Mesotelioma pleural maligno. Presentación de un caso y revisión de la literatura / Malignant Pleural Mesothelioma. Case Report and Literature Review

RESUMEN Los mesoteliomas pleurales son tumores primarios de células mesoteliales. Recubren la cavidad pleural, se encuentran estrechamente relacionados con la exposición al asbesto. Tienen una baja incidencia y mal pronóstico. Se presenta el caso de un paciente de 46 años, de color de piel blanca, que acudió al Hospital General Universitario Dr. Gustavo Aldereguía Lima de Cienfuegos por presentar dolor en la espalda y hombros, fiebre y pérdida de peso. En el examen físico se constató murmullo vesicular abolido en 1/3 inferior del pulmón derecho. La radiografía de tórax y la tomografía evidenciaron derrame pleural derecho de mediana a gran cuantía y engrosamiento pleural. Los estudios histológicos indicaron que la lesión era compatible con un mesotelioma pleural de variante epitelioide. El paciente se mantiene con tratamiento citostático y seguimiento en consulta. El objetivo del presente trabajo es describir el caso de un paciente con mesotelioma pleural maligno, una enfermedad tumoral rara, ingresado en el Servicio de Medicina Interna del Hospital General Universitario Dr. Gustavo Aldereguía Lima de Cienfuegos.

ABSTRACT Pleural mesotheliomas are primary mesothelial cell tumors. Lining the pleural cavity, they are closely related to asbestos exposure. They have a low incidence and poor prognosis. A 46-years-old patient, white-skinned, who attended the Dr. Gustavo Aldereguía Lima University General Hospital in Cienfuegos due to pain in the back and shoulders, fever, and weight loss is presented. The physical examination revealed an abolished vesicular murmur in the lower 1/3 of the right lung. The chest X-ray and CT scan showed a medium to large right pleural effusion and pleural thickening. Histological studies indicated that the lesion was compatible with epithelioid variant pleural mesothelioma. The patient is maintained with cytostatic treatment and follow-up in consultation. The objective of this study is to describe the case of a patient with malignant pleural mesothelioma, admitted to the Internal Medicine Service of the Dr. Gustavo Aldereguía Lima General University Hospital of Cienfuegos.

Síndrome de Doege Potter / Doege-Potter syndrome

Resumen El síndrome de Doege Potter es un síndrome caracterizado por hipoglucemia severa asociada a tumor fibroso de la pleura. Presentamos el caso de una paciente de 67 años con antecedente por biopsia de tumor fibroso de pleura, quien seis meses después de este diagnóstico presenta episodios de alteración del estado de conciencia con desorientación y documentación de hipoglucemia con triada de Whipple presente. Se realiza test de ayuno el cual es positivo para hipoglucemia no hiperinsulinémica y dado sus antecedentes, se hace el diagnóstico de un síndrome de Doege Potter. Se realiza manejo quirúrgico con resección total de masa tumoral con posterior resolución de la hipoglucemia.(Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1503).

Abstract Doege-Potter syndrome is characterized by severe hypoglycemia associated with a fibrous tumor of the pleura. We present the case of a 67-year-old patient with a history of a fibrous tumor of the pleura, diagnosed through biopsy, who six months after this diagnosis experienced episodes of altered consciousness with disorientation, and documented hypoglycemia with Whipple's triad. A fasting test was positive for non-hyperinsulinemic hypoglycemia and, given his history, he was diagnosed with Doege-Potter syndrome. He was treated surgically through total removal of the tumor mass, with subsequent resolution of the symptoms.(Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1503).

Tumor fibroso solitario gigante de pleura en paciente joven / Giant Solitary Fibrous Tumor of the Pleura in Young Patient / Tumor fibroso solitário gigante de pleura em paciente jovem

Resumen Introducción : el tumor fibroso solitario de pleura (TFSP) es una patología poco frecuente, corresponde a menos del 5 % de los tumores primarios de pleura, siendo aún más raros los tumores mayores de 15 cm y aquellos en pacientes menores de 35 años, ya que el pico de incidencia es entre los 60 a 70 años con un solo caso reportado en Colombia. Presentación del caso : paciente femenina de 33 años, sin exposición a asbesto o cigarrillo. Consultó por cuadro de disnea de moderados esfuerzos y dolor dorsal derecho. La tomografía axial computarizada (TAC) contrastada de tórax evidenció una gran masa sólida de contornos lobulados que comprometía el 70 % del hemitórax derecho de origen extrapulmonar y que comprimía la aurícula derecha, la inmunohistoquímica de la biopsia percutánea clasificó la lesión como tumor fibroso solitario. Fue llevada a toracotomía posterolateral derecha extendida, donde se encontró un tumor dependiente de la pleura parietal derecha resecado en su totalidad. Histopatología e inmunohistoquímica del producto de resección quirúrgica, compatibles con tumor fibroso solitario de pleura, tamaño 30 x 23 x 16 cm sin características de malignidad. La evolución posoperatoria fue satisfactoria, con un egreso hospitalario temprano. Discusión : en una paciente joven, el TFSP gigante es inusual, posterior a su diagnóstico, y a pesar de tener algunas características que sugerían malignidad: tamaño y origen en la pleura parietal, la resección quirúrgica fue el tratamiento indicado para este tumor, con posterior confirmación histopatológica compatible con tumor benigno.

