Ganglioneuroma de mediastino posterior: a propósito de un caso

RESUMEN Los tumores de origen neuroectodérmico son la principal causa de tumores de mediastino. De ellos el ganglioneuroma es el de mayor benignidad y el menos frecuente, con una incidencia de 1 en 100,000 niños. Ocurre predominantemente en la infancia con una media de presentación a los siete años. La mayor parte de estos tumores cursan asintomáticos y son diagnosticados de manera incidental. El 43 % de ellos tienen manifestación intratorácica y están localizados en los ganglios simpáticos del mediastino posterior. Los medios de diagnóstico por imágenes aportan ventajas para mostrar la localización, tamaño y extensión del tumor. Estos exámenes resultan de gran beneficio para el cirujano. El pronóstico es generalmente favorable, lo que constituye signo de buen pronóstico, la aparición posterior a los cinco años de edad y las localizaciones en el mediastino posterior. Se presenta el caso de una paciente a la que se le realizó toracoscopia con resección total del tumor, con evolución favorable durante su seguimiento. A esta paciente se le diagnosticó un ganglioneuroma en mediastino posterior. Se realizó una revisión bibliográfica a propósito de este caso. Se presenta este reporte porque el ganglioneuroma es un tumor benigno poco frecuente en edad pediátrica.

ABSTRACT Neuroectodermal tumors are the main cause of mediastinal tumors. Of these, the ganglioneuroma is the most benign and the least frequent, with an incidence of 1 in 100,000 children. It occurs predominantly in childhood with a mean presentation at age seven. Most of these tumors are asymptomatic and diagnosed incidentally. The 43 % of them showing intrathoracic manifestation and located in the sympathetic ganglia of the posterior mediastinum. Imaging studies provide advantages for the location, size and extent of the tumor. These tests are of great benefit to the surgeon. The prognosis is generally favorable, which is a sign of good prognosis when it appears after five years of age and when it is located in the posterior mediastinum. The case of a patient who underwent thoracoscopy with total resection of the tumor, with a favorable evolution during her follow-up is presented. This patient was diagnosed with a ganglioneuroma in the posterior mediastinum. A literature review was carried out regarding this case. This report is presented because ganglioneuroma is a rare benign tumor in children.

Teratoma mediastinal con áreas de malignidad, reporte de un caso y discusión / Mediastinal Teratoma with Malignant Areas: Case Report and Discussion

Resumen Varios tipos de tumores pueden surgir en el mediastino anterior, de los cuales los tumores de células germinales constituyen el 10- 15%, y el más frecuente es el teratoma maduro. El hallazgo de un componente maligno en un teratoma maduro es raro, habiéndose descrito pocos casos de malignidad en forma de carcinoma escamoso, adenocarcinoma, sarcoma o tumores neuroendocrinos. Presentamos el caso de una mujer joven con diagnóstico de teratoma maduro y desarrollo de adenocarcinoma en la pared, lo que confiere un pronóstico desfavorable, con opciones de tratamiento mal definidas dada la excepcionalidad de la enfermedad. Este caso clínico destaca que se requiere un muestreo histopatológico cuidadoso de las áreas sólidas en un teratoma, incluso en pacientes jóvenes cuyas lesiones son más pequeñas. Aunque existen recomendaciones a favor de la quimioterapia, se basan en series de un número limitado de pacientes. La resección completa de la neoplasia y el seguimiento multidisciplinario serán de relevancia para el control de las recidivas locales y a distancia.

Abstract Several types of tumors may occur in the anterior mediastinum, of which germ cell tumors constitute 10-15%, the most frequent being the mature teratoma. The finding of a malignant component in mature teratoma is rare, and few cases of malignancies such as squamous carcinoma, adenocarcinoma, sarcoma, or neuroendocrine tumors have been described. We present the case of a young woman diagnosed with mature teratoma and development of adenocarcinoma within tumor wall, conferring an unfavorable prognosis, with poorly defined treatment options given the exceptional a mature of the disease. This clinical case highlights the fact that careful histopathological sampling of solid areas is required in a teratoma, even in young patients whose lesions are smaller. Although there are recommendations in favor of chemotherapy, they are based on series of a limited number of patients. Complete resection of the neoplasm and multidisciplinary follow-up would be of relevance for the control of local and distant recurrences.

Survival Outcomes of Patients With Mediastinal Germ Cell Tumors: Experience of a Cancer Center in South America.

PURPOSE: Mediastinal germ cell tumors (GCT) are rare neoplasms associated with poor survival prognosis. Due to their low incidence, limited information is available about this disease in South America. The objective of this study is to report the clinical characteristics and outcomes of patients with mediastinal GCT in a cancer center in Colombia. MATERIALS AND METHODS: We conducted a retrospective analysis of patients with mediastinal GCT treated at the National Cancer Institute at Bogota (Colombia) between 2008 and 2020. Survival curves were presented using the Kaplan-Meier method. Chi-square and Cox proportional hazard model tests were used for data analysis. RESULTS: Sixty-one patients were included in the study. Of them, 60 were male and 51 (83.6%) of whom had non-seminomatous germ cell tumors (NSGCT). Twenty-nine patients (47.5%) presented with superior vena cava syndrome, and 18 (29.5%) patients had extrapulmonary metastatic involvement. The three-year overall survival (OS) of NSGCT patients was 26%. The 3-year OS of NSGCT patients who underwent surgical resection of residual mediastinal mass after chemotherapy was 59%. Non-surgical management after first-line chemotherapy was associated with a worse survival prognosis in NSGCT patients (p = 0.002). Ten patients with mediastinal seminomatous germ cell tumors (SCGT) achieved a 3-year OS of 100%. CONCLUSION: Mediastinal NSGCT had poor outcomes. Surgery of the residual mass after first-line chemotherapy seems to improve the outcome of NSGCT patients. Advanced disease at presentation may reflect inadequate access to reference cancer centers in Colombia and potentially explain poor survival outcomes in this cohort. On the other hand, mediastinal SCGT is a biologically different disease; most patients will achieve disease remission and long-term survival with first-line chemotherapy.

