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 tumour of the pleura.

Solitary fibrous tumour (SFT) of the pleura is a rare, usually benign primary tumour of the pleura. Spectrum of presentation can vary from an incidental finding on chest radiograph done for some other purpose, features of compression of surrounding structures to symptoms resulting from the tumour per se. We report a case of a female who presented with complaints of cough and chest pain in whom a diagnosis of SFT was confirmed on tru-cut biopsy and immunohistochemistry studies. The patient underwent thoracotomy and successful removal of the tumour.

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.

Solitary Fibrous Tumor of the Pleura Manifesting as an Air-Containing Cystic Mass: Radiologic and Histopathologic Correlation

Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm that typically presents as a well-defined lobular soft tissue mass commonly arising from the pleura. We report an extremely rare case of an SFT containing air arising from the right major fissure in a 58-year-old woman. Chest CT showed an ovoid air-containing cystic mass with an internal, homogeneously enhancing solid nodule. To our knowledge, this is the first case in the literature. The histopathologic findings were correlated with the radiologic findings, and the mechanism of air retention within the tumor is discussed.

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 maligno de extremidades / Malignant solitary fibrous tumour of the extremities

Tumor fibroso solitário extrapleural (TFS) é neoplasia mesenquimal rara, sendo que há menos de 40 casos descritos com localização em extremidades até o presente momento. Acomete preferencialmente pacientes na quinta década e não tem predileção por sexo. Relatamos um caso com os aspectos clínicos, radiológicos, histológicos e imunoistoquímicos característicos, discutindo a importância do diagnóstico diferencial com outros sarcomas.Pouco é conhecido sobre o comportamento biológico do TFS. Fatores de risco para menor sobrevida livre de metástases incluem: margens cirúrgicas comprometidas, tamanho maior que 10 cm e histologia maligna. Excisão completa é o tratamento recomendado.

Extrapleural solitary fibrous tumour (SFT) is a rare mesenchymal neoplasm, with less than 40 cases reported affecting the extremities so far. This tumour affects preferentially middle-aged patients (median: 50 years) and shows no predilection for sex. We report a case with typical clinical, radiologic, histopathologic and immunohistochemical features, discussing the importance of differential diagnosis of other sarcomas. Little is known about the biological behavior of SFT. Risk factors for shorter metastasis-free survival include impaired surgical margins, tumor size greater than 10 cm and malignant histology. Complete excision is the recommended treatment.

Haematologic paraneoplastic syndromes in pleural mesothelioma: A report of two cases and review of the literature.

Paraneoplastic syndromes are common in mesotheliomas but there is no report from India. Two cases of pleural mesothelioma with paraneoplastic haematologic syndromes, one with neutrophilic leukemoid reaction and the other with thrombocytosis, are presented in this report.

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.

Tumor fibroso de pleura / Solitary fibrous tumors of the pleura

Neste artigo apresentamos um caso de tumor fibroso solitário da pleura, em um homem de 49 anos, cujo diagnóstico foi feito em nosso hospital, anos após o surgimento dos primeiros sintomas e sinais na radiografia do tórax. Após descrição do caso, fizemos uma breve revisão sobre aspectos mais importantes deste raro tumor, visando auxiliar no diagnóstico diferencial das neoplasias torácicas.