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

Resumen Objetivo: Reportar el caso de una masa gigante en hemitórax izquierdo de 19 cm de diámetro en un paciente de 59 años que debutó con disnea, tos y dolor torácico, confirmándose por estudio imagenológico. Materiales y Método: Registro clínico de un paciente al cual se le diagnostica tumor fibroso solitario de pleura, siendo intervenido quirúrgicamente para exéresis de la lesión. Resultados: Se realiza toracotomía posterolateral izquierda para exéresis de tumor gigante, requiriendo además, resección de diafragma y pericardiectomía parcial con evolución favorable. Discusión: El tumor fibroso solitario es una neoplasia rara derivada del mesénquima que afecta más comúnmente a la pleura, típicamente bien circunscrita, pediculada, con vasos dentro del pedículo tumoral, pudiendo llegar a ser de gran tamaño, siendo considerados gigantes cuando tienen más de 15 cm de diámetro. Conclusión: El diagnóstico correcto es de vital importancia, ya que con la resección quirúrgica es potencialmente curable. El tratamiento quirúrgico puede efectuarse por toracotomía o videotoracoscopia, dependiendo del tamaño del tumor. A pesar del comportamiento benigno, requiere seguimiento a largo plazo debido a la tendencia a la recidiva.

Aim: To report the case of a 19 cm diameter giant mass in the left hemithorax in a 59-year-old patient who presented with dyspnea, cough and chest pain, confirmed by imaging study. Materials and Method: Clinical record of a patient who was diagnosed with a solitary fibrous tumor of the pleura, undergoing surgery to excise the lesion. Results: A left posterolateral thoracotomy was performed to excise the giant tumor, also requiring resection of the diaphragm and partial pericardiectomy with favorable evolution. Discussion: The solitary fibrous tumor is a rare neoplasm derived from the mesenchyme that most commonly affects the pleura, typically well circumscribed, pedunculated, with vessels within the tumor pedicle, and can become large, being considered giant when they are larger than 15 cm diameter. Conclusión: The correct diagnosis is of vital importance, since surgical resection it a potentially curable treatment. Surgical treatment can be performed by thoracotomy or videothoracoscopy, depending on the size of the tumor. Despite the benign behavior, it requires long-term follow-up due to the tendency to recur.

Mesotelioma pleural benigno: apresentação de um caso singular / Benign pleural mesothelioma: presentation of a singular case

Os autores relatam um caso de mesotelioma pleural benigno. São abordados aspectos histológicos, etiopatogênicos, genéticos, epidemiológicos e clínicos, bem como a casuística estudada, a terapêutica instituída e o segmento obtido. Abordam os critérios para o diagnóstico, resultante da somação de vários fatores, dando ênfase ao quadro clínico compatível, confirmação histopatológica, imuno-histopatológicos compatíveis com resposta positiva clínico-radiográfica e eficaz após a cirurgia proposta.

Cases of benign mesothelioma of the pleura are reported by authors. Histological, etiopathogenic, genetic, epidemiological and clinical aspects are approached as well the casuistry that was studied, established therapy and the acquired segment. It approaches the criteria for diagnosis resulted from many factors emphasizing compatible clinical condition, histopathological confirmation, compatible immuno-histopathology, effective and positive clinical radiography answer after the proposed surgery.

Solitary fibrous tumor of the pleura: 3 case reports / fibroso solitário de pleura: relato de 3 casos

Summary Introduction: solitary fibrous tumor of the pleura (SFTP) is a rare tumor arising from mesenchymatous cells in submesothelial pleural tissue which, unlike mesothelioma, is not related to asbestos or smoking. Methods: report of four patients who underwent surgical treatment for giant SFTP and review of the pertinent literature. Results: of the four patients operated, two presented symptoms including cough, chest pain and feeling of compression, whereas the other two subjects were asymptomatic. All patients underwent complete surgical resection by wide posterolateral thoracotomy, and surgical specimens removed with minimum bleeding. None of the cases required complementary lobectomy or segmentectomy. All tumors were histologically benign. Conclusion: complete resection of the lesion is the treatment of choice in all SFTP cases. Prognosis of the benign lesion is excellent, although close follow-up is necessary. In the rarer, more aggressive forms, treatment may be complemented by adjunctive chemotherapy or radiotherapy, the benefits of which have yet to be confirmed. .

