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.

Anesthetic management of a large mediastinal mass for tracheal stent placement / Manejo anestésico de grande massa mediastinal para a colocação de stent traqueal

ABSTRACT The anesthetic management of patients with large mediastinal masses can be complicated due to the pressure effects of the mass on the airway or major vessels. We present the successful anesthetic management of a 64-year-old female with a large mediastinal mass that encroached on the great vessels and compressed the trachea. A tracheal stent was placed to relieve the tracheal compression under general anesthesia. Spontaneous ventilation was maintained during the perioperative period with the use of a classic laryngeal mask airway. We discuss the utility of laryngeal mask airway for anesthetic management of tracheal stenting in patients with mediastinal masses.

RESUMO O manejo anestésico de pacientes com grandes massas situadas no mediastino pode ser complicado por causa dos efeitos da pressão da massa sobre as vias aéreas ou grandes vasos. Relatamos o manejo anestésico bem-sucedido de uma paciente de 64 anos com uma grande massa mediastinal que invadiu os grandes vasos e comprimiu a traqueia. Um stent traqueal foi colocado para aliviar a compressão da traqueia, sob anestesia geral. A ventilação espontânea foi mantida durante o período perioperatório com o uso de uma máscara laríngea clássica. Discutimos a utilidade da máscara laríngea para o manejo da colocação de stent traqueal em pacientes com massas situadas no mediastino.

Hemotórax espontáneo en paciente con schwannoma de mediastino / Spontaneous hemothorax caused by a schwannoma of the posterior mediastinum

Background: Schwannomas are the most common neurogenic tumors of the posterior mediastinum. They are usually asymptomatic and the symptoms associated with them are chest pain, cough and dyspnea. Case report: We report a 36 years old female consulting in the emergency room due to sudden onset dyspnea. The imaging study confirmed the presence of a hemothorax secondary to a tumor of the posterior mediastinum, which was surgically excised. The pathological study reported a Schwannoma.

Introducción: El mediastino es un área anatómica que contiene estructuras y células pluripotenciales que pueden originar distintos tumores. Los Schwannomas son los tumores neurogénicos más comunes del mediastino posterior, se caracterizan por tener un crecimiento lento. A menudo son asintomáticos y se diagnostican como hallazgos radiológicos. Cuando son sintomáticos, lo más común es que presenten dolor torácico, tos y disnea. El hemotórax es una rara forma de manifestación. Caso clínico: Se presenta en este trabajo el caso de una mujer de 36 años, que ingresa al servicio de urgencia por un cuadro de disnea súbita. El estudio por imágenes confirma el diagnóstico de hemotórax, secundario a un tumor de mediastino posterior, el cual requiere cirugía. Anatomía patológica corrobora el diagnóstico presuntivo de Swchannoma de mediastino posterior. Conclusión: La cirugía es necesaria para poder resecar completamente el tumor y en el Schwannoma benigno es curativa.

Conducta en el síndrome de vena cava superior (SVCS) / Management in superior vena cava syndrome

Objetivos: Mostrar la conducta realizada en este síndrome. Material y Métodos: Serie retrospectiva de 5 casos tratados entre el 1 de abril de 1995 y el 31 de marzo de 2013. Diagnóstico por imágenes y toma de biopsia con tratamiento específico posterior. Resultados: Todos se debieron a causas tumorales: 2 timomas (uno irresecable), 1 linfoma Hodgkin, 1 leiomiosarcoma de vena cava y 1 caso de cáncer pulmonar a pequeñas células. Fallecieron por progresión de enfermedad 2 casos, 2 están libres de enfermedad a 12 y 11 meses de tratamiento y 1 enfermo se perdió en el seguimiento. Conclusiones: Se define la necesidad de seguir un algoritmo que determine los pasos a seguir ante la presencia de este síndrome.

