Pediatric NUT-midline carcinoma: Therapeutic success employing a sarcoma based multimodal approach.

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Pediatric NUT-midline carcinoma: Therapeutic success employing a sarcoma based multimodal approach.

Pediatr Hematol Oncol. 2017 May;34(4):231-237

Authors: Storck S, Kennedy AL, Marcus KJ, Teot L, Vaughn J, Gnekow AK, Märkl B, Leuschner I, DuBois SG, French CA, Frühwald MC

Abstract
A subset of poorly differentiated squamous cell carcinomas, NUT midline carcinomas (NMC) are characterized by a translocation t(15;19)(q13;p13) [ 1 ]. The prognosis is generally dismal [ 2 ] and therapeutic success has been limited to exceptional cases [ 3 ]. We present two cases of pediatric NMC from two different institutions treated according to a multimodal sarcoma approach involving surgery, chemotherapy, and focal radiotherapy. One patient has remained in complete continuous remission for over 6 years, while the other is in CR in early follow-up off therapy. Our proposed multimodal strategy apparently meets the aggressive biologic nature of NMC and should be considered for further evaluation in this context potentially in the setting of a clinical trial.

PMID: 29040054 [PubMed - indexed for MEDLINE]

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