Atypical teratoid rhabdoid tumor surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

Surgery plays a critical role in obtaining tissue to make an accurate diagnosis. Surgery alone is not curative. In addition, 30% of the AT/RTs are located supratentorially and there is a predilection for the cerebello-pontine angle which makes surgical resection difficult. One-third or more children will have disseminated disease at the time of diagnosis. Total or near-total resections are often not possible.

Surgery

• Surgery is the mainstay of treatment for atypical teratoid rhabdoid tumor.
• Surgery plays a critical role in obtaining tissue to make an accurate diagnosis. Surgery alone is not curative.
• In addition, 30% of the atypical teratoid rhabdoid tumors are located supratentorially and there is a predilection for the cerebello-pontine angle, which makes surgical resection difficult. One-third or more children will have disseminated disease at the time of diagnosis. Total or near-total resections are often not possible.

Stem Cell Transplantation

• Stem cell transplantation is indicated in very aggressive atypical teratoid rhabdoid tumors, to receive high doses of chemotherapy.