Rhabdoid tumours can occur in almost any anatomic location, commonly in the central nervous system (atypical teratoid/rhabdoid tumour). Other locations include kidneys (renal rhabdoid tumour), head and neck, paravertebral muscles, liver, bladder, mediastinum, retroperitoneum, pelvis (extracranial extrarenal malignant rhabdoid tumour) and, rarely, the ovary (small-cell carcinoma of the ovary, hypercalcaemic type).
There are limited data on the effectiveness of surveillance in children at increased risk of RTPS1. RTPS1 is extremely rare, and this limits the ability of clinical researchers to perform large prospective studies to address the utility of surveillance. In addition, there are uncertainties with regard to the natural history of SMARCB1-associated malignancies.
These recommendations represent expert opinion and are informed by a paper published by attendees of the 2017 American Association for Cancer Research (AACR) Childhood Cancer Predisposition Workshop and SIOPE host genome working group recommendations.rr