A case of pseudomyogenic hemangioendothelioma misdiagnosed as low-grade malignant fibrous histiocytoma and review of literature / 中南大学学报(医学版)

Pseudomyogenic hemangioendothelioma (PHE) is a rare angiogenic tumor. Histologically, the morphological characteristics of neoplastic vessels and endothelial differentiation are not obvious, and it is easy to be confused with epithelioid sarcoma, epithelioid hemangioendothelioma and myogenic tumor. PHE usually occurs in arms and legs in young people and has a significant male predominance. The tumor has a predilection for the distal extremities and its typical manifestation is multiple center invasion of a single limb, which can involve all layers of skin and subcutaneous tissues,and is often accompanied by abvious pain. Histologically, PHE is characterized by infiltrative growth of tumor. Most tumor lesions are composed of sheets and loose fascicles of plump spindle or epithelioid cells within a background of variably prominent inflammatory infiltration, which was commonly composed of neutrophils. Some cells may resemble rhabdomyoblasts, and nuclear atypia and mitosis were rare. The tumor cells generally expressed positive cytokeratin (CK), ETS-related gene (ERG), Friend leukemia virus integration 1 (FLI1) and integrase interactor 1(INI1). In some cases, the tumor cells expressed CD31. A case of a young woman was reported in this paper, who presented with a subcutaneous mass with severe pain and was chronologically misdiagnosed with herpes zoster, low-grade malignant fibrous histiocytoma and epithelioid hemangioendothelioma. In this study, the clinical and pathological features, differential diagnosis and the latest progress in therapy of PHE were analyzed based on relevant literature.

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 / 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)

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.

Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration for the Diagnosis of Central Lung Parenchymal Lesions

PURPOSE: The purpose of this study was to evaluate the usefulness of convex probe endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) for detecting malignancy in parenchymal pulmonary lesions located adjacent to the central airways. MATERIALS AND METHODS: We retrospectively reviewed the diagnostic performance of EBUS-TBNA in consecutive patients with high clinical suspicion of a centrally located primary lung cancer who had undergone EBUS-TBNA at the Samsung Medical Center between May 2009 and June 2011. RESULTS: Thirty-seven patients underwent EBUS-TBNA for intrapulmonary lesions adjacent to the central airways. Seven lesions were located adjacent to the trachea and 30 lesions were located adjacent to the bronchi. Cytologic and histologic samples obtained via EBUS-TBNA were diagnostic in 32 of 37 (86.4%) of patients. The final diagnosis was lung cancer in 30 patients (7 small cell lung cancer, 23 non-small cell lung cancer), lymphoma in one and malignant fibrous histiocytoma in one patient. The diagnostic sensitivity of EBUS-TBNA in detecting malignancy and detecting both malignancy and benignity was 91.4% and 86.5%, respectively. Two patients experienced minor complications. CONCLUSION: EBUS-TBNA is an effective and safe method for tissue diagnosis of parenchymal lesions that lie centrally close to the airways. EBUS-TBNA should be considered the procedure of choice for patients with centrally located lesions without endobronchial involvement.

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...

Benign vs malignant soft tissue neoplasms: Limitations of magnetic resonance imaging.

Aims: Various features have been described in the literature to differentiate benign from malignant lesions. The aim of the present study was to study the accuracy of each of these features and that of magnetic resonance imaging (MRI) in diagnosing malignant lesions. Materials and Methods: Fifty-five consecutive patients presenting with neoplastic (both benign and malignant) lesions diagnosed clinically and on ultrasound were studied and their MRI features were compared with the findings on surgical exploration and histopathologic examination. Results: There were 32 (58%) benign and 23 (42%) malignant masses. Malignant masses were more common in patients older than 20 years (83%), and these had symptoms of less than 6 months duration (75%), as against benign lesions. The swelling was painful in 8 malignant masses and these were more common in the upper limbs (61%). Various features of malignant lesions were size more than 5 cm in 83%, change in signal intensity from homogenous on T1-weighted images to heterogenous on T2-weighted images in 74%, irregular margins in 74%, and heterogenous contrast enhancement in 91%. The accuracy of these features was 76%, 58%, 78%, and 60%, respectively. Most benign and malignant lesions were intramuscular in location. A significant number (38%) of benign lesions were located in the intermuscular facial plane. Definitive diagnosis was made in 42% of the lesions. Conclusions: MRI is an excellent modality for evaluating soft tissue neoplasms; however, prediction of a specific diagnosis and differentiation of malignant and benign lesions is not always possible.

