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Multimodal Management of Congenital Orbital Malignant Rhabdoid Tumor

Review of Literature and Report of a Rare Case

Biswas, Ahitagni MD, DNB, ECMO, FRCR*; Kumar, Ritesh MD*; Bakhshi, Sameer MD; Sen, Seema MD; Sharma, Mehar C. MD, FRCPath§

Journal of Pediatric Hematology/Oncology: January 04, 2019 - Volume Publish Ahead of Print - Issue - p
doi: 10.1097/MPH.0000000000001402
Clinical and Laboratory Observations: PDF Only

Background: Malignant rhabdoid tumor (MRT) is a rare and aggressive tumor with a dismal prognosis. It commonly arises in the brain (65%), soft tissues (26%), and the kidney (9%). Primary orbital involvement is extremely rare. Although it has been mostly described in children below 2 years old, presentation at birth is sparsely reported.

Observation: We have described a case of congenital orbital MRT, who presented with rapidly progressive right-sided proptosis and was initially treated with subtotal resection and postoperative chemotherapy with ICE (Ifosfamide, Carboplatin, Etoposide) regimen. On local progression the child was treated with palliative radiotherapy (20 Gy) to the right orbit and second-line chemotherapy with VAC (Vincristine, Adriamycin, Cyclophosphamide) regimen. Unfortunately he died due to progressive disease 4 months after the initial diagnosis.

Conclusions: This report highlights the importance of awareness of orbital MRT as a differential diagnosis of rapidly progressing proptosis in the neonatal period. This tumor is often refractory to conventional multimodality treatment and more intensive and innovative treatment approaches are clearly needed in future.

*Departments of Radiotherapy and Oncology

Medical Oncology

Ocular Pathology

§Pathology, All India Institute of Medical Sciences, New Delhi, India

The authors declare no conflict of interest.

Reprints: Ahitagni Biswas, MD, DNB, ECMO, FRCR, Department of Radiotherapy and Oncology, All India Institute of Medical Sciences, New Delhi 110029, India (e-mail:

Received July 15, 2018

Accepted November 8, 2018

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