Bloqueo plano erector de la espina en toracotomía pediátrica / Pediatric thoracotomy in erector spinae plane block

Erector spinae plane block (ESP) is a regional anesthesia technique consisting of blocking the interfascial plane, where local anesthetic (LA) is injected at the site where the dorsal branch of the spinal nerve emerges. There are various publications on the application of the block (EPS) in the adult population, however there are few reports of the use of this block in pediatric surgery. We present the first case report of an infant under 9 months of age with the presence of a tumor in the anterior mediastinum, who underwent a resection through the 6th intercostal space, previous blockage of the erector spinae under ultrasound vision. The patient did not present adverse effects and was discharged on the fourth postoperative day.

El bloqueo del plano erector de la espina (ESP) es una técnica de anestesia regional consistente en bloquear el plano interfascial, donde se inyecta anestésico local (LA) en el sitio donde emerge la rama dorsal del nervio espinal. Existen diversas publicaciones sobre la aplicación del bloqueo (EPS) en población adulta, sin embargo, hay pocos reportes del uso de este bloqueo en cirugía pediátrica. Presentamos el primer reporte de caso de una lactante menor de 9 meses de edad con presencia de tumor en mediastino anterior, al cual se le hizo una resección a través del 6° espacio intercostal, previo bloqueo del erector de la espina bajo visión ecográfica. La paciente no presentó efectos adversos y fue dada de alta al cuarto día posoperatorio.

Tumor mediastinal de células germinales en un paciente con melanoptisis / Mediastinal germ cell tumor in a patient with melanoptysis / Tumor de células germinativas mediastinais em paciente com melanoptise

Figuras A Y B: Radiografía de Tórax Anteroposterior (AP) y Lateral. Presencia de gran lesión al parecer del mediastino medio (Flechas blancas), con diámetros de 11 x 15 x 12 cm, de densidad homogénea, con efecto de masa y desviación de todas las estructuras (Flechas negras) y compresión sobre el lóbulo superior derecho (*), sin calcificaciones en su interior. Figura C. Tomografía computarizada de tórax con contraste corte coronal. En la región central del mediastino anterior, se identifica una masa hipodensa, heterogénea, con densidad de predominio de tejido blando, con algunas imágenes de densidad cálcica en su interior, de 14.3 x 10.2 x 12.6 (Línea amarilla) cm, que se encuentra desplazando la aorta ascendente, con trayecto tortuoso (Flecha azul), la flecha roja señala el tronco braquiocefálico. Figura D. Tomografía computarizada de tórax corte axial en ventana mediastinal. Masa hipodensa, heterogénea, con densidad de predominio de tejido blando, con algunas imágenes de densidad cálcica en su interior, la cual se encuentra desplazando y ocupando gran parte de la porción anterior del hemitórax derecho. Así mismo se encuentra desplazando estructuras mediastinales como el tronco de la pulmonar (Flecha azul), esta masa se encuentra generando atelectasia pasiva del lóbulo medio derecho (Flecha roja). Cómo citar. López-Salazar JP, Delgado-Serrano J. Tumor mediastinal de células germinales en un paciente con melanoptisis. MedUNAB. 2020;23(2): 191-194. doi:10.29375/01237047.3748

Figures A and B: Anteroposterior (AP) and Lateral Chest X-ray. Presence of a large mass, apparently from the middle mediastinum (white arrows), diameters of 11 x 15 x 12 cm, homogeneous density, with the mass effect on other mediastinal structures (black arrows) and compression on the right upper lobe (*), without inner calcifications. Figure C. Chest Computed Tomography, coronal section. In the anterior mediastinum it shows a hypodense, heterogeneous mass, with predominance of soft tissue density, with some calcifications inside, diameters of 14.3 x 10.2 x 12.6 (yellow line) cm, displacing the ascending aorta (blue arrow), the red arrow indicates the brachiocephalic trunk. Figure D. Computed tomography of the chest, axial section. Hypodense, heterogeneous mass, with predominance of soft tissue density, with some calcifications inside, which occupying a large parto f the anterior right hemithorax. It is also displacing mediastinal structures such as the trunk of the pulmonary artery (blue arrow), this mass is generating passive atelectasis of the right middle lobe (red arrow). Cómo citar. López-Salazar JP, Delgado-Serrano J. Tumor mediastinal de células germinales en un paciente con melanoptisis. MedUNAB. 2020;23(2): 191-194. doi:10.29375/01237047.3748

