Radically different treatment recommendations for newly diagnosed XXXX : pitfalls in assessment of risk.

@entity1 @entity5 @entity66 @entity521
@entity521 risk stratification is based on stage, age, and biology and prescribes surgery for low-risk disease, moderate-dose chemotherapy for intermediate-risk disease, and maximal therapy (including myeloablative treatment with stem cell transplantation) for high-risk disease. Four cases are described that depict pitfalls in risk assessment with potentially far-reaching consequences. This report focuses on a subset of four @entity1 referred for second opinions. Stage was defined by the International @entity521 Staging System. The first recommendations were for maximal therapy, but second opinions were radically different (ie, surgery alone). Ages at diagnosis were 15 to 25 months. Shimada histopathology was unfavorable in three of the four @entity1 , but chromosomal, serum, and urine prognostic markers were favorable. All four @entity1 did well without cytotoxic therapy (follow-up: 2 years 10 months plus to 4 years 8 months plus). @entity1 1 had abdominal and upper thoracic/supraclavicular masses (stage 4); the former was resected and the latter spontaneously regressed. @entity1 2 had @entity66 , without bone marrow involvement, but imaging studies showed lesions in vertebral bodies. Biopsies of the latter showed no @entity521 and the @entity5 (with regional lymph nodes) was resected, changing stage from 4 to 2B. @entity1 3 had a retroperitoneal mass but no distant disease. Though initially deemed to be unresectable, the @entity5 was excised, changing the classification from high risk (stage 3 with unfavorable histopathology) to low risk (stage 1). @entity1 4 had a pelvic mass, with unfavorable histopathology, and bilateral inguinal lymph node involvement (stage 3); all soft tissue disease was resected. The absence of cortical bone and extensive bone marrow metastatic involvement in a young @entity521 @entity1 should cause a shift in attention to biologic prognostic markers. Some @entity1 classified as having high-risk @entity521 might actually do well with no cytotoxic therapy.
