Abstract: Extrarenal rhabdoid tumor is a rare malignancy of infants and children, typically presenting in the soft tissue of deep, axial locations. We describe a rare dermal presentation of congenital extrarenal rhabdoid tumor in the left paraspinal region of a 6-month-old girl with germline deletion of chromosome 22q11.21q11.23. This case demonstrates that like other rhabdoid tumors, the SMARCB1 gene is also responsible for cutaneous extrarenal rhabdoid tumor oncogenesis.
Departments of *Pathology
§Molecular and Medical Genetics and Pediatrics
¶Molecular and Medical Genetics, Oregon Health and Science University, Portland, OR.
Reprints: Andrea L. Chakrapani, MD, Department of Pathology, Oregon Health and Science University (mailcode L113), 3181 SW Sam Jackson Park Road, Portland, OR 97239 (e-mail: Laueran@ohsu.edu).
The authors declare no conflicts of interest.