Abstract Introduction : The solitary fibrous tumor of the pleura (SFTP) is an uncommon pathology. It corresponds to less than 5 % of the primary tumors of the pleura. Even much rare is to find one solitary fibrous tumor, of more than 15 cm in patients younger than 35 years since the incidence peak is between 60 to 70 years. There has been only one case reported in Colombia. Case presentation : The subject of study was a 33-year-old female patient, no exposure to asbestos or cigarette. The chief complaints of the patient were mild dyspnea and right dorsal pain. Thoracic contrast-enhanced computerized tomography showed a large solid mass of lobulated contours that compromised 70 % of the right hemithorax, of extrapulmonary origin compressing the right atrium. Immunohistochemistry of the percutaneous biopsy demonstrated a solitary fibrous tumor. The patient underwent an extended right posterolateral thoracotomy. We found and completely resected a tumor hanging on the right parietal pleura. Histopathology and immunohistochemistry of the product of the surgical resection were compatible with a solitary fibrous tumor of the pleura, 30 x 23 x 16cm in size, and no malignancy. The evolution after surgery was satisfactory with an early hospital discharge. Discussion : In a young patient, a giant TFSP is unusual. After diagnosis and despite having some characteristics that suggested malignancy: its size and origin in the parietal pleura, surgical resection was the appropriate procedure for this tumor, with subsequent histopathological confirmation compatible with a benign tumor.

Resumo Introdução : o tumor fibroso solitário de pleura (TFSP) é uma patologia pouco frequente, corresponde a menos do 5 % dos tumores primários de pleura, sendo ainda mais estranhos os tumores maiores de 15 cm e aqueles em pacientes menores de 35 anos, pois o pico de incidência é entre os 60 a 70 anos com um só caso reportado na Colômbia. Apresentação de caso : paciente feminina de 33 anos, sem exposição a asbesto ou cigarro. Consultou por quadro de dispneia de moderados esforços e dor dorsal direita. A tomografia axial computadorizada (TAC) contrastada de tórax evidenciou uma grande massa sólida de contornos lobulados que comprometia o 70 % do hemitórax direito de origem extrapulmonar e que comprimia a aurícula direita, imuno-histoquímica da biopsia percutânea classificou a lesão como tumor fibroso solitário. Foi levada à toracotomia posterolateral direita estendida, encontrando um tumor dependente da pleura parietal direita ressecado em sua totalidade. Histopatologia e imuno-histoquímica do produto de ressecção cirúrgica, compatíveis com tumor fibroso solitário de pleura, tamanho 30 x 23 x 16cm sem características de malignidade. A evolução pós-cirúrgica foi satisfatória, conseguindo um egresso hospitalar precoce. Discussão : em uma paciente jovem, o TFSP gigante é inusual, posterior a seu diagnóstico e apesar de ter algumas características que sugeriam malignidade; tamanho e origem na pleura parietal, a ressecção cirúrgica foi o tratamento indicado para este tumor, com posterior confirmação hispatológica compatível com tumor benigno.

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. .

Primary pleural liposarcoma, pleomorphic variant.

Primary pleural liposarcoma (PPL) is a rare tumor derived from primitive mesenchymal tissue. We report a case of a 49-year-old female patient complaining of thoracic pain and dyspnea for 3 months. The chest X-ray showed a left basal opacity of lobulated contours and the thoracic computer tomography (CT) scan revealed a left pleural collection/mass, of 18 HU density and passive pulmonary atelectasis. The patient was taken to surgery and the cytologic examination of the gelatinous mass found in the procedure confirmed the diagnosis of a pleomorphic variant of pleural liposarcoma. We emphasise in the importance of careful inspection of the origin of the tumor in the diagnostic images to allow accurate diagnosis.

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.

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.