Objectives: Management in this syndrome. Material and Methods: Retrospective series of 5 cases treated between April 1, 1995 to March 31, 2013. Diagnostic imaging and taking biopsy with subsequent specific treatment. Results: All patients were due to tumor causes: 2 thymomas (one unresectable), 1 lymphoma Hodgkin, 1 Leiomyosarcoma of vena cava and 1 case of small cell lung cancer. Died of disease progression 2 cases, 2 are free of disease to 12 and 11 months of treatment and 1 patient was lost to follow-up. Conclusions: The need to follow an algorithm which determines the steps to follow in the presence of this syndrome is defined.

Síndrome de Kinsbourne: relato de caso / Kinsbourne syndrome: case report / Síndrome de Kinsbourne: relato de caso

JUSTIFICATIVA E OBJETIVOS: A síndrome de Kinsbourne é uma doença neurológica rara que afeta, principalmente, crianças previamente hígidas, na faixa etária entre seis e trinta e seis meses, caracterizada pela presença de opsoclonia (movimentos oculares rápidos, irregulares, horizontais e verticais), mioclonias que podem afetar tronco, extremidades ou face e ataxia cerebelar. Pode ser considerada uma síndrome paraneoplásica pela associação com neuroblastomas, ganglioneuroma e raramente hepatoblastomas. Foi objetivo deste artigo apresentar os aspectos mais relevantes da síndrome de Kinsbourne, bem como a técnica anestésica usada para ressecção de tumor de mediastino em uma criança portadora desta síndrome. RELATO DO CASO: Criança de um ano e cinco meses com diagnóstico de tumor do mediastino posterior portadora de síndrome de Kinsbourne. Pré-medicação com midazolam oral. Indução da anestesia com sevoflurano, óxido nitroso, fentanil e rocurônio. Manutenção da anestesia com sevoflurano, óxido nitroso, fentanil e rocurônio. Reversão do bloqueio neuromuscular com neostigmina associado à atropina. Analgesia pós-operatória com o uso de dipirona, cetoprofeno e morfina. Levada para a Unidade de Terapia Intensiva extubada, com parâmetros hemodinâmicos e respiratórios estáveis. Alta da UTI quatro dias após a cirurgia e alta hospitalar no sétimo dia de pós-operatório sem intercorrências. Anátomo-patológico evidenciou para ganglioneuroblastoma. CONCLUSÕES: A síndrome de Kinsbourne é uma doença neurológica rara. As drogas usadas em nosso paciente mostraram ser seguras e permitiram uma anestesia sem intercorrências. Drogas que desencadeiam ou agravam opsoclonia e mioclonias, como cetamina e etomidato, deverão ser evitadas nesses pacientes.

BACKGROUND AND OBJECTIVES: Kinsbourne syndrome is a rare neurological disorder that primarily affects children previously healthy and aged between 6 and 36 months. It is characterized by opsoclonus (rapid, irregular, horizontal and vertical eye movements) and myoclonus that may affect trunk, limbs or face, and cerebellar ataxia. It may be considered a paraneoplastic syndrome by association with neuroblastomas, hepatoblastomas and, rarely, ganglioneuromas. The aim of this paper was to present the most relevant aspects of Kinsbourne syndrome, as well as the technique used for resection of mediastinal tumor in a child with this syndrome. CASE REPORT: Child, 1 year and 5 months, with a diagnosis of posterior mediastinal tumor and Kinsbourne syndrome. Premedicated with oral midazolam. Anesthesia induced with sevoflurane, nitrous oxide, fentanyl, and rocuronium. Maintenance of anesthesia with sevoflurane, nitrous oxide, fentanyl, and rocuronium. Neuromuscular block reversal with neostigmine combined with atropine. Postoperative analgesia with the use of dipyrone, morphine, and ketoprofen. Taken to the intensive care unit extubated, with stable hemodynamic and respiratory parameters. ICU discharge four days after surgery and hospital discharged on the seventh postoperative day without complications. Anatomopathological examination revealed ganglioneuroblastoma. CONCLUSIONS: Kinsbourne syndrome is a rare neurological disorder. The drugs used in our patient proved safe and allowed an uneventful anesthesia. Drugs that trigger or aggravate opsoclonus and myoclonus, such as ketamine and etomidate, should be avoided in these patients.

