Solitary fibrous tumor: Two cases report with a focus on the differential diagnosis / Tumor Fibroso Solitário: Relato de dois casos com foco no diagnóstico diferencial

Solitary fibrous tumor (SFT) is a neoplasm of fibroblastic/myofibroblastic origin with intermediate biological behavior. We report here two cases of SFT affecting an unusual anatomical site in 58-year-old and 40-year-old female patients and discuss the differential diagnosis of this lesion. In case 01, the lesion showed the clinical appearance of an asymptomatic "blister" with normal color, rubbery consistency, measuring 0.3 cm, and affected the lower lip; while in case 02, a symptomatic red nodular lesion with a soft consistency and measuring 0.5 cm affected the floor of the mouth. Excisional biopsies were performed. Microscopically, two well-delimited benign neoplasms were observed, exhibiting the proliferation of ovoid to spindle-shaped mesenchymal cells, vascular spaces with staghorn arrangement, and the absence of mitosis figures. Immunohistochemistry was performed in case 01 to assist in the diagnosis. Weak and diffuse immunostaining was observed for α-SMA and intense and diffuse immunopositivity for Bcl-2 and CD34. Based on histopathological and immunohistochemical features, a diagnosis of SFT was rendered in both cases. The low occurrence and nonspecific clinical features of oral SFT may make its clinical diagnosis difficult. Also, morphological and immunohistochemical are essential for differential diagnosis with other mesenchymal neoplasms.(AU)

O tumor fibroso solitário (TFS) é uma neoplasia de origem fibroblástica/miofibroblastica com comportamento biológico intermediário. Nesse artigo relatamos dois casos de TFS afetando sítios anatômicos incomuns em pacientes do sexo feminino de 58 anos e 40 anos e discutir os seus diagnósticos. No caso 01 clinicamente a lesão apresentou um aspecto de "bolha" assintomática, coloração normal da mucosa, consistência borrachoide medindo 0,3 cm, em região de lábio inferior, enquanto que no caso 02, como uma lesão sintomática, vermelha, nodular com consistência mole e medindo 0,5 cm afetando o assoalho bucal. As biópsias excecionais foram realizadas. Microscopicamente, observamos duas lesões neoplásicas bem delimitadas exibindo uma proliferação de células mesenquimais variando de ovoides a fusiformes, vasos sanguíneos em formato de "chifre de veado", com ausência de figuras de mitoses. No caso 01 foi realizado análise imuno-histoquímica para auxiliar no diagnóstico. Foi observado uma marcação fraca e difusa de α-SMA e uma intensa e difusa imunopositividade para o Bcl-2 e CD34.Baseado nos achados histopatológicos e imuno-histoquímicos o diagnóstico de TFS foi estabelecido em ambos os casos. A baixa ocorrência e os achados clínicos inespecíficos do TFS oral podem dificultar o diagnóstico clínico. Além disso, as análises morfológicas e imuno-histoquimicas são essenciais para realização do diagnóstico diferencial com outras neoplasias mesenquimais.(AU)_

Solitary fibrous tumor of the lacrimal gland mimicking pleomorphic adenoma / Tumor fibroso solitário de glândula lacrimal simulando adenoma pleomórfico

ABSTRACT Solitary fibrous tumor (SFT) is a rare mesenchymal spindle-cell neoplasm commonly found in the pleura; it is rare in the orbit and extremely rare in the lacrimal gland. We herein report a case of SFT of the lacrimal gland that mimicked a pleo morphic adenoma. We discuss the clinical, radiological, histopathological, and immunohistochemical findings that provided insight and rationale to accurately diagnose this case.

RESUMO Os autores relatam um caso de tumor fibroso solitário (TFS) de glândula lacrimal simulando um adenoma pleomórfico. O TFS é um raro tumor mesenquimal de células fusiformes, comumente encontrado na membrana pleural, raramente en contrado na órbita e extremamente raro na glândula lacrimal. Os autores discutem os achados clínicos, radiológicos, histológicos e imunohistoquímicos que são a chave para o correto diagnóstico desta rara entidade.

Tumor fibroso solitario de la glándula tiroides con extensión intratorácica / Solitary fibrous tumor of the thyroid gland with intrathoracic extension

El tumor fibroso solitario (TFS) es una neoplasia mesenquimal de células fusiformes infrecuente descrita inicialmente en la pleura pero con localización reconocida en otros sitios. El TFS de la glándula tiroides (TFST) es aun más raro. Se describe el caso de una mujer de 65 años de edad, con masa cervical de crecimiento progresivo, a tal punto que ya la masa se hacía notable en parte inferior de su cuello, siendo este el único síntoma narrado. La paciente presentaba una gran masa tiroidea en el lóbulo derecho con extensión intratorácica. Se practicó hemitiroidectomia derecha (paciente tenia historia previa de resección del lóbulo tiroideo izquierdo por lesión benigna) Total, además de toracotomía endoscópica por la extensión de la masa la cual en la tomografía contrastada, se originaba en el lóbulo tiroideo derecho y descendía paralelo a la columna dorsal desplazando a la tráquea y el esófago. La patología reportó un tumor de 15 centímetros, con células fusiformes y patrón de crecimiento hemangiopericítico sin pleomorfismo, atipia, mitosis o necrosis. Luego de practicar diferentes estudios y marcadores tumorales, de todos estudios fueron positivos CD34, Bcl2, CD99 y vimentina. Se diagnosticó tumor fibroso solitario de glándula tiroides. El nervio vago derecho fue lesionado en la cirugía, actualmente está en terapia de voz. Al momento actual casi seis meses luego de su cirugía, no se documenta recidiva tumoral.

Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm initially described as spindle cells in the pleura but recognized location elsewhere. The TFS thyroid gland (TFST) even infrequent. A case of a 65 years old woman with progressive growth of a right cervical mass without associated symptoms described other than cosmesis is described . The patient had a thyroid mass in the right lobe with intrathoracic extension. A right Hemi thyroidectomy and thoracotomy for the extent of the mass was performed. Pathology reported a 15 cm tumor with spindle cell and growth pattern hemangiopericitic without pleomorphism, atypia, mitosis or necrosis. Immunohistochemistry was positive for CD34, Bcl-2, CD99 and vimentin, making the diagnosis of solitary fibrous tumor of thyroid gland. The patient's right Vagus nerve was injured intraoperatively and she is currently under voice therapy. Currently now, almost six months after her surgery she is free of disease.

Endobronchial solitary fibrous tumor

Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that appears primarily in the pleura and rarely in intrapulmonary or endobronchial topography. The authors report the case of a 47-year-old woman who presented obstructive respiratory symptoms for 4 years. The chest computed tomography and bronchoscopy showed an obstructive polypoid lesion located between the trachea and the left main bronchus associated with distal atelectasis of the left lung. A resection of the lesion was performed and, macroscopically, the mass was oval, encapsulated, and firm, measuring 2.3 × 1.7 × 1.5 cm. Histology revealed low-grade mesenchymal spindle cell neoplasm, with alternating cellularity, myxoid areas, and mature adipose tissue outbreaks, as well as blood vessels with irregular walls. The immunohistochemical study was positive for CD34, CD99, and BCL2. The diagnosis was SFT in an unusual topography. The patient's symptoms remitted after tumor excision, and no systemic problems were evident. SFTs primarily affect adults and often follow a benign course; however, their behavior is unpredictable. The presence of necrosis and mitotic activity may portend a poor prognosis. Endobronchial SFTs are rare but should be evaluated and monitored similar to SFTs at other sites, with a long-term follow-up

Tumor fibroso solitário retroperitoneal volumoso / Giant solitary fibrous tumor of retroperitoneum

O tumor fibroso solitário (TFS) é uma neoplasia mesenquimal rara caracterizada pela proliferação de células fusiformes, geralmente acometendo a pleura. É diagnosticado, de modo geral, após estudo anátomo-patológico e por imuno-histoquímica, já que muitas vezes encontra-se em local pouco usual e assemelha-se a outras neoplasias. Relatamos o caso de um paciente de 46 anos com massa retroperitoneal compatível com tumor fibroso solitário de baixo grau (AU)

The sol itary fibrous tumor (SFT) is a rare mesenchymal neoplasm characterized by proliferation of spindle cells, usually affecting the pleura. It is usually diagnosed after pathological study and by immunohistochemistry, as it is often located in an unusual location and resembles other cancers. Here we report the case of a 46-year-old patient with retroperitoneal mass consistent with low grade solitary fibrous tumor (AU)

Malignant solitary fibrous tumor of the thyroid: a case-report and review of the literature / Tumor fibroso solitário maligno da tiroide: relato de caso e revisão da literatura

Solitary fibrous tumor (SFT) is an uncommon spindle-cell neoplasm that most often involves the pleura, rarely occurring in extra-thoracic locations. Twenty-six cases of SFT arising in the thyroid gland have been described. We report a case of a 60-year-old woman presenting an 8-month history of enlargement of the neck associated with dysphagia. The patient underwent a right hemithyroidectomy and SFT of the thyroid was diagnosed. Immunohistochemistry showed positivity for CD34 marker, and the high number of mitoses and the presence of cellular atypia suggested that the tumor was malignant. To our knowledge, this is the second case of malignant SFT of the thyroid gland ever reported. Due to the rarity of these tumors, the indication of adjuvant therapy and prognosis are uncertain. Long-term follow-up after surgical resection seems to be advisable.

O tumor fibroso solitário (SFT) é uma neoplasia rara de células fusiformes que mais frequentemente envolve a pleura, raramente ocorrendo em áreas extratorácicas. Já foram descritos 26 casos de SFT da tiroide. Relatamos o caso de uma paciente de 60 anos de idade com um histórico de 8 meses de aumento do pescoço associado à disfagia. A paciente foi submetida a uma hemitiroidectomia direita e foi diagnosticado um SFT de tiroide. A imuno-histoquímica mostrou resultados positivos para o marcador CD34, e o grande número de mitoses e a presença de atipia celular sugerem que o tumor era maligno. Em nosso conhecimento, este é o segundo caso de STF da tiroide maligno já relatado. Dada a rara ocorrência desses tumores, a indicação de tratamento adjuvante e o prognóstico são incertos. Recomenda-se o acompanhamento de longo prazo depois da ressecção cirúrgica.