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.

Liver metastasis of malignant fibrous histiocytoma: a case report

Malignant fibrous histiocytoma [MFH] is the most common soft-tissue sarcoma occurring in adult life. It is relatively uncommon in the head and neck area. Surgery is the most reliable treatment for MFH, but the 5-year survival rate for cases of this tumour in the head and neck region is low in comparison with MFH of the extremities and trunk. Most metastases occurred in the lungs [90%], followed by bone [8%]. Liver metastases of MFH are very rare [1%]. We report on a case of a 62-year-old woman undergoing repeat surgery for an MFH of the neck, with liver metastasis found 14 months after the first surgery. She underwent liver resection and the postoperative course was uneventful

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.

Two different types of malignant fibrous histiocytomas from pet dogs

We describe 2 cases of malignant fibrous histiocytomas (MFHs) that spontaneously developed in young pet dogs. To classify these tumors, we applied a panel of antibodies (vimentin, desmin, alpha-SMA, and ED1) and Azan staining for collagen. The MFHs were most consistent with osteoclast-like giant and inflammatory cell types. The first case had positive staining for ED1 and vimentin, and given the osteoclast-like giant cells, calcification sites accompanying peripheral giant cell infiltrates. The latter case, the inflammatory cell type, exhibited a storiform-pleomorphic variant of neoplastic cells, including an ossifying matrix. MFHs are among the most highly aggressive tumors occurring in soft tissue sarcomas in elderly dogs; however, MFHs have been poorly studied from a diagnostic point of view. Herein, we describe the histologic and immunohistologic features of MFHs in detail, thus classifying the subtypes of these tumors.

Fibrohistiocitoma maligno (fibroxantosarcoma) de cuero cabelludo: a propósito de un caso / Histiocytoma malignant fibrous (Fibroxantosarcoma) of scalp: a purpose of a case

Los tejidos blandos comprenden los músculos, tendones, la grasa, el tejido fibroso, el tejido sinovial, los vasos y los nervios. Alrededor del 60 por ciento de los sarcomas de tejidos blandos se originan en los miembros, siendo 3 veces más frecuentes en los miembros inferiores que en los superiores. En 30 por ciento de los casos se localiza en el tronco y en 40 por ciento son retroperitoneales. En el resto del 10 por ciento se trata de tumores de cabeza y cuello. La entidad denominada fibrohistiocitoma comprenden un gran número de tumores calcificados anteriormente como fibrosarcomas o como variedades polimorfas de otras sarcomas, y se caracteriza por una mezcla de células fusiformes (fibrosas) y células redondas (histiocitarias) dispuestas en un patrón estoriforme, junto con abundantes células gigantes y zonas de polimorfismo. La importancia de los estudios de inmunohistoquímica en los tumores de partes blandas radica en la necesidad que tiene el patólogo de precisar el diagnóstico histopatológico de lesiones benignas y malignas pseudosarcomatosas o sarcomatosas y poder diferenciarlas de neoplasias de otro origen. Se trata de paciente masculino de 65 años quien consulta por presentar desde agosto del 2006, caracterizado por lesiones ulcerosa en cuero cabelludo sangrantes quién se le tomo una primera biopsia que reporta. La nueva Biopsia tomada en enero en este centro reporta Carcinoma Epidermoide moderadamente a poco diferenciado en región fronto parietal de cuero cabelludo recibe 1 ciclo de quimioterapia, se toma una biopsia nueva reportando su inmunohistoquímica Fibrohistiocitoma Maligno (fibroxantosarcoma)/Tumor de Cuero Cabelludo, en vista de resultado se planifica nueva quimioterapia, se observo mejoría satisfactoria en la lesión en cuero cabelludo, por lo que decide oncología y el servicio de Medicina Interna dar de alta y seguir sus quimioterapias por consulta externa.