JUSTIFICATIVA Y OBJETIVOS: El síndrome de Kinsbourne es una enfermedad neurológica rara que afecta principalmente a los niños anteriormente sanos, en una franja etaria entre los 6 y los 36 meses, y que se caracteriza por la presencia de opsoclonia (movimientos oculares rápidos, irregulares, horizontales y verticales), mioclonias que pueden afectar el tronco, las extremidades o la cara, y por la ataxia cerebelar. Puede ser considerado un síndrome paraneoplásico por la asociación con los neuroblastomas, ganglioneroma y raramente hepatoblastomas. El objetivo de este artículo, fue presentar los aspectos más relevantes del síndrome de Kinsbourne, como también la técnica anestésica usada para la resección del tumor del mediastino en un niño portador de ese síndrome. RELATO DEL CASO: Niño de 1 año y 5 meses con diagnóstico de tumor del mediastino posterior, portador del síndrome de Kinsbourne. Premedicación con midazolam oral. Inducción de la anestesia con sevoflurano, óxido nitroso, fentanilo y rocuronio. Mantenimiento de la anestesia con sevoflurano, óxido nitroso, fentanilo y rocuronio. Reversión del bloqueo neuromuscular con neostigmina asociado a la atropina. Analgesia postoperatoria con el uso de dipirona, cetoprofeno y morfina. Fue derivado a la Unidad de Cuidados Intensivos y desentubado con parámetros hemodinámicos y respiratorios estables. El alta de la UCI fue cuatro días después de la cirugía y el alta hospitalaria fue al séptimo día del postoperatorio sin intercurrencias. La anatomo-patología arrojó un ganglioneuroblastoma. CONCLUSIONES: El síndrome de Kinsbourne es una enfermedad neurológica rara. Los fármacos usados en nuestro paciente, mostraron ser seguros permitiendo una anestesia sin intercurrencias. Los fármacos que desencadenan o que agravan la opsoclonia y mioclonias, como la cetamina y el etomidato, deberán ser evitados en esos pacientes.

Leiomiosarcoma primario de mediastino que compromete el sistema de la vena cava superior: resección quirúrgica asociada a reconstrucción venosa / Primary leiomyosarcoma of the mediastinum involving the superior vena cava system: surgical resection and venous reconstruction

Mediastinal tumors have multiple presentation forms and various pathological origins. Sometimes they reach a large size and can involve the system of superior vena cava by compression or infiltration. We present a 48 year old man admited for dyspnea, headache and palpitations in decubitus, for the last two months. The X-ray and chest computed tomography showed an anterior mediastinal tumor and the tru-cut pathological study concluded leiomyosarcoma. He underwent surgical treatment; the tumor compromised the system of the superior vena cava and was resected with ligation and resection the left brachiocephalic trunk. Part of the superior vena cava was reconstructed with autologous pericardium. The right phrenic nerve was surrounded by the tumor mass and was resected in block. The patient recovered satisfactorily and was discharged on the eleventh day post surgery. The histopathologic study of the surgical specimen confirmed a leiomyosarcoma G2 (3,100 gr). Full adjuvant radiotherapy was done. At 15 months after surgery the patient is in good condition without evidence of recurrence or superior vena cava syndrome.

Los tumores del mediastino pueden tener múltiples formas de presentación y diversos orígenes anato-mopatológicos. A veces alcanzan gran tamaño y pueden comprometer por compresión o infiltración el sistema de la vena cava superior. Presentamos el caso de un hombre de 48 años que consultó por disnea, cefalea y palpitaciones en decúbito de dos meses de evolución. En la radiografía y en la tomografía computada de tórax se demostró un tumor de mediastino anterior, el estudio anatomopatológico mediante biopsia trucut concluyó leiomiosarcoma. Se realizó tratamiento quirúrgico, se resecó la masa tumoral que comprometía el sistema de la vena cava superior, se ligó y resecó el tronco venoso braquiocefálico izquierdo o innominado y se reconstruyó parte de la vena cava superior con parche de pericardio autólogo. El nervio frénico derecho se encontraba rodeado por la masa tumoral y se resecó junto con el tumor. El paciente evolucionó satisfactoriamente y fue dado de alta al décimo primer día post operado. El estudio de anatomía patológica de la pieza operatoria confirmó un leiomiosarcoma G2 de 3.100 gramos. Se completó tratamiento con radioterapia post operatoria. A 15 meses post operado el paciente se encuentra en buenas condiciones generales, sin evidencias de recidiva y sin síndrome de vena cava superior.