Figuras A e B: Radiografia de tórax (anteroposterior (AP) e lateral). Presença de grande massa, aparentemente do mediastino médio (Setas brancas), diâmetros de 11 x 15 x 12 cm, densidade homogênea, com efeito de massa em outras estruturas mediastinais (setas pretas) e compressão no lobo superior direito (*), sem calcificações internas. Figura C. Tomografia computadorizada de tórax, corte coronal. No mediastino anterior, mostra uma massa hipodensa e heterogênea, com predominância da densidade de partes moles, com algumas calcificações internas, diâmetros de 14,3 x 10,2 x 12,6 (Linha amarela) cm, deslocando a aorta ascendente (seta azul), a seta vermelha indica o tronco braquiocefálico. Figura D. Tomografia computadorizada de tórax, corte axial. Massa hipodensa, heterogênea, com predominância da densidade de partes moles, com algunas calcificações internas, que ocupam grande parte do hemitórax anterior direito. Também está deslocando estruturas mediastinais, como o tronco da artéria pulmonar (Seta azul), essa massa está gerando atelectasia passiva do lobo médio direito (Sseta vermelha). Cómo citar. López-Salazar JP, Delgado-Serrano J. Tumor mediastinal de células germinales en un paciente con melanoptisis. MedUNAB. 2020;23(2): 191-194. doi:10.29375/01237047.3748

Sarcoma cardíaco metastático a miembros inferiores / Cardiac sarcoma with metastasis to the lower limbs / Sarcoma cardíaco metastático dos membros inferiores

Introducción. Las neoplasias de corazón son patologías infrecuentes con tasas de incidencias en reportes de autopsias menores al 0.02 %. El 75 % de esos tumores son de comportamiento benigno y solo el 25 % restante se considera cáncer. Aunque se presentan en ambos sexos, la relación hombres mujeres es de 2.5:1 respectivamente. Presentación del caso. Reportamos el caso de un paciente de 41 años a quien se diagnosticó con un sarcoma primario de corazón, en atrio izquierdo, con inmunohistoquímica compatible con sarcoma indiferenciado con metástasis a miembros inferiores, que debutó con deterioro de la clase funcional y pérdida de peso involuntaria, lo cual lo llevó a consultar al médico. Discusión. Los sarcomas indiferenciados constituyen una patología poco frecuente en la literatura médica, encontrando solo unos pocos reportes de casos que comparten datos relacionados con el sitio de localización de la lesión y los síntomas clínicos de los pacientes. Sin embargo, aquí se pone de manifiesto un extraño caso de metástasis a miembros inferiores, el cual fue considerado luego de descartar la presencia de un foramen oval permeable o lesiones pulmonares que hicieran pensar en una secuencia de migración tumoral diferente. Conclusiones. Se trata de una patología compleja con pobre pronóstico a largo plazo, la cual requiere mayor investigación y tratamiento multifactorial con equipos multidisciplinarios para mejorar la calidad de vida de los pacientes.

Introduction. Neoplasms of the heart are infrequent pathologies with incidence rates in autopsy reports less than 0.02%. 75 % of these tumors are benign and only the remaining 25 % are considered cancerous. Although they occur in both sexes, the male to female ratio is 2.5: 1 respectively. Case report. We report the case of a 41-year-old patient who was diagnosed with a primary heart sarcoma, in the left atrium, with immunohistochemistry compatible with undifferentiated sarcoma with metastases to the lower limbs, who debuted with impairment of functional class and loss of involuntary weight which led him to consult the doctor. Discussion. Undifferentiated sarcomas constitute a rare pathology in the medical literature, finding only a few cases reports that share data, such as the location of the lesion and the clinical symptoms of the patients. However, this case reveals a strange case of metastasis to lower limbs, which was considered, after ruling out the presence of a patent foramen ovale or lung lesions that suggested a different tumor migration sequence. Conclusions. This is a complex pathology with a poor long-term prognosis that requires more re- search and multifactorial treatment with multidisciplinary teams to improve the quality of life of patients

Introdução. As neoplasias do coração são patologias pouco frequentes, com taxas de incidência em relatórios de autópsia inferiores a 0,02%. 75% desses tumores são benignos e apenas 25% restantes são considerados câncer. Embora ocorram em ambos os sexos, a proporção entre homens e mulheres é de 2,5: 1 respectivamente. Apresentação do caso. Relatamos o caso de um paciente de 41 anos de idade com diagnóstico de sarcoma primário do coração, no átrio esquerdo, com imuno-histoquímica compatível com sarcoma indiferenciado com metástase nos membros inferiores, que começou com deterioração da classe funcional e perda involuntária de peso, o que o levou a consultar o médico. Discussão. Os sarcomas indiferenciados constituem uma patologia rara na literatura médica, encontrando apenas alguns relatos de casos que compartilham dados relacionados ao local da localização da lesão e aos sintomas clínicos dos pacientes. No entanto, aqui é revelado um caso estranho de metástase nos membros inferiores, que foi considerado após descartar a presença de forame oval permeável ou lesões pulmonares que sugerem uma sequência de migração tumoral diferente. Conclusões. É uma patologia complexa, com mau prognóstico a longo prazo, que requer mais investigação e tratamento multifatorial com equipes multidisciplinares para melhorar a qualidade de vida dos pacientes.

