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 ovary (small-cell carcinoma of the ovary).
There are limited data on the effectiveness of surveillance in children at increased risk of RTPS. RTPS 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 carcinomas.
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.r