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.

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

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

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.

Case for diagnosis / Caso para diagnóstico

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

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

Fibrohistiocitoma maligno de las extremidades - una revisión retrospectica en el Instituto Nacional de Enfermedades Neoplásicas Dr. Eduardo Cáceres Graziani del año 1996 al 2006 / Malignant Fibrohistiocytoma of limbs - a review retrospectica at the National Institute of neoplastic diseases Dr. Eduardo Cáceres Graziani from 1996 to 2006

Objetivo: Conocer las características clínicas y patológicas de los pacientes con fibrohisticitoma maligno (FHM) de las extremidades diagnosticados en el Instituto Nacional de Enfermedades Neoplásicas. Material y Metodos: Se realizó un estudio descriptivo retrospectivo tipo serie de casos de 222 pacientes con diagnostico de FHM de las extremidades del año 1997 al 2006. Resultados: El promedio de edad fue 56.3 años (rango 18-96 años). El tamaño tumoral medio fue de 10.3 cm (rango 1-35 cm). 2,7% fueron grado histológico I; el 12.2%, grado II y el 66%, grado III. La recurrencia local fue 23.1% y la metástasis a distancia ocurrió en 27.6% (68.2 % a los pulmones). El tiempo de sobrevida media a 5 años fue 58%. La profundidad del tumor influenció en la SLE, p < 0.015. En el análisis multivariado el único factor asociado a una mayor tasa de recurrencia local fueron los márgenes quirúrgicos positivos (p < .003). Los factores pronósticos significativos en el desarrollo de enfermedad a distancia fueron la profundidad (p < .004) y la recurrencia local (p < .02); mientras que los factores pronostico que afectan la sobrevida fueron, la profundidad del tumor (p < .04), la presencia de metástasis a los ganglios linfáticos regionales (p < .02) y los márgenes quirúrgicos positivos (P < .03). Los tratamientos adyuvantes no mejoraron la sobrevida. Conclusiones: Los factores pronóstico de recurrencia local fueron los márgenes quirúrgicos positivos. Los factores pronóstico de recaídas a distancia fueron la profundidad y recurrencia local, mientras que los factores importantes que afectan la sobrevida fueron la profundidad del tumor, la presencia de metástasis a los ganglios linfáticos regionales al momento del diagnostico y los márgenes quirúrgicos positivos.

Objective: Malignant fibrous histiocytoma (FHM) is the most common subtype of soft tissue sarcoma (STS). The purpose of this study is to analyze the prognostic factors influencing local recurrence and survival. Material and Methods: A retrospective review was performed at the National Institute of Neoplastic Diseases (INEN) in all patients with a diagnosis of extremity FHM, between 1997 and 2006. Results: We reviewed cases histories of 222 patients with histologically confirmed FHM. Mean age was 56.3 years (range 18-96 years). Mean tumor size was 10.3 cm (range 1-35 cm). 2.7% of the cases had histological grade I, 12.2%, grade II and 66%, grade III. Local recurrence was 23.1% with an average of 16 months. Distant metastasis occurred in 27.6% of patients. The median survival time was 108 months with a cumulative survival rate at 5 years of 58%. The depth of the tumor influenced the disease-free survival (DFS) (depth vs. superficial, P <0.015). In the multivariate analysis the only factor associated with a higher rate of local recurrence was positive surgical margins (P<.003). Significant prognostic factors in the development of distant disease were depth (P <.004) and the recurrence (P <.02), while still important prognostic factors affecting survival were tumor depth (P <.04), presence of metastases to regional lymph nodes (P <.02) and positive surgical margins (P <.03). Adjuvant radiotherapy reduced local recurrence but did not reach statistical significance. Chemotherapy had no significant improvement in survival or local recurrence rates and metastasis. Conclusion: The only prognostic factor for local recurrence was positive surgical margins. Prognostic factor for distant relapse were the depth of tumor and local recurrence, while other important factors affecting survival were tumor depth, presence of metastases to regional lymph nodes at diagnosis and positive surgical margins.

Fibrohistiocitoma maligno en pared abdominal: reporte de un caso / Malignant fibrohystiocytoma of the abdominal wall: a case report

El fibrohistiocitoma maligno es el sarcoma de partes blandas más común en la edad adulta, en un grupo etario mayor de 50 años y afecta principalmente a hombres. Estos tumores mesenquimales, con sus distintas variedades morfológicas, precisan de un análisis inmunohistoquímico para su diagnóstico diferencial, fundamentalmente con el carcinoma sarcomatoide, el tumor miofibroblástico inflamatorio y el leiomiosarcoma. Macroscópicamente son neoplasias voluminosas, con múltiples áreas de necrosis en la superficie de corte; desde el punto de vista microscópico el tumor muestra una proliferación desordenada de células fusocelulares, con un patrón esteliforme y/o presencia de células multinucleadas con atipia, mitosis atípicas y un estroma que muestra gran cantidad de colágeno así como un número variable de células inflamatorias mononucleadas e histiocitos espumosos. Su diagnóstico es realizado en base a su morfología e inmunohistoquímica. Presentamos el caso de un paciente masculino de 54 años y la forma como se manejó en nuestro departamento.