EBUS-TBNA versus surgical mediastinoscopy for mediastinal lymph node staging in potentially operable non-small cell lung cancer: a systematic review and meta-analysis / EBUS-TBNA versus mediastinoscopia cirúrgica no estadiamento linfonodal mediastinal do câncer de pulmão de células não pequenas potencialmente operável: revisão sistemática e meta-análise

ABSTRACT Objective: Lung cancer (LC) is one of the leading causes of death worldwide. Accurate mediastinal staging is mandatory in order to assess prognosis and to select patients for surgical treatment. EBUS-TBNA is a minimally invasive procedure that allows sampling of mediastinal lymph nodes (LNs). Some studies have suggested that EBUS-TBNA is preferable to surgical mediastinoscopy for mediastinal staging of LC. The objective of this systematic review and meta-analysis was to compare EBUS-TBNA and mediastinoscopy in terms of their effectiveness for mediastinal LN staging in potentially operable non-small cell lung cancer (NSCLC). Methods: This was a systematic review and meta-analysis, in which we searched various databases. We included studies comparing the accuracy of EBUS-TBNA with that of mediastinoscopy for mediastinal LN staging in patients with NSCLC. In the meta-analysis, we calculated sensitivity, specificity, positive likelihood ratios, and negative likelihood ratios. We also analyzed the risk difference for the reported complications associated with each procedure. Results: The search identified 4,201 articles, 5 of which (with a combined total of 532 patients) were selected for inclusion in the meta-analysis. There were no statistically significant differences between EBUS-TBNA and mediastinoscopy in terms of the sensitivity (81% vs. 75%), specificity (100% for both), positive likelihood ratio (101.03 vs. 95.70), or negative likelihood ratio (0.21 vs. 0.23). The area under the summary ROC curve was 0.9881 and 0.9895 for EBUS-TBNA and mediastinoscopy, respectively. Although the number of complications was higher for mediastinoscopy, the difference was not significant (risk difference: −0.03; 95% CI: −0.07 to 0.01; I2 = 76%). Conclusions: EBUS-TBNA and mediastinoscopy produced similar results for mediastinal staging of NSCLC. EBUS-TBNA can be the procedure of first choice for LN staging in patients with NSCLC.

RESUMO Objetivo: O câncer de pulmão (CP) é uma das principais causas de morte no mundo. Um estadiamento mediastinal preciso é obrigatório para avaliação do prognóstico e seleção de pacientes para tratamento cirúrgico. EBUS-TBNA é um procedimento minimamente invasivo que permite a amostragem de linfonodos mediastinais. Alguns estudos sugerem que a EBUS-TBNA é preferível que a mediastinoscopia cirúrgica no estadiamento mediastinal do CP. O objetivo desta revisão sistemática e meta-análise foi comparar a eficácia da EBUS-TBNA e da mediastinoscopia no estadiamento linfonodal mediastinal do câncer de pulmão de células não pequenas (CPCNP) potencialmente operável. Métodos: Foram pesquisados diversos bancos de dados. Estudos comparando a precisão da EBUS-TBNA e da mediastinoscopia no estadiamento linfonodal mediastinal em pacientes com CPCNP foram incluídos. Na meta-análise, foram calculadas sensibilidade e especificidade, bem como razões de verossimilhança positiva e negativa. A diferença de risco de complicações relatadas para cada procedimento também foi analisada. Resultados: A pesquisa identificou 4.201 artigos, dos quais 5 foram selecionados para a meta-análise (total combinado de 532 pacientes). Não houve diferenças estatisticamente significativas entre EBUS-TBNA e mediastinoscopia: sensibilidade (81% vs. 75%), especificidade (100% para ambas), razão de verossimilhança positiva (101,03 vs. 95,70) e razão de verossimilhança negativa (0,21 vs. 0,23). A área sob a curva summary ROC para EBUS-TBNA e para mediastinoscopia foi de 0,9881 e 0,9895, respectivamente. Embora o número de complicações tenha sido maior para mediastinoscopia, não foi encontrada diferença significativa (diferença de risco: −0,03; IC95%: −0,07 to 0,01; I2 = 76%). Conclusões: EBUS-TBNA e mediastinoscopia apresentaram resultados semelhantes no estadiamento mediastinal do CPCNP. EBUS-TBNA pode ser o procedimento de primeira escolha no estadiamento linfonodal em pacientes com CPCNP.

Consideraciones en torno a los tumores del mediastino / Considerations about mediastinal tumours

Introducción. El desarrollo de las imágenes diagnósticas hizo posible el diagnóstico topográfico de los tumores de mediastino; sin embargo, aún con los avances actuales de la cirugía, la anestesiología y la reanimación, el acceso quirúrgico a ese espacio medio de la caja torácica continúa siendo el gran reto que enfrentan los cirujanos torácicos. Objetivo. Profundizar en los diferentes aspectos cognoscitivos actualizados sobre los tumores mediastinales.Métodos. Búsqueda documental digital en bases de datos: Scielo, Lilacs, Web of Science, PubMed, realizada en enero de 2019, de publicaciones sobre el tema en la presente centuria. Resultados. Las neoplasias mediastinales son poco frecuentes, pueden aparecer a cualquier edad, aunque son más frecuentes entre la tercera y quinta décadas, y la mayoría se descubren incidentalmente en una radiografía de tórax de rutina en pacientes asintomáticos. Los tumores malignos del mediastino son poco frecuentes, pero los benignos constituyen un desafío diagnóstico para radiólogos y patólogos. En las neoplasias malignas se identifica una amplia gama de variedades histológicas, atribuibles a las características del órgano afectado. Conclusiones. El diagnóstico definitivo generalmente se establece mediante el estudio histopatológico posquirúrgico, si bien la tomografía computarizada, asociada o no a la biopsia percutánea, es el estándar de oro para el diagnóstico preoperatorio. La selección de la vía de entrada al tórax, así como el procedimiento quirúrgico, están condicionados por la localización y el tamaño del tumor en el mediastino

