Eelloo et al. [140] reported a 51-year old female patient with a germline pathogenic SMARCB1 variant and multiple benign and malignant tumours including schwannomas, follicular lymphoma (WHO grade II), neurofibroma, uterine leiomyoma, MPNST and a neuroendocrine carcinoma of the kidney. This evidence concerns the gene SMARCB1 and malignant peripheral nerve sheath tumor.