Tumor maligno de la vaina del nervio periférico del mediastino en un paciente con neurofibromatosis tipo 1 / Mediastinal malignant peripheral nerve sheath tumor in a neurofibromatosis type 1 patient

El tumor maligno de la vaina del nervio periférico (TMVNP), es una neoplasia maligna originada en las células de Schwan de la vaina de revestimiento del nervio periférico. Describir el caso de un hombre con neurofibromatosis tipo 1 (NF1), quién presentó un TMVNP de bajo grado, y realizar una discusión sobre esta enfermedad. Hombre de 28 años, con antecedente de NF1 diagnosticada a los 15 años de edad, con dolor pleurítico izquierdo, disnea y pérdida de peso de 10 meses de evolución. Al examen de tórax, se observó marcada hipercifosis dorsal con disminución del murmullo pulmonar. La radiografía de tórax y tomografía axial computarizada (TAC), evidenciaron gran masa radioopaca bien delimitada en mediastino posterior. Por lo anterior, se realizo biopsia por punción con aguja gruesa guiada por TAC, en la cual se identificó una neoplasia maligna mesenquimal. Se decidió realizar resección del tumor a través de toracotomía posterolateral, en la que se obtuvo gran masa de 8x9x9 cm, de superficie externa irregular, pardo-violácea y consistencia firme. El estudio histopatologico e inmunofenotípico concluyo el diagnóstico de TMVNP en mediastino posterior Grado 1. Posterior a la cirugía, el paciente se encuentra asintomático. Se presentó un caso de TMVNP originado en un paciente con NF1, presentación que generalmente cursa con peor pronóstico, además se realizo una breve revisión de los aspectos más relevantes de esta enfermedad, algunos de los cuales han tenido un avance vertiginoso en años recientes.

The malignant peripheral nerve sheath tumor (MPNST) is a malignant neoplasm originated in the Schwan cells of the periferic nerves sheath. We describe a case of a man with Neurofibroatosis Type 1 (NF1), who developed a low grade MPNST, and subsequent to a discussion of this disease. 28-year-old Man with pleuritic pain in the left hemithorax, dyspnea and weight loss, with a previous diagnosis of NF1, from the age of 15 and a family history of NF1. At chest examination the patient had an intense thoracic kyphosis, with a decline in the ventilation of the inferior two thirds of the left hemithorax, where a dull sound to percusión was also found. The chest X rays showed a large radiopaque and well delimited mass in the posterior mediastinum, that pushed the cardiovascular structures to the anterior region, which was also documented by chest computed tomography (CT). In view of the above, a puncture biopsy was performed with thick needle guided by CT, from where a malignant mesenhymal neoplasm was identified. It was decided to perform the resection of the tumor of the left posterior mediastinum, by left posterior lateral thoracotomy, in which a large mass of 8x9x9 cm was obtanied, with irregular external surface, brown-violet, and firm. The histopathological and inmunophenotypic study concluded the diagnosis of MPNST in the posterior mediastinum grade 1. Following surgery the patient was asymptomatic. We present a case of MPNST which originated in a patient with NF1, who would usually have a worse prognosis. A brief review of the more relevant aspects of this disease was also reported, some of which have shown important progress in recent years.