Introduction. The development of diagnostic images made the topographic diagnosis of mediastinal tumors possible; however, even with current advances in surgery, anesthesiology, and resuscitation, surgical access to this mid-rib cage space continues to be the great challenge faced by chest surgeons.Objective. To deepen in the different updated cognitive aspects about mediastinal tumors.Methods. Digital documentary search in databases: Scielo, Lilacs, Web of Science, and PubMed, performed in January 2019, of publications on the subject in the present century.Results. Mediastinal neoplasms are rare, they can appear at any age, although they are most common between the third and fifth decades, and most are discovered incidentally on a routine chest radiograph in asymptomatic patients. Malignant tumors of the mediastinum are rare, but benign tumors pose a diagnostic challenge for radiologists and pathologists. In malignant neoplasms, a wide range of histological varieties is identified, attributable to the characteristics of the affected organ.Conclusions. The definitive diagnosis is generally established by postoperative histopathological study, although computed tomography, associated or not with percutaneous biopsy, is the gold standard for preoperative diagnosis. The selection of the entry route to the thorax, as well as the surgical procedure, are conditioned by the location and size of the tumor in the mediastinum

Teratocarcinoma gigante de mediastino anterosuperior / Giant teratocarcinoma of anterosuperior mediastinum

RESUMEN Los tumores de células germinales no son tan frecuentes, dentro de ellos se encuentra el teratoma como el más usual en ubicación mediastinal, es más habitual en hombres jóvenes cuando es maligno (teratocarcinoma), con una relación por género de 13,5:1, y una edad promedio de 26 años. Se describe la evolución de un hombre de 19 años de edad con un tumor mediastinal, con metástasis pulmonares e infiltración intracardíaca, resecado satisfactoriamente, cuyos síntomas más frecuentes fueron: disnea, dolor retroesternal, fiebre, tos, pérdida de peso y síndrome de vena cava superior. El diagnóstico se realizó por radiografía de tórax y tomografía axial computarizada. Se logró realizar la excéresis total del tumor y las metástasis, así como la tumoración intracardíaca, con reparación de la válvula tricúspide. El paciente ha tenido una evolución satisfactoria durante el primer año de seguimiento.

ABSTRACT Germ cell tumors are not so frequent; among them, teratomas are most common in the mediastinal location. Teratomas in young men are usually malignant (teratocarcinoma), with a gender ratio of 13.5:1, and an average age of 26 years. We describe the evolution of a 19-year-old man with a mediastinal tumor, with pulmonary metastases and intracardiac infiltration, successfully removed; whose most frequent symptoms were: dyspnea, retrosternal pain, fever, cough, weight loss and superior vena cava syndrome. The diagnosis was made by chest x-ray and computed tomography. Total removal of the tumor and metastases was achieved, as well as the intracardiac tumor, with tricuspid valve repair. The patient had a satisfactory outcome during the first follow-up year.

Tumor de células germinales primario de mediastino (seminoma de mediastino) / Cell Germ primary tumor of mediastinum (seminoma of mediastinum)

RESUMEN Fundamento: el tumor de células germinales de mediastino anterior, es una formación de células neoplásicas localizada en mediastino. Se forman por defectos congénitos en la etapa embrionaria por migración de la célula germinal primordial y derivan de células que están dentro de las gónadas (germinales), pueden migrar y localizarse fuera de estas (extra gonadal) como el caso que se presentó, y situarse en mediastino anterior (seminoma). La localización más reportada de los extra gonadales es en mediastino anterior. Objetivo: describir un enfermo con tumor primario de células germinales del mediastino anterior. Caso clínico: paciente de 23 años de edad, masculino, con antecedentes de asma bronquial, acudió al cuerpo de guardia con tos seca y frecuente, pérdida de peso de 7 kg en un mes y fiebre de 38˚C hace dos días. Al examen físico, ligera palidez cutáneo mucosa, murmullo vesicular abolido en hemitórax derecho sin estertores. Después de estudios analíticos, radiografía de tórax, tomografía axial computarizada de pulmón y estudio histológico, se concluyó como neoplasia de células germinales primitiva extra gonadal de mediastino anterior. Conclusiones: la localización más frecuente de los tumores de células germinales de mediastino, extragonadal, es mediastino anterior. Son los tumores sólidos de mediastino más frecuentes en varones y afecta entre los 20 y 40 años de edad, hecho infrecuente en la práctica clínica.

ABSTRACT Background: the anterior mediastinal germ cell tumor is a formation of neoplastic cells located in the mediastinum. They are formed by congenital defects in the embryonic stage by migration of the primordial germ cell and dermal cells that are within the gonads (germinal), being able to migrate and localize outside of these (extra gonadal) as the case presented, and to be located in the anterior mediastinum (Seminoma). The most reported location of the extra gonadal is in the anterior mediastinum. Objective: to describe a patient with primary tumor of germ cell of the anterior mediastinum. Clinical case: a 23-year-old male patient with a history of bronchial asthma attended the emergency room with a dry, frequent cough, weight loss of 7 kg in one month and fever of 38˚C for 2 days. At physical examination, slight mucous skin pallor, vesicular murmur abolished in right hemi-thorax without rales. After analytical studies, chest x-ray, computerized lung tomography and histological study, it was concluded as primitive extra-gonadal germ cell neoplastic of anterior mediastinum. Conclusions: the most frequent location of mediastinal germ cell tumors, extra-gonadal, is anterior mediastinum. They are the most frequent mediastinal solid tumors in men and affect between 20 and 40 years of age; being the case that occupies a male patient of 23 years, uncommon in clinical practice.