Resumo Introdução: o tumor fibroso solitário de pleura (TFSP) é um tumor raro com origem nas células mesenquimatosas do tecido pleural submesotelial, que, ao contrário do mesotelioma, não tem relação com asbesto ou tabagismo. Método: relato de caso de quatro pacientes submetidos a tratamento cirúrgico para TFSP gigante e revisão da literatura pertinente. Resultados: dos quatro pacientes operados, dois apresentaram sintomas como tosse, dores no peito e sensação de compressão enquanto os demais foram assintomáticos. Todos os pacientes foram submetidos à ressecção cirúrgica total por toracotomia posterolateral ampla, sendo os espécimes cirúrgicos removidos com mínimo sangramento. Em nenhum dos casos houve necessidade de lobectomia ou segmentectomia complementar. Todos os tumores eram histologicamente benignos. Conclusão: a ressecção total da lesão constitui o tratamento de escolha em todos os casos de TFSP. O prognóstico de lesões benignas é excelente embora o acompanhamento seja necessário. Nas formas mais raras e agressivas, o tratamento pode incluir quimioterapia ou radioterapia adjunta, cujos benefícios ainda não foram confirmados. .

Tumor fibroso solitario pleural: cirugía y recurrencia tardía / Pleural solitary fibrous tumor: surgery and late recurrence

Se presenta el caso de una paciente con diagnóstico de tumor fibroso solitario pleural derecho gigante de 30 cm de diámetro mayor, quien tuvo tratamiento quirúrgico del tumor mediante toracotomía amplia con remoción completa de la masa tumoral, se consideró cirugía RO, sin embargo, luego de 20 años más tarde presentó recurrencia de la enfermedad considerada de larga latencia.

A case report of a patient with a diagnosis of pleural solitary fibrous giant tumor 30 cm in diameter is presented, who underwent to surgical treatment with complete ablation of the tumor by wide thoracotomy and it was considered RO surgery. 20 year after surgery the pacient was diagnosed as later recurrence of the disease considered long latency.

Tumor fibroso solitario de la pleura asociado a síndromes paraneoplásicos / Solitary fibrous tumor of pleura associated with paraneoplastic syndromes

Introduction: solitary fibrous tumors of pleura (SFTP) are occasionally associated with paraneoplastic syndromes such as hypoglycemia and diffuse skeletal hyperostosis. Clinical case: we report a 60 years old male presenting with episodes of disorientation and inappropriate behavior, along with cough and weight loss. On physical examination there was digital clubbing. A chest X ray showed a big tumor in the right hemithorax. Fasting blood glucose was 24 mg/dl and erythrocyte sedimentation rate was elevated. Large bone X rays showed hyperostosis. A needle biopsy of the tumor showed a SFTP. The tumor was excised and the final pathological diagnosis was a benign SFTP. Hypoglycemia subsided after surgery. After four months of follow up, finger clubbing was disappearing.

