Atypical Teratoid/Rhabdoid Tumor: Surgery

Abstract

To obtain tumor samples for histological examination and for genetic investigations, to debulk the tumor mass to decompress the surrounding neural structures and to favor the adjuvant treatments, and to treat the possibly associated hydrocephalus are the main goals of surgery when dealing with atypical teratoid/rhabdoid tumor (AT/RT). The extent of tumor resection should be radical or as large as possible to relief from preoperative clinical signs and to extend the patient’s survival, which remains very short in this highly malignant tumor. Unfortunately, the huge size of the tumor at diagnosis and the young age of the affected children may prevent a massive tumor excision so that, in some instances, it could be advisable to resect the tumor in two or more steps, in order to obtain a satisfactory mass reduction safely. Such a strategy bears also a minor risk of postoperative neurological deficits. Indeed, the surgical removal of AT/RT carries a high risk of surgically related complications since the tumor infiltration may preclude the functional preservation of the surrounding parenchyma. Gross total tumor resection can currently be obtained in about 70 % of cases. The surgical mortality rate is 1–3 %; the postoperative morbidity ranges from 5–10 % to 30 % in case of AT/RT involving the cerebellopontine angle.