Plexiform fibrohistiocytic tumor.

Plexiform fibrohistiocytic tumor is an uncommon mesenchymal tumor that can cause difficulty in diagnosis and surgical management. On clinical and histologic examination, these tumors can potentially be misdiagnosed as sebaceous/epidermal cysts. We report a case of plexiform fibrohistiocytic tumor in a young female, which on initial clinical evaluation was diagnosed as sebaceous cyst. The tumor was deeply invasive, with positive margins on two excisional biopsy specimens. The importance of recognition of this entity, potential pitfalls and prognosis are discussed and the literature is reviewed.

Fibrohistiocitoma maligno: localización cefálica como presentación poco frecuente / Malignant Fibrohistiocitoma: cephalic location as presentation little frequents

Los sarcomas de tejidos blandos son tumores malignos derivados del mesodermo.El fibrohistiocitoma maligno(FHM) es el más común de los sarcomas de tejidos blandos en adultos(20-25%),aunque no es común como tumor primario localizado en piel. La edad de presentación es entre los 50 y 70 años; dos tercios de ellos ocurren en hombres, siendo las personas de raza blanca las más afectadas. Comprometen por lo general raíz de miembros y tronco. La extremidad cefálica fue la localización de los pacientes de nuestra serie, sitio infrecuente según la literatura. El objetivo de este trabajo es presentar tres pacientes varones, dos con lesiones en cuero cabelludo y otro en pabellón auricular. Planteamos el concepto de forodaño como posible factor predisponente.

Malignant primary fibrous histiocytoma of the right ventricle

We report a rare case of primary cardiac malignant fibroushistiocytoma. A 65-year-oldmanpresentedwithepigastric tenderness. Echocardiography detected large pericardial effusion and right ventricular mass 5 x 4 cm. Tumor biopsy revealed malignant fibroushistiocytoma. Thorough investigation did not detect any extra-cardiac primary source or metastasis. Tumor resection was offered to the patient but he refused. Despite refusal the patient was still alive one year after diagnosis

Disseminated malignant fibrous histiocytoma (giant cell rich): a case report.

Giant cell rich malignant fibrous histiocytoma accounts for 3 -15% of all malignant fibrous histiocytomas. Currently, the nomenclature giant cell malignant fibrous histiocytoma is reserved for undifferentiated pleomorphic sarcomas with prominent osteoclastic giant cells. It is considered to be synonymous with malignant giant cell tumor of soft parts. We report a case of disseminated giant cell malignant fibrous histiocytoma involving the scalp, cervical node, lungs, spine, abdominal wall, base of penis, gluteal cleft, paraspinal region and back. The diagnosis was established after staining for a panel of immunohistochemical markers namely cytokeratin, vimentin, S100, desmin, CD68 and smooth muscle actin. CD68 positivity in tumor cells helped in arriving at the final diagnosis. It is essential to recognize this tumor as a giant cell rich distinct entity and differentiate from other giant cell rich pleomorphic sarcomas since therapeutic and prognostic differences are being appreciated currently.

Fibrohistiocitoma maligno / Malignant fibrous histiocytoma

El fibrohistiocitoma maligno es el sarcoma de partes blandas más frecuente en el adulto mayor. Este tiende a afectar las partes blandas profundas de las extremidades y el retroperitoneo. Presentamos el caso de un paciente de 71 años de edad de seis meses de evolución en pierna izquierda quien presentó disnea y dolor toráxico dos días antes de su ingreso. Debido a su gran agresividad, poca frecuencia y difícil diagnóstico se hace una breve revisión.

The malignant fi brous histiocytoma is the most common malignant sarcoma of older adults. It tends to occur in the deep soft tissue of theextremities and the retroperitoneum. We report the case of 71 years old man, with a tumor lesion of six month on left leg who had disnea andchest pain two days ago. Because its highly aggressive, infrequency and diffi cult diagnosis make a brief revision.