Treatment of unresectable malignant rhabdoid tumor of the orbit with tandem high‐dose chemotherapy and gamma‐knife radiosurgery

Abstract

Malignant rhabdoid tumor (MRT) is a rare and highly aggressive tumor that primarily occurs in very young children. We report here a patient with a primary MRT of the orbit who received tandem high-dose chemotherapy and gamma-knife radiosurgery. Although the tumor was not completely removed, and the initial chemotherapy failed, the patient achieved long-term survival after this modality of treatment. This approach may be one to be further considered in patients with MRT.

A Case of Congenital Orbital Malignant Rhabdoid Tumor: Systemic Metastasis following Exenteration

“…Interestingly, two previous reports of congenital MRT confined to the orbit showed good prognosis after combined treatment: chemotherapy followed by surgical resection of residual tumor, or Gamma Knife radiosurgery after tumor burden reduction with chemotherapy [6, 7]. Briefly, the former case was treated with three cycles of the ICE (ifosfamide, carboplatin, and etoposide) regimen following monthly intrathecal injections of methotrexate and cytarabine and high-dose chemotherapy in advance of surgical resection of the tumor, and completed by three additional cycles of chemotherapy with the same regimen [6].…”

“…Although MRT characteristically has an early age at onset, MRT occasionally presenting at birth is referred to as congenital. There have been only two published reports of cases with congenital MRT confined to the orbit [6, 7]. Interestingly, both of these patients had a good outcome, showing complete remission after chemotherapy followed by surgical resection and chemotherapy with Gamma Knife radiosurgery, respectively.…”

Malignant rhabdoid tumors of soft tissues in children. Literature review

“…В одной из них C. Madigan et al показана длительная БСВ у 5 (5/14) больных, 4 из которых прошли полное удаление опухоли, тогда как пациенты, имеющие отдаленные метастазы и/или невозможность удаления первич-ного очага, погибли [85]. Встречаются публикации, метод локального контроля в которых включает ис-пользование гамма-ножа в случае невозможности радикального хирургического лечения [86]. Таким образом, хирургические опции терапии с макси-мально возможной радикальностью должны быть, безусловно, использованы в лечении пациентов со ЗРО.…”

Malignant rhabdoid tumor of the orbit

“…Literatürde yer alan olgularda kemoterapi ve radyoterapi yanıtı kötü olmuş, çoğu hasta erken dönemde kaybedilmiştir. Yaşa-yan hastalar, kemoterapi ve radyoterapi ile birlikte yeterli cerrahi yapılabilen olgulardır (9,13,14). Cerrahi tedavide negatif sınır sağlanabilmesi hedeflenir ancak enükleasyon ve egzanterasyon ilk planda tercih edilecek bir yöntem değildir.…”