados.

Aspectos clinicopatológicos e imunoistoquímicis do histocitoma fibroso maligno estoriforme-pleomórfico em um cão / Clinicopathological and immunohistochemical features of storiform-pleomorphic malignant fibrous histiocytoma in a dog

Histiocitoma fibroso maligno (MFH) é um neoplasma pleocelular de origem incerta e prognóstico sombrio. O correto diagnóstico através de exames imunoistoquímicos é difícil e, na maioria das vezes, inconsistente. Descreve-se os aspectos clinicopatológicos e imunoistoquímicos de um caso de MFH em um cão Rottweiler, macho, de seis anos. O neoplasma foi testado para tricômico de Masson, citoqueratina, vimentina, lisozima, proteína S100 GFAP e NSE. A positividade para vimentina, lisozima e proteína S100 nas células redondas e multinucleadas são características dos histiócitos. Resultados negativos foram observados para os demais anticorpos. Com base nos resultados, diagnósticos diferenciais foram discutidos e a origem histiocítica ou de células pluripotenciais ds células tumoriais pôde ser sugerida. O MFH ainda permanece um desafio diagnóstico em animais e humanos

Malignant fibrous histiocytoma (MFH) is a pleocellular neoplasm of uncertain cellular origin and poor prognosis. The right diagnosis through immunohistochemical test is difficult and most of the time inconsistent. Clinicopathological features of a MFH in a six-year-old male Rottweiler were described. Additional immunohistochemical data were also provided for diagnosis. The tumor was tested for Masson trichrome, cytokeratin, vimentin, lysozyme, S100 protein, GFAP and NSE. Positive results for vimentin, lyzozyme and S100 protein observed in round and multinucleated cells are characteristic of histiocytes. The result allowed the distinction between the differential diagnosis and suggested an histicytic or pluripotential cell origin. The MFH still remains a diagnostic challenge in dogs and humans