Requirement for novel surgical and clinical protocols for the treatment of XXXX : A report of two cases and a review of the literature.

@entity1 @entity632 @entity66 @entity5 @entity391 @entity786 @entity29 @entity2269 @entity3041 @entity1277 @entity1891 @entity490
UNASSIGNED: @entity2269 ( @entity2269 ) is a rare and highly aggressive @entity5 that was initially described in 1989. @entity2269 predominantly affects young @entity1 and typically occurs in the intra-abdominal area. The present study describes the cases of two @entity1 with @entity2269 . The first @entity1 was a 23-year-old male who presented with @entity490 in the right flank, coupled with difficulty urinating and @entity29 . The second @entity1 was 12-year-old female who presented with @entity490 , @entity391 and @entity1277 . A computed tomography scan of the abdomen revealed the presence of an extensive pelvic mass in each @entity1 , however, a visceral origin was not clearly identifiable in the first @entity1 . In the second @entity1 , a large soft-tissue @entity5 was located posterior to the pancreatic tail and the stomach, with no anatomical line visible between the stomach and splenic vein. Ultrasound-guided biopsy in the first @entity1 and videolaparoscopy in the second @entity1 followed by immunohistochemical analysis clarified the presence of a malignant @entity5 composed of small, blue, round cells. Due to right ureter involvement and @entity3041 in the first @entity1 , a treatment strategy of surgical debulking of the @entity5 was selected. The surgical procedure involved en bloc resection of the lesion associated with a pelvic peritonectomy, followed by post-operative radiotherapy. However, the second @entity1 exhibited @entity66 , therefore, a chemotherapeutic protocol of @entity1891 , @entity632 and @entity786 , as well as radiation therapy, was scheduled. Disease relapse was observed in the abdominal cavity of the first @entity1 after one year, while the second @entity1 remains asymptomatic. Following analysis of present two cases, it was concluded that aggressive treatment regimens may induce @entity5 regression. However, relapse of the disease is frequent and long-term survival is rare with the currently available therapeutic strategies.