The malignant fibrohystiocytoma is the most frequent soft tissue sarcoma in the adult age, in an age group mayor of 50 years old, and affect principal to the man. These mesenquimal tumors with distinct morphological varieties need an immunohistochemistry analysis for his differential diagnostic, principal with the sarcomatoide carcinoma, the miofibroblastic inflammatory tumor and the leiomiosarcomas. Macroscopically there are bulky neoplasm with multiples necrosis areas in the superficial incision, of the microscopically point of view, the tumor show a no order proliferation of the fusocellular cells, with a esteliform patron and or the presence of multinucleate cells with atypical mitosis and a stroma, with show a big quantities of collagen and a variety numbers of inflammatory cells mononucleotic and spumoni hystiocyts. The diagnostic is realized in base to his morphologic and the immunohistochemistry. We present the case of a male patient 54 years old and the form of his management.

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.

Tumor fibroso solitario gigante de la pleura / Giant solitary fibrous tumor of the pleura

Introducción: El tumor fibroso solitario es el segundo tumor primario de la pleura y puede alcanzar hasta 39 cm de diámetro; para tener la denominación de “gigante” debe ocupar al menos 40 % del hemitórax afectado. Por lo general su comportamiento es benigno, pero existen criterios de malignidad. El objetivo de esta investigación fue efectuar una revisión de la evaluación inicial, diagnóstico, manejo quirúrgico, resultado del tratamiento y pronóstico. Material y métodos: Estudio descriptivo, observacional, longitudinal y retrospectivo, realizado de 2002 a 2006, en pacientes operados con diagnóstico de tumor fibroso solitario gigante de la pleura. Resultados: Se incluyeron seis pacientes, 83.3 % del sexo femenino, con edad promedio de 48 años; todos sintomáticos con predominio de disnea, tos y dolor; en 66.7 % se encontró del lado izquierdo; a 83.3 % se realizó angiografía y embolización preoperatorias, logrando resección completa en todos; predominó aporte arterial de la arteria mamaria interna. Se encontró una tasa de complicaciones transoperatorias de 17 %. En 66.7 % se identificó un pedículo ascular; el tumor mayor midió 40 cm de diámetro con peso de 4500 g; solo uno presentó actividad mitótica elevada. El seguimiento promedio fue de 14 meses. Conclusiones: La sintomatología encontrada fue acorde con informes previos, aunque en porcentajes mayores. El diagnóstico correcto es de vital importancia, ya que con la resección quirúrgica el tumor fibroso solitario es potencialmente curable, sin embargo, requiere seguimiento a largo plazo. Dado el tamaño de este tipo de tumores es aconsejable llevar a cabo embolización preoperatoria.

BACKGROUND: Solitary fibrous tumor is the second primary malignancy of the pleura and can reach up to 39 cm in diameter; however, to be referred to as 'giant' it must occupy at least 40% of the affected hemithorax. Although this tumor usually shows a benign behavior, malignancy criteria have been described. The aim of the study was to assess the initial evaluation, diagnostic procedures, surgical management, treatment outcome, and prognosis. METHODS: We performed a descriptive, observational, longitudinal, and retrospective study from 2002 to 2006 on patients who underwent surgery with a diagnosis of giant solitary fibrous tumor of the pleura. RESULTS: Six patients were included; 83.3% were females. Mean age was 48 years. All patients were symptomatic, mainly dyspnea, cough and chest pain; 66.7% were left-sided. Preoperative angiography and embolization were performed in 83.3% cases with successful surgical resection. The predominant blood supply was derived from the internal mammalian artery. Intraoperative complication rate was 17%. A vascular pedicle was found in 66.7%. The largest lesion was 40 cm in diameter and weighed 4500 g. Only one case showed high mitotic activity. Mean follow-up to date is 14 months. CONCLUSIONS: Symptomatology found was consistent with previous reports but in higher percentages. Accurate diagnosis is critical because surgical resection involves a potential cure; however, long-term follow-up is mandatory. Preoperative embolization is recommended due to tumor size.