Síncope y bocio nodular: desafío diagnóstico / Syncope associated to a thyroid nodule: report of one case

We report a 60 years old male presenting with a history of repeating syncopal episodes lasting seven days. The patient had a history of an intestinal lymphoma treated four years ago. Imaging studies showed a macro nodule in the left thyroid lobe. The pathological study disclosed a type B non Hodgkin lymphoma. The patient was treated with chemotherapy with a favorable outcome. The association of syncope and cervical tumors has been described previously.

Mediastinal haemangioma with pericardial effusion: A rare entity.

Mediastinal haemangioma is a rare benign vascular tumour. A young male presented with complaints of cough and dyspnoea. Serial chest radiographs were suggestive of progressive mediastinal widening and cardiomegaly. Pericardiocentesis revealed haemorrhagic fluid which was negative for microbiology and malignant cells. Patient was unresponsive to antituberculosis treatment and steroids. Computed tomography (CT) of thorax revealed an anterior mediastinal mass lesion with pericardial effusion which on biopsy was found to be a mediastinal haemangioma.

Superior vena cava syndrome: a presenting feature of mediastinal germ cell tumor

Superior vena cava syndrome [SVCS] is rare in children. Non Hodgkin's lymphoma [NHL] is the most common cause of SVCS in children. This report an adolescent male who presented with SVCS due to mixed germ cell tumor [GCT] of the anterior mediastinum with predominant yolk cell component. Such etiology of SVCS is rarely reported

Bócio de tireóide intratorácico e timoma invasivo: apresentação simultânea incomum / Intrathoracic goiter and invasive thymoma: rare concomitant presentation

Apresentamos uma rara situação de ocorrência simultânea de dois tumores de mediastino com diferentes topografias e histologias, encontrados durante a ressecção de volumosa massa mediastinal em paciente assintomático. A possibilidade de diferentes tumores contidos numa mesma massa tumoral está relatada; entretanto, não encontramos na literatura médica relato de diferentes tumores em localizações distintas. Os bócios de tiróide intratorácicos e os timomas representam uma grande parcela dos tumores encontrados no mediastino. O tratamento cirúrgico, sempre que possível, desempenha papel fundamental na perspectiva de cura. A exploração cirúrgica minuciosa é fundamental para uma ressecção completa e possível achado de lesões concomitantes.

We present a rare situation in which two mediastinal tumors of different topology and histology were found during the resection of an extensive mediastinal tumor in an asymptomatic patient. Different histologies within the same mass have been reported, although, to our knowledge, there have been no reports of different tumors at distinct locations. Thymomas and intrathoracic goiters account for a large proportion of the tumors found in the mediastinum. When feasible, surgical resection plays a fundamental role in effecting a cure. In order to identify concomitant lesions and perform a complete resection, detailed surgical exploration is required.

Adenoma paratiroideo ectopico mediastinal: diagnostico por sonda de deteccion gamma y reseccion por videotoracoscopia / Ectopic mediastinal parathyroid adenoma: detection with a radioisotopic probe and resolution with videothoracoscopy

Presentamos el caso de una mujer de 66 años con diagnóstico de hiperparatiroidismo primariopor adenoma paratiroideo ectópico mediastinal realizado por gammagrafía paratiroidea con 99mtcmetoxi-isobutil-isonitrito (Tc99-MIBI). Fue intervenida exitosamente mediante la técnica de videotoracoscopia.Durante el procedimiento se utilizó la medición de parathormona y a los 10 minutos de extirpado el adenoma seobtuvo un descenso mayor del 50% del basal. La sonda de detección gamma no fue efectiva in situ por la superposicióndel radioisótopo con el miocardio pero confirmó la radioactividad dentro del quirófano una vez extirpadoel adenoma. La paciente normalizó los valores de calcemia y parathormona en el postoperatorio y permaneciónormocalcémica luego de 9 meses de seguimiento. En nuestro caso, la localización prequirúrgica y elmonitoreo de parathormona intraoperatoria fueron de gran utilidad para el éxito quirúrgico, la sonda detectora marcada con Tc99-MIBI no fue efectiva in situ pero confirmó la radioactividad una vez extirpado el adenoma. El tratamiento quirúrgico endoscópico por videotoracoscopia fue bien tolerado, menos doloroso que la toracotomía y acortó el tiempo de internación

