Atypical Teratoid Rhabdoid Tumor - Prognosis

Prognosis

The prognosis for AT/RT has been very poor, although there are some indications that an IRSIII-based therapy can produce long-term survival (60 to 72 months). Two-year survival is less than 20%, average survival postoperatively is 11 months, and doctors often recommend palliative care, especially with younger children because of the poor outcomes. Recently a protocol utilized by a multicenter trial reported in the Journal of Clinical Oncology resulted in a 70% survival rate at 2–3 years, with most relapses occurring within months, leading to hope that there is a point beyond which patients can be considered cured.

Patients with metastasis (disseminated tumor), larger tumors, tumors that could not be fully removed, or tumor recurrence, and who were younger than 36 months had the worst outcomes (i.e., shorter survival times).

A retrospective survey from 36 AT/RT St. Jude Children's Hospital patients from 1984 to 2003 showed that the Two-year event-free survival (EFS) for children under three was 11%, the overall survival (OS) rate was 17%. For children aged 3 years or older the EFS was 78% and the OS 89%. A retrospective register at the Cleveland Children's hospital on 42 AT/RT patients found median survival time is 16.25 months and a survival rate around 33%. One-quarter of these cases did not show the mutation in the INI1/hSNF5 gene.

The longest term survivals reported in the literature are:

• (a) Hilden and associates reported a child who was still free from disease at 46 months from diagnosis.
• (b) Olson and associates reported a child who was disease free at five years from diagnosis based on the IRS III protocol.
• (c) In 2003 Hirth reported a case who had been disease free for over six years.
• (d) Zimmerman in 2005 reported 50-to-72 month survival rates on four patients using an IRS III-based protocol. Two of these long term survivors had been treated after an AT/RT recurrence.
• (e) A NYU study (Gardner 2004) has 4 of 12 longer term AT/RT survivors; the oldest was alive at 46 months after diagnosis.
• (f) Aurélie Fabre, 2004, reported a 16-year survivor of a soft-tissue rhabdoid tumor.

Cancer treatments in long-term survivors who are children usually cause a series of negative effects on physical well being, fertility, cognition, and learning.