Thymic teratoma presenting as non-immune hydrops fetalis.

Teratomas are one of the most frequent tumors in the pediatric population. They occur anywhere along the midline of the body, following the course of the embryonic germ cell ridge. In the mediastinal location, they exert space occupying effects, leading to a myriad of complications, including non-immune hydrops fetalis. We describe a fatal case of an immature thymic teratoma in a neonate presenting with hydrops fetalis. This case emphasizes the importance of early diagnosis and surgical intervention in such cases.

Thymic teratoma presenting as non-immune hydrops fetalis

Teratomas are one of the most frequent tumors in the pediatric population. They occur anywhere along the midline of the body, following the course of the embryonic germ cell ridge. In the mediastinal location, they exert space occupying effects, leading to a myriad of complications, including non-immune hydrops fetalis. We describe a fatal case of an immature thymic teratoma in a neonate presenting with hydrops fetalis. This case emphasizes the importance of early diagnosis and surgical intervention in such cases.

Tumor lamination in mediastinal giant tumors / Laminação tumoral nos tumores gigantes do mediastino

ABSTRACT Mediastinum tumors may grow slowly and reach giant proportions without symptoms, hindering surgical removal. Tumor big dimensions difficult surgical maneuvers, with risk of uncontrollable bleeding and prejudice to surrounding structures. It may be necessary the use of exceptional measures such as venous-venous circulatory deviation, pre-operatory embolization and total extracorporeal circulation. We describe the technique of tumor lamination that allows for complete or almost complete resection of such tumors that in many occasions are not resectable. The description is based on the results of four patients treated with mediastinum giant tumors.

RESUMO Tumores do mediastino podem crescer lentamente e atingir proporções gigantes sem apresentar sintomas, tornando a remoção cirúrgica problemática. As dimensões exacerbadas da neoplasia dificultam as manobras cirúrgicas, com risco de hemorragia incontrolável e comprometimento de estruturas adjacentes, levando à utilização de medidas de exceção, como a derivação circulatória veno-venosa, a embolização pré-operatória e a circulação extracorpórea total. Diante disto, descrevemos a técnica de laminação tumoral, que permite a ressecção total ou quase total de tumores considerados, muitas vezes, irressecáveis, tendo por base os resultados alcançados em quatro pacientes portadores de neoplasias gigantes do mediastino.

Yolk sac primary tumor of mediastino: a rare case in a young adult / Tumor de saco vitelínico primário do mediastino: raro caso em um adulto jovem

ABSTRACT Germ cell tumors are rare neoplasms that mostly occur in the gonads, although they can also affect other body sites, especially the anterior mediastinum (50 to 70% of all extragonadal germ cell tumors). We report a case of a primary mediastinal yolk sac tumor, a rare and aggressive germ cell tumors subtype. This was a 38-year-old man who was admitted to Hospital do Servidor Público Estadual "Francisco Morato de Oliveira", complaining about dyspnea and dry cough for 1 year. The computed tomography scan of his chest revealed a large mass in the anterior mediastinum with heterogeneous enhancement to the contrast associated with pleural effusion. There were also high serum levels of alpha-fetoprotein. After neoadjuvant chemotherapy, the patient underwent surgical resection of the mass, followed by pathological examination, which confirmed a primary mediastinal yolk sac tumor, a nonseminomatous subtype of germ cell tumors. Primary mediastinal yolk sac tumors have poor prognosis, despite advances in therapy with surgical resection and cisplatin-based chemotherapy. This poor prognosis is due to the degree of invasion and unresectability in most patients by the time of the diagnosis.

RESUMO Os tumores de células germinativas são neoplasias raras que acometem mais frequentemente as gônadas, embora possam também ocorrer em outras localizações do corpo, destacando-se o mediastino anterior (50 a 70% de todos os tumores de células germinativas extragonadais). No presente artigo, relatamos um caso de tumor de saco vitelínico mediastinal primário, de subtipo raro e agressivo de tumor de células germinativas. Tratava-se de um homem, 38 anos, admitido no Hospital do Servidor Público Estadual "Francisco Morato de Oliveira", com quadro de dispneia e tosse seca há 1 ano. Na investigação clínica, foi solicitada tomografia computadorizada de tórax, que mostrou volumosa massa no mediastino anterior com realce heterogêneo ao meio de contraste associada a derrame pleural. Havia ainda aumento dos níveis séricos da alfafetoproteína. Após quimioterapia neoadjuvante pré-operatória, o paciente foi submetido à ressecção cirúrgica, seguida de estudo anatomopatológico da peça, no qual demonstrou tratar-se de um tumor de saco vitelínico primário do mediastino. Os tumores de saco vitelínicos primários do mediastino têm prognóstico reservado, apesar do avanço na terapêutica com a ressecção cirúrgica e a quimioterapia à base de cisplatina. Isto se deve ao grau de invasão e de irressecabilidade na maioria dos pacientes no momento do diagnóstico.