Tumor fibroso solitario del tiroides / Solitary fibrous tumor of the thyroid

Solitary fibrous tumor (SFT) is a rare spindle cell neoplasm mostly found in the pleural tissue but also described in other parenchymas. There are twenty four cases of thyroid SFT, all of them with similar histological appearance and immunohistochemical reaction pattern. We report the case of a thyroid tumor in a thirty year old man. The tumor was characterized by a proliferation of spindle cells arranged in a storiform and hemangiopericitoid pattern, without cell atypia and necrosis, and with low mitotic rate. The tumor showed a strong positive immunohistochemical reaction for CD-34 and bcl-2. There were no reactions for Desmin, Pancytokeratin and S-100. All of these features confirm the diagnosis of a thyroid SFT. It is important to know that this entity can arise in the thyroid gland to direct the analysis in a correct way.

El tumor fibroso solitario (TFS) es una neoplasia de células fusadas, de muy baja frecuencia, encontrada mayormente en la pleura pero también descrita en sitios extrapleurales. En la literatura se han reportado 24 casos de TFS originados en el tiroides, todos con características patológicas e inmunohistoquímicas (IHQ) similares. Reportamos el caso de un tumor tiroideo en un hombre de 30 años. El tumor se caracterizó por ser una proliferación de células fusadas dispuestas en un patrón estoriforme y hemangiopericitoide, sin necrosis ni atipias, con baja actividad mitótica. El estudio IHQ presentó positividad para CD34 y bcl-2 y negatividad para Desmina, Pancitokeratina y S-100, características concordantes con un TFS. Es importante saber que esta entidad puede surgir en el tiroides y así poder orientar el estudio para su diagnóstico correcto.

Hepatic solitary fibrous tumor: Report of a rare case.

Solitary fibrous tumor (SFT) of the liver is an extremely rare neoplasm of mesenchymal origin. In the English literature, less than 40 cases of SFTs of the liver have been reported. The present case concerns a 34-year-old female who presented to us with complaint of dyspepsia. On examination, there was hepatomegaly. On ultrasound examination, an SOL in the liver was detected. Large tumor measuring 14.5 × 10 × 8 cm was resected. Microscopic evaluation of the tumor showed a well-circumscribed, low to moderately cellular tumor demonstrating spindle- and fibroblast-like cells within the collagenous stroma. Immunohistochemistry revealed diffuse strong cytoplasmic immunopositivity of CD34, Bcl2, and vimentin. A diagnosis of a benign SFT was given. The patient remained well 4 years after surgery. SFT is a rare mesenchymal neoplasm that occasionally involves the liver in adult patients. Most SFTs are benign, but some may have malignant histological features. With less than 40 reported cases in the literature, little can be said regarding its natural history or the benefits of adjuvant radio chemotherapy. Complete surgical resection remains the cornerstone of its treatment.

Hemangiopericitoma de aurícula izquierda: Caso clínico / Primary left atrial hemangiopericytoma: Report of one case

We report a 41-year-old male presenting with progressive dyspnea lasting one month. A CAT scan disclosed a left atrial mass, that was surgically excised. The pathological study of the surgical piece showed a primary hemangiopericytoma. One month later, the patient consulted for cervical pain and a positron emission tomography showed multiple metastases. The patient died two months later.

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.

Solitary fibrous tumor of the orbit presenting in pregnancy.

A 32-year-old woman, three months pregnant, reported with the complaint of protrusion of the right eye for six months. She gave history of rapid protrusion of eyeball for the last two months along with the history of double vision for the last one month. Computer tomography (CT) scan revealed a well-defined mass lesion in the intraconal space of the right orbit which was excised through a lateral orbitotomy approach. Histological examination and immunohistochemistry revealed a solitary fibrous tumor, which showed a rapid progression in pregnancy.

Solitary Fibrous Tumor of the Trachea: CT Findings with a Pathological Correlation

We present the multidetector CT findings with a pathologic correlation for the case of a solitary fibrous tumor located in the trachea. The MDCT revealed a well-circumscribed intraluminal mass arising from the trachea, with strong nodular enhancement in the periphery of the mass. The enhancement pattern of the mass corresponded histopathologically to a focal hypocellular area in the center and prominent blood vessels along the periphery of the mass. We also present volume-rendered and virtual bronchoscopic images of this rare submucosal tracheal tumor.

A Dumbbell-Shaped Solitary Fibrous Tumor of the Cervical Spinal Cord

A 40-year-old Asian female presented with a 2-month history of right shoulder pain and right triceps weakness. MRI revealed an extramedullary, extradural, dumbbell-shaped spinal cord tumor with C6 to C7 iso- and hyperintensity on T1 and T2 weighted imaging, respectively. Histological examination revealed monomorphous spindle cells with a storiform pattern. Immunohistochemistry was positive for CD34, CD99, and negative for EMA, SMA, and S100; solitary fibrous tumor (SFT) was confirmed.