Solitary fibrous tumor of the kidney: a report of two cases / Tumor fibroso solitário do rim: relato de dois casos

ABSTRACT Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm, composed of monotonous spindle cells, intermingled with collagen fibers. Kidney location is sporadic, with few reports in literature. We present two cases of 40- and 48-year-old males, one with incidental detection of a peripheral mass; another with hilar lesion perceived following investigation of hematuria. Both revealed a spindle-cell proliferation, without mitosis and necrosis. Immunohistochemical studies: positivity for CD34, CD99 and B-cell lymphoma 2 (Bcl-2). No sign of disease were evident nine and five months after surgery, respectively. SFT is mostly benign, but can show malignant behavior. Morphologic and immunohistochemical characteristics are essential for diagnosis.

RESUMEN Tumor fibroso solitario (TFS) es una neoplasia mesenquimal infrecuente de células fusiformes monótonas, separadas por bandas de colágeno. Localización renal es poco habitual, con escasos casos descriptos. Reportamos dos casos de pacientes masculinos, de 40 e 48 anos, el uno con detección incidental de masaperiférica; el otro con lesión hiliar descubierta en la investigación de hematuria. Los dos revelaronproliferación fusocelular sin necrosis ni mitosis. Inmunohistoquímica: positividad para CD34, CD99 y linfoma de células B2 (Bcl-2). No hay evidencia de recidiva a los nueve y cinco meses de la cirugía, respectivamente. TFS es, por lo general, benigno, pero puede revelar comportamiento agresivo. Características morfológicas y inmunohistoquímicas son esencialespara el diagnóstico.

RESUMO Tumor fibroso solitário (TFS) é uma neoplasia mesenquimatosa rara composta por células fusiformes monótonas, intercaladas por fibras de colágeno. Localização renal é incomum, com escassos casos descritos. Relatamos dois casos do gênero masculino, com 40 e 48 anos, um com detecção incidental de massa periférica; outro com lesão hilar descoberta na sequência de investigação por hematúria. Ambos revelaram proliferação fusocelular sem mitoses nem necrose. Imuno-histoquímica: positividade para CD34, CD99 e linfoma de células B2 (Bcl-2). Não há evidência de recidiva nove e cinco meses após cirurgia, respetivamente. TFS é maioritariamente benigno, mas pode revelar comportamento agressivo. Características morfológicas e imuno-histoquímicas são essenciais para o diagnóstico.

Tumor fibroso solitario pleural como causa de disnea en un paciente de 53 años / Pleural fibrous solitary tumor as cause of dyspnea in a patient 53 years old

El tumor fbroso solitario pleural es una neoplasia infrecuente originada en las células mesoteliales de la superfcie pleural con una incidencia calculada en alrededor de 2.8 casos por 100.000 pacientes al año [1]; además, representa el 8% de las neoplasias benignas del tórax y 10% de los tumores pleurales [2]. El curso clínico de la mayoría de estos tumores es benigno, raramente pueden recurrir localmente o dar metástasis. Las recurrencias pueden ser tardías, con un período de latencia de hasta 30 años [3], la mayoría de los casos se localizan en la pleura visceral o parietal; además, pueden aparecer en otras áreas tales como peritoneo, mediastino, meninges, pulmón, tiroides, parótida, órbita, nariz y fosas nasales. Clínicamente este tumor es asintomático y suele diagnosticarse de manera casual en una radiografía de tórax. Los síntomas ocasionalmente descritos son tos, disnea, dolor torácico y derrame pleural, y entre las manifestaciones sistémicas fguran artralgias, acropaquias e hipoglicemia

The pleural solitary fibrous tumor is an infrequent neoplasm originated in the mesothelial cells of the pleural surface with an incidence calculated at around 2.8 cases per 100,000 patients per year [1]; In addition, it represents 8% of benign neoplasms of the thorax and 10% of pleural tumors [2]. The clinical course of most of these tumors It is benign, rarely they can recur locally or metastasize. The recurrences may be late, with a latency period of up to 30 years [3], most cases are located in the visceral pleura or parietal; In addition, they may appear in other areas such as peritoneum, mediastinum, meninges, lung, thyroid, parotid, orbit, nose and pits Nasal Clinically, this tumor is asymptomatic and is usually diagnosed casual way on a chest x-ray. The symptoms occasionally described are cough, dyspnea, chest pain and pleural effusion, and between Systemic manifestations include arthralgias, acropachias and hypoglycemia

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 localizado de pleura: reporte de un caso / Localized fibrous tumor of pleura: a case report

Fundamento: los tumores fibrosos localizados de pleura son raros, de etiología incierta; la mayoría de los pacientes con esta enfermedad sobrepasan la quinta década de vida y más de la mitad están asintomáticos al diagnóstico, principalmente cuando los tumores son pequeños. Objetivo: describir el caso de una paciente con tumor fibroso localizado de pleura que, por su edad, sintomatología y por las características histológicas del tumor, representa una situación clínica infrecuente, con dificultades para el diagnóstico. Presentación de caso: mujer saludable, de 39 años, con dolor tóraco-lumbar persistente, de un año de evolución; en radiografía de tórax se visualizó imagen retro cardíaca izquierda corroborada por tomografía computarizada. Se realizó exéresis total de una masa de 4,8 cm, pedunculada, con origen en la pleura visceral, cuyo diagnóstico histopatológico fue un tumor fibroso localizado de pleura con características quísticas. Después de varios meses de seguimiento la paciente permanecía saludable y sin signos de recurrencia. Conclusiones: este caso constituye una forma infrecuente de presentación del tumor fibroso localizado de pleura por tratarse de una paciente menor de 40 años, con una sintomatología no frecuente y por el comportamiento benigno que ha tenido el tumor aunque presentó características quísticas.

Background: localized fibrous tumors of the pleura are rare, with uncertain etiology; most of the patients with this disease exceed the fifth decade of life and more than a half are without symptoms at the diagnosis, mainly when tumors are small. Objective: to describe a case of a female patient with a diagnosis of a localized fibrous tumor of pleura in which due to her age, the symptomatology and histological features of the tumor, it represents an infrequent clinical situation with difficulties for the diagnosis. Case presentation: a 39-year-old healthy woman with one year history of persistent thoracic lumbar pain; in chest radiography a left retrocardiac image was observed and it was corroborated by chest CT scan. Total exeresis was performed in a 4.8 cm mass, pedunculated, originating from the visceral pleura, whose histopathological diagnosis was a localized fibrous tumor of the pleura with cystic features. After several months of follow-up, the patient remained healthy and did not show recurrent signs. Conclusions: this case is an infrequent presentation of a localized fibrous tumor of the pleura for having been diagnosed in a patient under 40 years old, with an infrequent symptomatology and because the tumor has shown a benign behavior even with cystic features.

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.