Periosteal Ewing Sarcoma with Distant Metastases: Report of Two Patients and Review of the Literature.

Periosteal Ewing sarcoma (ES) is an exceedingly rare topographic subtype of the ES. To our knowledge, only 60 patients have been reported in the medical English language literature. It predominantly affects men in the second decade of life and arises in the long tubular bone diaphysis. PES rarely develops distant metastases. We report two patients of this rare ES location that were found on the distal tibial shaft and proximal femoral diaphysis of a 21-year-old man and an 8-year-old boy, respectively. Both patients were treated with neoadjuvant chemotherapy, wide resection, and adjuvant chemotherapy. One of our patients had lung metastases at the time of diagnosis and died 5 years later. The other patient presented intramedullary humeral bone metastasis 19 years after diagnosis. There has been no evidence of disease in the 26 years of follow-up. Close follow-up of periosteal ES is recommended because distant metastases may exceptionally occur, even several years after diagnosis.

Clinical and therapeutic management of a pleural fibrous tumor with ALK translocation.

We present a clinical case of a solitary fibrous pleural tumor (TFSP) with abnormal behavior due to its high aggressiveness and poor short-term prognosis, where an ALK translocation was identified. TFSP is an infrequent tumor, with an estimated incidence of around 8 per 100,000 inhabitants and with few cases reported in the literature. The most common is that it is a neoplasm of benign behavior, with debut as a localized mass and whose The treatment of choice is surgical resection. All the data in the literature contrast with our experience, which we report below.

Presentamos un caso clínico de un tumor fibroso solitario pleural (TFSP) de comportamiento anómalo por su alta agresividad y mal pronóstico a corto plazo, en el que se identificó una translocación de ALK. El TFSP es un tumor infrecuente, con una incidencia estimada en torno a 8 casos por 100,000 habitantes y con pocos reportes en la literatura. Lo más habitual es que se trate de una neoplasia de comportamiento benigno, con debut como una masa localizada y cuyo tratamiento de elección es la resección quirúrgica. Todos los datos de la literatura contrastan con nuestra experiencia, que presentamos a continuación.