Condrosarcoma mesenquimal extraesquelético en un paciente pediátrico: revisión de la literatura a propósito de un caso / Extraskeletal mesenchymal chondrosarcoma in a pediatric patient: a case report

El condrosarcoma mesenquimal extraesquelético pertenece a un grupo de tumores cartilaginosos malignos, que son infrecuentes y habitualmente no presentan compromiso óseo. Se presenta más frecuentemente en la órbita, sistema nervioso central y en los tejidos blandos de los miembros inferiores. El compromiso metastásico es generalmente pulmonar y ganglionar, especialmente en pacientes adultos en quienes se ha demostrado una mayor mortalidad. Este artículo tiene como objetivo presentar el caso de un paciente masculino de 14 años de edad, que ingresó a nuestra institución con una lesión sólida localizada a nivel de los tejidos blandos en el aspecto posterior de la pierna derecha, cuyo estudio histopatológico confirmó un condrosarcoma mesenquimal extraesquelético, junto con una revisión bibliográfica del tema.

The extraskeletal mesenchymal chondrosarcoma belongs to a group of infrequent malignant cartilaginous tumors, which are not obvious mainly in the bone. The most common site of such tumors is within the orbit, the central nervous system and the soft tissues of the lower limbs. The commitment is usually metastatic to the lung and lymph nodes, especially in adult patients, who have shown an increased mortality. The paper aims at presenting the case of male patient aged 14, who was admitted to our institution with a solid lesion located at the level of soft tissues in the posterior aspect of the right leg and whose histopathological study confirmed Extraskeletal mesenchymal chondrosarcoma (CME) and the literature review about the same.

Primary synovial sarcoma of the kidney with rhabdoid features.

Synovial sarcoma is a soft tissue sarcoma with clearly defined histologic, immunophenotypic, and molecular features. It occurs predominantly in the extremities of young adults but has been reported in many other anatomic sites. Histologically, it is classified as biphasic, monophasic, and poorly differentiated. The latter category, which includes tumors with a rhabdoid morphology, has been associated with a more aggressive behavior. Generally, the biphasic variant does not pose any diagnostic problem because of its typical histologic appearance; in contrast, the monophasic and poorly differentiated variants may represent a diagnostic challenge because their microscopic features can be confused with those of other spindle cell tumors with rhabdoid features. The application of molecular techniques, such as reverse transcriptase polymerase chain reaction to detect the fusion transcript associated with the characteristic t(X;18) translocation of synovial sarcoma, has enabled the confirmation of this diagnosis, even in cases of unusual localization, such as the one we present here.