Tumores de testículo em crianças e adolescentes / Testicular tumors in children and adolescents

OBJETIVO: Avaliar comparativamente aspectos clínicos e epidemiológicos dos tumores de testículo na infância e adolescência. MÉTODOS: Análise retrospectiva dos prontuários de pacientes com neoplasias de testículo ou paratesticulares. Foram classificados como crianças os menores de 10 anos e como adolescentes os pacientes entre 10 e 20 anos. Os resultados obtidos foram comparados por meio do teste para duas proporções: teste não paramétrico de Mann-Whitney e teste de log-rank. RESULTADOS: No período de janeiro de 1992 a julho de 2009, foram admitidos 60 pacientes: 34 crianças e 26 adolescentes com neoplasias de testículo ou paratesticulares. As principais manifestações foram tumor e dor na bolsa escrotal. A queixa de dor foi mais comum em adolescentes (p = 0,006). Estes apresentaram tempo médio de história de 4,9 meses, mais prolongado do que crianças, com 2,3 meses (p = 0,01). Os tipos histológicos encontrados foram: tumores de células germinativas em 32/60 (53 por cento), rabdomiossarcomas (RMSs) em 23/60 (38,3 por cento) e outros em 5/60 (8,3 por cento). Os adolescentes apresentaram maior frequência de RMSs, metástases em linfonodos (p = 0,003) e a distância (p = 0,035). As diferenças na sobrevida dos pacientes estudados não foram estatisticamente significantes, havendo apenas indicativo de que a sobrevida, nos casos de RMS, é maior nas crianças (p = 0,072). CONCLUSÕES: Os adolescentes com tumor testicular apresentaram maior tempo de história, tipo histológico agressivo e doença avançada ao diagnóstico quando comparados às crianças, a despeito da pequena amostra.

OBJECTIVE: To perform a comparative assessment of the clinical and epidemiological aspects of testicular tumors in childhood and adolescence. METHODS: Retrospective analysis of medical records of patients with testicular or paratesticular neoplasms. Patients under 10 years were classified as children and patients between 10 and 20 were classified as adolescents. The obtained results were compared through the two-sample test for proportions: non-parametric Mann-Whitney test and log-rank test. RESULTS: 60 patients were admitted in the period from January 1992 to July 2009: 34 children and 26 adolescents with testicular or paratesticular neoplasms. The main manifestations were testicular tumor and scrotal pain. Pain complaints were more common in adolescents (p = 0.006), who presented a mean time from disease onset to diagnosis of 4.9 months, longer than children, who presented a period of 2.3 months from disease onset to diagnosis (p = 0.01). Histological types were as follows: germ cell tumors in 32/60 (53 percent), rhabdomyosarcomas (RMSs) in 23/60 (38.3 percent), and other in 5/60 (8.3 percent). Adolescents presented a higher incidence of RMSs, lymph node metastases (p = 0.003) and distant metastases (p = 0.035). Differences in survival rates among the studied patients were not statistically significant, the only indicative being that survival in RMS cases is longer for children (p = 0.072). CONCLUSIONS: Compared to children, adolescents with testicular tumor presented longer time from disease onset to diagnosis, aggressive histological type and advanced illness at diagnosis, despite the small sample analyzed.

Rabdomiosarcoma primario de corazón como causa de síncope recurrente en el adulto / Recurrent sycope secundary to primary cardiac rhabdomyosarcoma

El corazón puede ser afectado por neoplasias primarias o secundarias. Estas últimas son más frecuentes. Sin embargo, las neoplasias primarias adquieren particular importancia ya que constituyen un grupo de diverso origen y comportamiento, que se traduce en un tratamiento y manifestaciones clínicas diferentes. Se presenta el caso de una mujer de 45 años, quien consultó por síncopes a repetición de un año de evolución, acompañados de palpitaciones y dolor torácico. El ecocardiograma identificó masa de 2.1 cm por 1.8 cm en aurícula izquierda. La biopsia endomiocárdica demostró un rabdomiosarcoma primario de corazón. La paciente muere luego de documentarse una sobrevida de 22 meses. En este caso se observó una sobrevida prolongada, gracias al adecuado estudio de sus síntomas, diagnóstico y tratamiento precoz.

Primary or secondary neoplasms can affect the heart. Secondary are more common. However, primary neoplasms are relevant because is a group with diverse genesis, behavior, treatment and clinical manifestations. We present a case of a 45 year-old woman, with recurrent syncope started 1 year before her first consult. She had palpitations and chest pain. Echocardiography identifed a left atrium mass of 2.1 x 1.8 cm. Endomyocardial biopsy document a primary rhabdomyosarcoma of the heart. The patient dies after a overall-survival of 22 months. This case presented had a good study of its symptoms with an accurate diagnosis and early treatment, which provided prolonged survival of this rare and aggressive neoplasm.

Obstrução urinária aguda por rabdomiossarcoma pélvico / Acute urinary obstruction due to pelvic rhabdomyosarcoma

Os autores relatam um caso raro de obstrução urinária aguda por rabdomiossarcoma pélvico envolvendo a próstata em uma criança de quatro anos e seis meses de idade. São comentados as características desses tumores, as formas de apresentação clínica, os aspectos diagnósticos por imagem — ultra-som, cistografia e tomografia computadorizada —, o tratamento com poliquimioterapia e o prognóstico.

The authors report a rare case of acute urinary obstruction caused by pelvic rhabdomyosarcoma in a 4-year-old children. The diagnosis aspects for imaging e some therapeutic considerations are related.

Botryoid rhabdomyosarcoma of common bile duct.

Common bile duct is an unusual site for occurrence of botryoid rhabdomyosarcoma. Clinically it is often misdiagnosed as infectious hepatitis. Early diagnosis and treatment has greatly improved the prognosis of this aggressive neoplasm. Here the authors present a case report of Bortroyid Rhabdomyosarcoma of CBD in a two and half year old child masquerading as obstructive jaundice.

Colangiorresonancia: nuestra experiencia / MR cholangiography: our experience

Presentamos nuestra experiencia en colangiorresonancia dirigida a determinar los aportes referidos al diagnóstico de la enfermedad obstructiva de la vía biliar y su utilidad comparativa con otros métodos estudiando 32 pacientes que en la mayoría de los casos nos aportaron estudios previos tales como ecografía, TC, colangiopancreatografía retrógrada endoscópica (CPRE) y colangiografía transparietohepática (CTPH). A la luz de los resultados obtenidos presentamos en forma integral los casos más significativos, concluyendo que la colangiorresonancia es un método sumamente útil para el diagnóstico de la enfermedad obstructiva de la vía biliar, con la ventaja de ser un método no invasivo, que no requiere preparación alguna y no utiliza sustancias de contraste

Multiple rhabdomyoma of the heart presenting with a congenital supraventricular tachycardia: report of case with ultrastructural study

A case of congenital rhabdomyoma of the heart in a 5-month-old Korean infant is described. The patient presented with a congenital supraventricular tachyarrhymia that was detected in utero by fetal sonography. The tumor was multiple, but no obvious association with tuberous sclerosis complex was demonstrated. Microscopic examination revealed classic "spider cells" with rich glycogen content. Ultrastructurally, the cells contained numerous leptofibrils, clumped Z band material, and desmosome-like cell junctions. The case is a second documented case of cardiac rhabdomyoma in this country, and its presentation as an etiological factor of supraventricular tachycardia is a very unusual manifestation.