Pediatric NUT Carcinoma Is a Rare and Challenging Tumor: Single Center Experience of Five Children

Background Nuclear protein of the testis (NUT) carcinoma is a rare and aggressive malignancy associated with rearrangements of the nuclear protein of the testis (NUT) gene on chromosome 15q14. Because of its rarity, this tumor is often underdiagnosed and underreported, and there is limited literatur...

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Published in:The oncologist (Dayton, Ohio) Ohio), 2019-11, Vol.24 (11), p.e1232-e1235
Main Authors: Prasad, Maya, Baheti, Akshay, Ramadwar, Mukta, Chinnaswamy, Girish, Vora, Tushar, Qureshi, Sajid
Format: Article
Language:English
Subjects:
Adolescent
Adult
Brief Communications
Carcinoma, Squamous Cell - metabolism
Carcinoma, Squamous Cell - pathology
Carcinoma, Squamous Cell - therapy
Child
Combined Modality Therapy
Female
Follow-Up Studies
Humans
Male
Middle Aged
Nuclear Proteins - metabolism
Oncogene Proteins - metabolism
Prognosis
Retrospective Studies
Young Adult
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Summary:Background Nuclear protein of the testis (NUT) carcinoma is a rare and aggressive malignancy associated with rearrangements of the nuclear protein of the testis (NUT) gene on chromosome 15q14. Because of its rarity, this tumor is often underdiagnosed and underreported, and there is limited literature regarding its biology and optimal management. Methods and Results We report our experience of five patients with pediatric NUT carcinoma, all of whom presented with midline head and neck mass. In spite of aggressive multimodality treatment, only one patient survives. Conclusion NUT carcinoma has a dismal prognosis in spite of aggressive multimodality management (surgery and adjuvant chemotherapy and/or radiation). Novel strategies are required to improve outcomes of patients with this tumor. This brief communication describes the clinical characteristics and outcomes of five children with nuclear protein of the testis carcinoma (NUT) carcinoma.
ISSN:1083-7159
1549-490X
DOI:10.1634/theoncologist.2019-0358