Histiocitoma fibroso maligno pleomórfico: presentación de un caso y revisión de la literatura / Malignant pleomorphic fibroushistiocytoma: report of a case and of literature review

Se presenta un caso clínico de histiocitoma fibroso maligno pleomórfico diagnosticado mediante valoración clínica, tomografía axial computarizada con contraste, biopsia por tru-cut preoperatoria y estudio inmunohistoquímico e histológico. A quien en vista del tamaño, grado de diferenciación tumoral y ausencia de metástasis se decide realizar exéresis quirúrgica de la tumoración de 4300 g, 50 cm x 30 cm y tratamiento radioterápico adyuvante. Evidenciándose satisfactoria evolución a sus seis meses de seguimiento, caracterizado por cicatrización estética y funcional, aumento de peso y ausencia de recidiva tumoral. En vista que la presentación de esta patología es poco frecuente en el mundo entero, se decide hacer una revisión de la literatura y discusión de los hallazgos anatomopatológicos y clínicos concernientes al caso.

Clinical case of patient with fibroses pleomorphic hystiocitome diagnosed by clinic valuation, on-line axial tomography with contrast, immunohystochemistry and pathology study. Who in view of the size, grade of differentiation tumor and metastasis absence decides to carry out a surgical exeresis of tumor 4300 g, with a length 50 cm x 30 cm and radiotherapy coadjutant therapy. Being evidenced, satisfactory evolution six months of pursuit, characterized by aesthetic and functional scaring, increase of weight and absence of relapse tumor. In view of the fact that the presentation of this pathology is not very frequent in the whole world, we decide to make a revision of the literature and discussion of the discoveries pathology and clinical concerning to the case.

Fibro-histiocitoma maligno da pálpebra: relato de caso / Malignant fibrous histiocytoma of the eyelid: case report

Os fibro-histiocitomas representam 1 por cento das massas orbitárias e podem ser histologicamente classificados em benignos, localmente agressivos e malignos - os últimos representando aproximadamente 11 por cento do total. Nesse estudo, os autores descrevem um caso raro de fibro-histiocitoma maligno de pálpebra, ressaltando as características clínicas e histopatológicas desta neoplasia.

Fibrous histiocytomas represent 1 percent of all orbital masses and may be classified into benign, locally aggressive and malignant - these representing nearly 11 percent of the total. In this study, the authors describe an unusual case of malignant fibrous histiocytoma of the eyelid, discussing clinical and histological features of this neoplasm.

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.

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.

Oral fibro-histiocytic lesions: immuno-histological investigation and review of an unusual presentation in the tongue

A right sided mid-tongue growth in a 14-year-old boy diagnosed initially as Pyogenic Granuloma was found subsequently to be a Benign Fibrous Histiocytoma. This lesion had three seeming recurrences with divergent histologic and clinical appearances within a short period of time. Although there was eventual consensus on the morphogenesis and benign histologic nature of the lesion this caused diagnostic and management problems and a potential prognostic dilemma. This report on a unique and aggressive head and neck lesion, possibly the third documented case ill the tongue, reviews the bewildering spectrum of clinical and immuno- histologic manifestations of fibrous histiocytic lesions. Wide excision with close follow up is recommended

Tumor-to-tumor metastases from lung carcinoma to soft tissue fibrous histiocytoma

Tumor-to-tumor metastases are uncommon. Lung cancer is the most frequent donor tumor while renal cell carcinoma is the most common malignant, and meningioma the most common benign recipient of metastases. Other reported host benign lesions include thyroid adenoma, adrenocortical adenoma, ovarian cystoma, and hepatic cavernous angioma. A 41-year-old male smoker presented with a mass in the right axillary's fossa, cough and weight loss. Chest x-ray showed left lung collapse. Fiberoptic bronchoscopy showed vegetation in the left main bronchus and biopsy revealed adenocarcinoma. Biopsy of the right axillary mass showed benign fibrous histiocytoma [BFH] with metastasis from adenocarcinoma. To our knowledge, this report represents the first case of tumor-to-tumor metastasis to BFH

Cardiac metastasis in malignant fibrous histiocytoma

Malignant fibrous histiocytoma [MFH] is an aggressive spindle cell cancer and is the most common soft tissue tumor in the elderly, primarily affecting the extremities. It has high metastatic potential and can spread to various viscera including liver, lung, bone, and brain; however, cardiac metastasis is an extreme rarity. Here, we present a 50-year-old male, diagnosed as pleiomorhphic storiform MFH a of right arm who developed parenchymal pulmonary metastases and a mass lesion in left atrium. Patient had a downhill course and eventually succumbed