We report a 66 years old woman with a diagnosis of primary Hyperparathyroidism. Localization to mediastinum was obtained with parathyroid scintigraphy using 99mtc-methoxyisobutyl-isonitrite (Tc99-MIBI). The patient was successfully operated upon by means of a videothora-coscopicapproach. During the procedure serum parathormone was measured before and 10 minutes after adenomectomy,showing a more than 50% reduction from the basal level. An attempt to detect the precise site of the adenomawith a Tc99-MIBI probe was unsuccessful because of its proximity to the myocardium, but radioactivity was confirmed on the surgical specimen after resection. The patient’s calcemia and parathormone levels became normalduring the postoperative course and she remains normocalcemic 9 months after the procedure. In our case,preoperative localization and intraoperative parathormone measurements were both very useful for confirming surgical success; the intraoperative localization with a radioactive probe was not useful, but radioactivity was confirmedafter resection on the surgical specimen. The endoscopic surgical procedure with videothoracoscopy waswell tolerated, less painful than a thoracotomy, and it shortened the hospitalization period

Paraganglioma de mediastino com metástases pulmonares / Pulmonary metastasis of mediastinal paraganglioma

Descrevemos uma paciente de 27 anos que se apresentou com paraganglioma de mediastino anterior e médio e nódulos pulmonares bilaterais. O tratamento consistiu na ressecção das lesões pulmonares através de toracotomia anterior bilateral transesternal e retirada do paraganglioma com auxílio de circulação extracorpórea. Como tratamento neoadjuvante foram usadas radioterapia e quimioterapia. A evolução pós-operatória foi satisfatória, e catorze meses depois a paciente encontrava-se assintomática.

Etiology and outcome of superior vena cava (SVC) obstruction in adults.

Between 1997 and 2002, 107 patients with symptoms of superior vena cava (SVC) obstruction presented at a university hospital in Northeast Thailand. Age averaged 50.7 years (range, 1 to 84). The male to female ratio was 5.7:1. Duration of symptoms before diagnosis was 29.4 days (range, 2 to 240), including facial swelling, cough, and chest discomfort. About 20% of cases developed respiratory failure and 11.2% died shortly after admission. The mean hospital stay was 23.7 days. Anteroposterior and lateral chest radiographs and computed chest tomography helped locate the lesion. Transbronchial biopsy through bronchoscopy, transthoracic needle biopsy under computed tomography, lymph node biopsy, pleural fluid cytology and/or biopsy were used for histopathologic sampling. High levels of alpha-fetoprotein and beta-HCG indicated an anterior mediastinal mass. The most common etiology of SVC obstruction was bronchogenic carcinoma (51.8%), followed by an anterior mediastinal mass (14.5%), lymphoma (13.6%--with an LDH of 262 to 1459 U/l), metastatic cancer (9.1%), and acute lymphoblastic leukemia (1.8%). Benign SVC thrombosis was found in four patients with Behcet's disease or some other idiopathy. Mediastinal fibrosis from melioidosis occurred in three patients, which is rare, has not been previouly reported. Most patients (63.6%) received a combination of radiotherapy and corticosteroid and this helped 55.2% improve.

Anterior mediastinal teratoma presenting as cardiac tamponade.

Anterior mediastinal teratoma presenting clinically as cardiac tamponade is unusual. We report the case of a 58-year-old woman, with an unremarkable prior history, who was admitted with cardiac tamponade. Emergency pericardial aspiration showed a thick, yellow fluid. Computed tomography scan of the chest was suggestive of anterior mediastinal teratoma rupturing into the pericardium. Within 24 hours, just before the planned surgery could be performed, it re-ruptured, warranting emergency surgery. Histopathology confirmed the diagnosis of a teratoma. Early surgery is recommended for even asymptomatic benign mediastinal teratomas to avoid this potentially lethal complication of rupture into the pericardial cavity, and cardiac tamponade.