Introducción: los tumores fibrosos solitarios de la pleura (TFSP) son neoplasias infrecuentes, consideradas lesiones benignas en general, con potencial maligno dependiendo de su histología. Se asocian en raras ocasiones a síndromes paraneoplásicos como la hipoglicemia (Síndrome de Doege-Potter) e hiperostosis ósea difusa (osteoartropatía hipertrófica pulmonar). Caso clínico: consulta un varón de 60 años en el Hospital Clínico de la Pontificia Universidad Católica de Chile por episodios de desorientación y conductas inapropiadas, junto con tos y baja de peso. Además refiere dolor en extremidades y aumento de volumen de falanges distales de manos. Al examen destaca disminución del murmullo pulmonar a derecha e hipocratismo digital. Se estudia con radiografía y tomografía de tórax revelando un gran tumor en hemitórax derecho de 20 por 18 cm con efecto de masa. Exámenes destacan hipoglicemia de 24 mg/dl y VHS elevada. Estudio endocrinológico y neurológico normal, radiografías de huesos largos y manos revelan hiperostosis ósea. Se estudia con biopsia de aguja gruesa, compatible con TFSP. Se realiza resección completa por toracotomía. Biopsia definitiva confirma diagnóstico de TFSP benigno. Paciente evoluciona satisfactoriamente, resolviéndose hipoglicemia y alteraciones conductuales. Se va de alta al octavo día postoperatorio. Seguimiento a los 4 meses sin nuevos episodios de hipoglicemia y resolución progresiva del hipocratismo digital. Radiografías de tórax sin recidiva. Conclusiones: Los TFSP son inhabituales, se pueden presentar con algunos síndromes paraneoplásicos que deben llevar a la sospecha diagnóstica. Su tratamiento consiste en la resección completa del tumor, logrando escasa recidiva y mejoría sintomática.

Tumor fibroso solitario pleural de lenta evolución / Giant solitary fibrous tumour of the pleura of slow evolution

El tumor fibroso solitario pleural es una neoplasia infrecuente originada en las células submesoteliales de la superficie pleural. Generalmente se manifiesta como una masa exofítica que crece hacia dicho espacio comprimiendo estructuras adyacentes. Su diferencia con el mesotelioma pleural maligno no sólo yace en su origen histológico, sino también en los antecedentes epidemiológicos y el curso clínico de la enfermedad. Presentamos el caso de un paciente con una masa pleural gigante de 11 años de evolución, los inconvenientes suscitados con el diagnóstico y, finalmente, la resolución quirúrgica definitiva.

The solitary fibrous tumor of the pleura is a rare neoplasm originating from submesothelial cells of the pleural membrane. Its usual manifestation is as an exophytic mass that grows into the pleural cavity compressing adjacent structures. The difference with malignant pleural mesothelioma does not only lie in its histological origin, but also in the epidemiological history and clinical course of the disease. We report a patient with a giant pleural mass of 11 years of evolution, the problems arising for the diagnosis, and finally the definitive surgical treatment.

Síndrome de Doege-Potter: hipoglicemia secundaria a tumor fibroso solitario de la pleura / Doege-Potter syndrome: hypoglycemia secondary to solitary fibrous tumor of the pleura. Report of one case

Doege-Potter syndrome is characterized for hypoglycemia associated with solitary pleural fibrous tumors. We report a 38-year-old woman with a history of weight loss, malaise and edema. After an episode of symptomatic hypoglycemia, she was admitted to the hospital, where she had new episodes of hypoglycemia. A Chest X ray and scan showed a right pleural tumor that was surgically excised. After surgery the episodes of hypoglycemia subsided. The pathological study of the tumor revealed a solitary fibrous pleural tumor. After 15 months of follow up, the patient is symptom free and without evidence of tumor relapse.

Giant solitary fibrous tumour of the pleura.

Solitary fibrous tumour of the pleura is a rare primary pleural neoplasm. These tumours are usually asymptomatic and are incidentally detected. Majority of these neoplasms are benign and surgical excision provides excellent results. With the widespread use of imaging and better diagnostic criteria, this tumour is likely to be detected more frequently. We encountered a patient with a giant solitary fibrous tumour of the pleura. In this report, we describe the case of a patient with a giant solitary fibrous tumour of the pleura, review the literature and present the details of management of this patient.

Tumor fibroso solitário da pleura: relato de caso / Solitary fibrous tumor of the pleura: a case report

Paciente de 75 anos, feminina, com dor torácica há 20 dias. Foi submetida à propedêutica radiológica, seguida de toracotomia com excisão de tumor fibroso solitário da pleura, neoplasia rara e de sintomatologia inespecífica, o que dificulta seu diagnóstico etiológico.

