Tumor fibroso solitario hepático gigante. Informe de caso en la Argentina / Giant solitary fibrous tumors of the liver. A case report in Argentina

RESUMEN Los tumores fibrosos solitarios hepáticos, una neoplasia poco frecuente, son tumores benignos del tejido submesotelial, pero con un potencial maligno no definido. Suelen presentarse asintomáticos, pero pueden debutar como una masa abdominal palpable y con síntomas de plenitud. Por imágenes pueden ser confundidos con otros tumores y está indicado el tratamiento quirúrgico. Presentamos el caso de una paciente de 70 años resuelto por hepatectomía lateral izquierda, y realizamos una revisión bibliográfica del tema.

ABSTRACT Solitary fibrous tumors of the liver are uncommon benign tumors originating from the submesothelial tissue with non-well-defined malignant potential. Most cases present as asymptomatic, some cases show abdominal bloating and a palpable mass. They may mimic other tumors on imaging tests and surgery is indicated. We report a case of a 70-year-old female patient treated with left lateral liver resection with literature review of the condition.

Tumor fibroso solitario y síndrome Doege-Potter / Solitary fibrous tumor and Doege-Potter syndrome

El tumor fibroso solitario es raro. Si bien su localización más frecuente es la pleura, han sido informados algunos casos de ubicación retroperitoneal. Es difícil diferenciarlo imagenológicamente de otras neoplasias, por lo que casi siempre el diagnóstico es histológico. Dado que los tumores fibrosos solitarios tienen presentaciones clínicas muy diversas, una mejor compresión de la ubicación y sus características imagenológicas ayudaría a abreviar la lista de diagnósticos diferenciales. Comunicamos un tumor fibroso solitario de localización retroperitoneal cuyo diagnóstico fue confirmado por examen histológico.

The solitary fibrous tumor is uncommon. Even though it frequently locates in the pleura, a few cases have been reported in the retroperitoneum. Differentiation from other neoplasms is difficult, and therefore the diagnosis is always attained through histological examination. Although solitary fibrous tumors have variable clinical behaviors, a better comprehension of the location and its imaging characteristics would help to decrease the list of differential diagnoses. We report a solitary fibrous tumor located in the retroperitoneum whose diagnosis was confirmed by histological examination.

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.

Tumor fibroso solitario del lóbulo caudado del hígado / Solitary fibrous tumor of the liver caudate lobe

Introduction: Solitary fibrous tumor of the liver (SFTL) constitutes such a rare finding that it has been published only as case reports and to the present date there are only 45 cases published in English medical literature. This article describes the case of a patient treated at our institution with SFTL. Case report: A male 59-years-old patient was diagnosed with a SFTL incidentally found in a computed abdominal tomography taken by another causes, tumor was originating from the caudate lobe. The patient was asymptomatic. At surgery, the tumor was observed originating directly from the caudate lobe through a pedicle measuring approximately 3 cm. The tumor was resected sectioning its implantation pedicle and ligating all arterial, venous and biliary vessels. The SFTL was well encapsulated, measured 15 x 9 x 6 cm and weighted 794 g. The histological diagnosis was a SFTL and was confirmed by immunohistochemistry with monoclonal antibodies that reacted positively to CD34 and vimentin and negatively to CD117, S100, smooth-muscle α-actin and desmin. Conclusions: SFTL is an uncommon tumor. The present case is the first reported in Chilean medical literature and presented all the habitually described radiologic, surgical and pathological characteristics.

Introducción: El tumor fibroso solitario del hígado (TFSH) constituye un hallazgo tan raro que se ha publicado únicamente como reportes de caso y hasta el presente se encontraron sólo 45 casos publicados en la literatura inglesa. El presente artículo describe el caso de un paciente que fue tratado en nuestra institución por un TFSH. Reporte de caso: Paciente masculino de 59 años de edad al que, en una tomografía abdominal computarizada que se tomó por otras causas, se encontró en forma incidental un gran tumor hepático localizado en el lóbulo caudado del hígado. El paciente no refería síntomas relacionados con el tumor. En la cirugía se observó que el tumor se originaba directamente del lóbulo caudado al cual lo unía un pedículo de aproximadamente 3 cm de diámetro transverso. Se seccionó el tumor desde su base de implantación en el hígado ligándose los vasos arteriales y venosos así como los conductos biliares que se encontraron. Este medía 15 x 9 x 6 cm y pesaba 794 g, se encontraba bien encapsulado. El diagnóstico histológico fue el de un TFSH, el cual se confirmó mediante inmunohistoquímica con anticuerpos monoclonales que resultaron positivos para CD34 y vimentina en las células tumorales y negativos para CD117, S100, α-actina de músculo liso y desmina. Conclusiones: El TFSH es un tumor raro. El presente caso de un TFSH es el primero reportado en la literatura médica chilena y presentó las características radiológicas, quirúrgicas y patológicas habitualmente descritas en estos casos.

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)

Tumor fibroso solitário de laringe: relato de dois novos / Solitary fibrous tumor of the larynx: report of two new cases

Solitary fibrous tumor is a rare neoplasm. Few cases have been described in the head and neck area, and less than 11 were located in the larynx. We described two new cases of solitary fibrous tumor of the larynx. A man, 64-year-old, and a woman, 77-year-old, both with submucosal and nodular supraglottic lesions, were submitted to surgical treatment and both showed CD-34 and bcl-2 immunoreactivity and S-100 and smooth-muscle actin negativity. After 24 and 22 months of postoperative follow-up, respectively, they did not show signs of active disease...

O tumor fibroso solitário é uma neoplasia rara. Foram descritos poucos casos em cabeça e pescoço, sendo não mais de 11 na laringe. Descrevemos dois novos casos de tumor fibroso solitário da laringe, um em um homem de 64 anos e outro em uma mulher de 77 anos, ambos com lesões supraglóticas submucosas e nodulares. Os casos foram submetidos a tratamento cirúrgico e ambos apresentaram imunorreatividade a CD-34 e bcl-2, e negatividade para S-100 e actina de músculo liso. Após 24 e 22 meses de seguimento pós-operatório, respectivamente, não apresentam sinais de doença em atividade...

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.

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.

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.