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Dramatic response to nivolumab in xeroderma pigmentosum skin tumor
Author(s) -
Chambon Fanny,
Osdoit Sophie,
Bagny Kelly,
Moro Anne,
Nguyen Jacqueline,
Réguerre Yves
Publication year - 2018
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.26837
Subject(s) - nivolumab , xeroderma pigmentosum , medicine , immunotherapy , scalp , skin cancer , dermatology , cancer research , melanoma , basal cell , oncology , cancer , pathology , dna repair , dna , biology , genetics
We report the case of a 6‐year‐old female with xeroderma pigmentosum (XP) who developed a nonoperable scalp tumor, treated with anti‐programmed cell death protein 1 (anti‐PD‐1) therapy (nivolumab). She presented with a sarcomatoid carcinoma of the scalp with bone lysis as well as vascular and meningeal contact. Nivolumab was initiated because it has emerged as a promising immunotherapy. We observed a dramatic tumor response with excellent tolerance. However, while on nivolumab therapy she developed two large skin melanomas and several squamous cell carcinomas, which have been resected. These results demonstrate that cancer immunotherapy in patients with XP can be impressive but complex and warrants further investigation.