A 75-year-old female patient with chest pain for 20 days. She was conducted to radiological workup, followed by thoracotomy with excision of solitary fibrous tumor of the pleura, a rare cancer and with nonspecific symptoms, which makes its etiological diagnosis difficult.

Tumor fibroso solitário na pele: relato de caso / Solitary fibrous tumor of the skin: case report

O tumor fibroso solitário (TFS) é uma neoplasia benigna muito rara, derivada de células mesenquimais. Há relatos de casos deste tumor em diversos sítios pleurais ou extrapleurais, sendo a região cutânea e subcutânea localizações ainda mais incomuns. O prognóstico, na maioria dos casos, é bom, mas o comportamento pode ser agressivo. O diagnóstico clínico diferencial é importante na prática diária do cirurgião plástico, pois não raramente aparecem lesões tumorais dos componentes do revestimento cutâneo, com características semelhantes ao TFS - consistência fibro elástica e não aderência a planos profundos - que devem ser bem conhecidas e diferenciadas de neoplasias malignas, para que o tratamento adequado seja instituído.

Solitary fibrous tumor (TFS) is a very rare benign tumor, derived from mesenchymal cells. There are reports of cases of this tumor in different pleural or extra-pleural’s sites, and the skin is even a more unusual spot. The prognosis in most cases is good, but the behavior in few cases can be aggressive. The differential diagnosis is important in the daily practice of plastic surgeons. There are several lesions well known with similar characteristics to TFS in skin layers: fibro elastic consistency, not adhered to deep planes. Those lesions should be differentiated from malignant neoplasms, so that proper treatment is instituted.

Tumor fibroso solitario de la pleura: análisis de 41 casos / Solitary fibrous tumor of the pleura: analysis about 41 cases

Solitary fibrous tumor of the pleura (TFSP) is an uncommon and mostly benign neoplasm. It grows slowly and presents with scant symptoms. Recurrence after surgical excision has been described. The aim of this report is to show our experience in managing this disease. Clinical as well as radiological, surgical, histopathological and follow-up data were analyzed in a clinical series. Forty one cases of TFSP that underwent surgery at our institution between 1991 and 2004 were retrospectively analyzed; 27 were females and 14 males with a median age of 60.4 years old; 24.4% of the cases were asymptomatic. All tumors were surgically excised, in average they weighed 945 g and 36.5% of the lesions showed a pedicle. Most of the lesions were originated in the visceral pleura. Surgical morbidity was 12.1% and no mortality was recorded. Three tumor recurred. TFSP is usually a benign neoplasm but it can recur. Complete surgical excision is the treatment of choice of primary and recurrent tumors.

Introducción: El tumor fibroso solitario de la pleura (TFSP) es una neoplasia infrecuente, considerada benigna, de crecimiento lento y poco sintomática pero que puede recidivar tras la resección quirúrgica. El objetivo de este estudio es mostrar la experiencia de nuestro centro con el manejo de estos tumores. Pacientes y Método: Análisis retrospectivo de 41 pacientes con TFSP intervenidos en nuestro Servicio entre 1991 y 2004. Se analizan datos clínicos, radiológicos, quirúrgicos, morbimortalidad, recurrencia y seguimiento. Resultados: De los 41 pacientes con TFSP resecados, 27 eran mujeres y 14 hombres. Edad media 60,4 años. 24,4% de los pacientes fueron asintomáticos. Todos los pacientes fueron intervenidos quirúrgicamente y se realizó estudio histopatológico en todas las piezas. El peso promedio de los tumores fue de 954 g y 36,5% eran tumores pediculados, el resto de base ancha. La mayoría (75,6%) se originaban de la pleura visceral. No hubo mortalidad operatoria y la morbilidad alcanzó el 12,1%. Tres tumores recidivaron. Conclusiones: El TFSP es una neoplasia rara generalmente benigna, pero que puede recidivar. El tratamiento es la resección quirúrgica completa y las recidivas deben ser resecadas nuevamente.