Plasmocitoma extramedular: una rara causa de masa mediastínica / Extramedullary mediastinal plasmacytoma: report of one case

Solitary extramedullary plasmacytomas represent 3% of plasma cell neoplasms. Their most common locations are the upper gastrointestinal and respiratory tract. We report a 70-year-old male presenting with progressive dyspnea and cough. A chest radiography showed widened mediastinum. Chest computed tomography showed a mediastinal mass. A bronchoscopy showed an extrinsic compression and complete occlusion of the primary bronchus. A self-expandable prosthesis was installed in left bronchus. A population of plasmacytoid cells was evidenced in the mass biopsy. Immunohistochemistry revealed CD20+ and CD138+. These tumor cells stained positively for lambda light chains, but negatively for kappa chains. Therefore an extramedullary plasmacytoma was diagnosed. A multiple myeloma was discarded with a normal blood count, serum calcium and creatinine levels. Serum protein electrophoresis had a monoclonal spike, serum IgG was 1963 mg/dl. Bone marrow aspiration had 1% of plasma cells. Bone x-rays were normal. Computed tomographies of the neck, thorax, abdomen and pelvis ruled out other plasmacytomas. Eight cycles of chemotherapy with thalidomide, melphalan and prednisone were indicated, obtaining complete remission of the tumor. The review of the literature shows that mediastinal extramedullary plasmacytomas are extremely rare. They generally appear in men aged between 50 and 60 years. The treatment of choice is radiotherapy, but given the location in the present case, chemotherapy was considered the best option. Recurrence is approximately 10 to 30% and 10% of patients progress to myeloma, thus requiring close monitoring.

Fenómeno de flujo, diagnóstico diferencial de la hipoxemia refractaria en pacientes con masa mediastinal anterior. Reporte de caso / Flow phenomenon, a differential diagnosis in refractory hypoxaemia in patients with anterior mediastinal mass. Case report

Introduction: Refractory hypoxaemia is a critical, life-threatening condition if not resolved promptly. The flow phenomenon is implicated in its development. Objectives: Clinical case presentation and non-systematic review of the literature on refractory hypoxaemia and flow phenomenon. Incidence, aetiology and pathophysiology are described. Materials and methods: Clinical case presentation authorised by the Ethics Committee of our institution of a young patient who presented with progressive dyspnoea, reaching functional class IV/IV. A search of the literature was conducted in Pub Med, Scielo and Bireme. Results: There is a growing interest in the physiological flow phenomenon leading to the development of refractory hypoxaemia in the absence of increased pressure in the cardiac cavities. Few reports are found. Conclusions: Flow phenomenon-related refractory hypoxaemia must be suspected as an exclusion diagnosis in patients with a mediastinal mass. Prone ventilation is proposed as a bridging therapy in order to revert a life-threatening condition.

Resumen Introducción: La hipoxemia refractaria es una condición crítica potencialmente mortal si no se corrige rápidamente. El fenómeno de flujo está implicado en su desarrollo. Objetivos: Presentación de un caso clínico y revisión no sistemática de la literatura sobre hipoxemia refractaria y el fenómeno de flujo, describimos su incidencia, etiología y fisiopatología. Material y métodos: Con autorización del comité de Ética de nuestra institución, presentamos un caso clínico de un paciente joven quien consulto por disnea progresiva hasta clase funcional IV/IV. La búsqueda bibliográfica se realizó en Pub Med, Scielo y Bireme. Resultados: Se ha despertado un creciente interés en el fenómeno fisiológico de flujo que conlleva al desarrollo de hipoxemia refractaria en ausencia de aumento de presión en las cavidades cardiacas. Se encuentran escasos reportes. Conclusiones: La hipoxemia refractaria por el fenómeno de flujo debe ser sospechada como diagnóstico de exclusión en pacientes con masa mediastinal. Proponemos la ventilación en prono como terapia puente para revertir una condición potencialmente mortal.

Ressecção de veia cava superior para neoplasias de pulmão e mediastino com reconstrução utilizando pericárdio bovino / Superior vena cava resection for lung and mediastinal neoplasms with reconstruction using bovine pericardium

A invasão de veia cava superior por neoplasias de pulmão e mediastino por muito tempo foram consideradas contraindicação para ressecção cirúrgica, casuando a síndrome de veia cava superior. Com o recente avanço de técnicas anestésicas, cirurgia torácica e cardiovascular, aperfeiçoamento de próteses vasculares, foi possível a extensão da radicalidade oncológica visando à ressecção completa e curados pacientes com tumores torácicos invasores da veia cava superior. Este artigo apresenta e descreve a técnica e abordagem utilizada para execução da ressecção da veia cava superior e reconstrução com pericárdio bovino, realizado com sucesso em três pacientes com neoplasias intra-torácicas no Instituto Mário Penna/Hospital Luxemburgo, Belo Horizonte - MG, todos realizados sem a utilização de circulação extracorpórea. (AU)

The invasion of the superior vena cava by lung and mediastinal neoplasms for a long time was considered contraindication for surgical resection, causing the superior vena cava syndrome. With the recent advancement of anesthetic techniques, thoracic and cardiovascular surgery, and improvement of vascular prostheses, it was possible to extend oncologic radicality aiming at complete resection and cured patients with thoracic tumors invading the superior vena cava. This article presents and describes the technique and approach used to perform superior vena cava resection and bovine pericardial reconstruction successfully performed in three patients with intra-thoracic neoplasms at Instituto Mário Penna / Hospital Luxemburgo, Belo Horizonte - MG, all performed Without the use of extracorporeal circulation. (AU)

