Pediatric Hematology Oncology Journal (Sep 2020)

Synchronous adrenocortical carcinoma and rhabdomyosarcoma in a child with p53 Overexpression on immunostaining

  • Sonali Mohapatra,
  • Santosh Kumar Mahalik,
  • Mukund Namdev Sable,
  • Kanishka Das

DOI
https://doi.org/10.1016/j.phoj.2020.06.010
Journal volume & issue
Vol. 5, no. 3
pp. 119 – 122

Abstract

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We describe a child with synchronous malignancies. The patient presented with adrenocortical tumor (ACT) of left adrenal gland and rhabdomyosarcoma (RMS) on the left thigh and successfully treated with surgery and chemotherapy. The occurrence of these two cancers in the same child met “Chompret criteria” for Li Fraumeni syndrome (LFS), although there was no family history of cancer. Overexpression of p53 was detected by immunostaining but unfortunately, we were unable to screen for TP53 mutations. The unique presentation of our patient is described, and the significance of finding two simultaneous solid tumors in a child, with the importance of counseling for cancer predisposition syndromes is discussed.

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