ABSTRACT
Results The median survival of patients ranges from 84 to 88 months.64,65 (Table 7.8). Approximately 70% of patients will develop local recurrence, and 40% will develop lymph node metastases. Neither of these impact on tumor mortality. Pulmonary metastases will develop in 21-40% of patients, and this is the predominant cause of death in these patients.
Diagnosis Desmoplastic small round cell tumors are rare, very aggressive tumors, usually occurring in adolescence and early adulthood. Our experience has been described.68 The median age at diagnosis is 22 years. Most patients present with non-specific symptoms, usually confined to the abdomen, with increasing abdominal girth, malaise, and palpable mass. The majority have an intra-abdominal or pelvic origin, and, at the time of presentation, disease will be very extensive, involving all of the peritoneal surfaces, the