Quiste broncógeno mediastinal puro: reporte de tres casos / Pure mediastinal bronchogenic cyst: three case reports

Fundamento: el quiste broncógeno mediastinal puro suele localizarse en el mediastino medio, aparece en ambos sexos y a cualquier edad, aunque por ser congénito es mayor en niños y jóvenes, por lo general son asintomáticos y su diagnóstico muchas veces es casual. Se realiza una introducción temática sobre esta tumoración en cuanto a su origen, cuadro clínico, diagnóstico y tratamiento. Se señala que pueden aparecer en otras partes del tórax y cuello. Objetivo: exponer las diferentes formas de presentación del quiste broncógeno mediastinal. Casos clínicos: tres pacientes mujeres, mayores de 45 años atendidas en los servicios de cirugía de los hospitales Amalia Simoni y Manuel Ascunce Domenech a las cuales se le extirpó una tumoración quistíca mediastinal y que fueron estudiadas con anteriodad mediante el análisis en su conjunto del cuadro clínico, la radiografía, ultrasonografía y tomografía axial computarizada del tórax y confirmado el diagnóstico de quiste broncógeno por estudio histopatológico. El tratamiento incluyó resección del tumor mediante toracotomía con buena evolución postoperatoria en todas las enfermas. Conclusiones: se presentan tres enfermas mayores de 45 años tratadas con resección de un quiste broncógeno mediastinal por toracotomía abierta, con buenos resultados terapéuticos.

Background: pure mediastinal bronchogenic cyst is usually located in the middle mediastinum. It is more frequent in both sexes and at any age, although as it is congenital its incidence is higher in children and teenagers. Generally these cysts are asymptomatic and their diagnoses are casual in a lot of cases. A thematic introduction about a mediastinal cystic tumor was performed taking into account its origin, clinical manifestations, diagnosis and treatment. It has been pointed that it might appear in other parts of thorax and neck. Objective: to show the different forms of appearance of mediastinal bronchogenic cyst. Clinical cases: three women over 45 years old were treated in the surgical services areas of Amalia Simoni and Manuel Ascunce Domenech hospitals, to whom a resection of the mediastinal cystic tumor was performed. They were studied in advance through the analysis of the symptoms, thorax radiography, ultrasonography, computerized axial tomography and then the diagnosis was confirmed with the histopathological study. The treatment included tumor excision through thoracotomy with good post-operatory evolution in all the patients. Conclusions: three patients older over 45 years old were treated with mediastinal cystic tumor excision through open thoracotomy with good therapeutic results.

Myelolipoma of the posterior mediastinum in a patient with chronic dyserythropoietic anemia.

Myelolipoma (ML) is an uncommon benign mesenchymal neoplasia composed of mature adipose and hematopoietic tissues of uncertain etiology. Less than 3% of MLs occur in the mediastinal topography. The main differential diagnosis involves extramedullary hematopoiesis; therefore, pathological evaluation is essential for the definitive diagnosis. The authors report the case of a 50-year-old man diagnosed with congenital dyserythropoiesis and secondary hemosiderosis, who presented a posterior mediastinal tumor. The tumor was resected. It was macroscopically characterized by mature fat tissue with fibrous areas and soft consistency, which was yellowish at the cut surface. Histology revealed a well-defined nodule composed of adipocytes and hematopoietic tissue represented by erythroid, granulocytic, and megakaryocytic series, which was consistent with the diagnosis of ML located in the posterior mediastinum. There was no recurrence of the lesion during the 3-year follow-up. The aim of this report is to show the diagnosis of an unusual mediastinal lesion in the context of a chronic hematologic disease.

Atelectasia aguda súbita pulmonar izquierda por linfoma no Hodgkin de mediastino anterior y superior

Paciente mujer de 23 años que ingresa a emergencia por cuadro insidioso y progresivo de un mes de evolución, con disfonía, ronquera, estridor laríngeo y dificultad respiratoria. Tomografía cervicotorácica mostró obstrucción extrínseca de la tráquea de más del 70% de su luz y obstrucción del bronquio izquierdo de más del 50% de su luz. Presenta súbitamente desaturación arterial y abolición del murmullo vesicular del hemitórax izquierdo, por radiografía de tórax muestra atelectasia masiva súbita del pulmón izquierdo, que revierte luego del manejo médico. Se confirma linfoma no Hodgkin en biopsia por congelación. Se discute el manejo oportuno ante una atelectasia.

A 23-year old female patient was admitted to Emergency because of an insidious and progressive condition lasting for one month, characterized by dysphonia, hoarseness, laryngeal stridor and respiratory distress. A cervico-thoracic CT scan showed an extrinsic tracheal obstruction, affecting more than 70% of its lumen, and a left bronchial obstruction affecting more than 50% of its lumen. All of a sudden she developed arterial oxygen desaturation and total absence of respiratory sounds in the left hemithorax. A chest X-ray film showed a sudden massive atelectasis of the left lung, which reverted after medical management. The frozen section biopsy specimen showed non-Hodgkin lymphoma. We discuss the timely management in cases of atelectasis.