NUT carcinoma of the mandible in a child: case report and systematic review. Issue 3 (March 2023)

• Record Type: Journal Article
• Authors: Jimenez, C.; Stanton, E.; Kondra, K.; Nickels, E.M.; Jacob, L.; Shah, R.; Hammoudeh, J.A.
• Is Part Of: International journal of oral & maxillofacial surgery. Volume 52:Issue 3(2023)
• Page Start: 304
• Page End: 312
• Publication Date: 2023-03
• Subjects: Nuclear proteins -- Mandibular neoplasms -- Carcinoma -- Child -- Head and neck neoplasms -- Systematic review; Mouth -- Surgery -- Periodicals; Maxilla -- Surgery -- Periodicals; Dentistry -- Periodicals; Dentistry, Operative; Oral Surgical Procedures; Surgery, Oral; Dentistry; Maxilla -- Surgery; Mouth -- Surgery; Electronic journals; Periodicals; 617.52059
• DOI: 10.1016/j.ijom.2022.07.002
• Languages: English
• ISSNs: 0901-5027

Abstract

Abstract: Nuclear protein of the testis (NUT) carcinoma is a rare, undifferentiated carcinoma that is characterized by NUTM1 gene rearrangements. Patients with NUT carcinoma have an overall survival of approximately 5% at 5 years despite a multimodal treatment approach. This report illustrates the management of mandibular NUT carcinoma in a pediatric patient, complemented by a systematic review of head and neck NUT carcinoma. A 5-year-old female presented with an enlarging jaw mass that was diagnosed as BRD4-NUTM1 carcinoma and was treated with hemimandibulectomy and chemoradiation. She remains disease-free 21 months after completion of therapy. A total of 63 patient cases reported in 34 articles were identified in the review. Only 26.9% (14/52) of tumors were correctly diagnosed initially as NUT carcinoma, whereas 73.1% (38/52) were incorrectly diagnosed as another malignancy; the initial diagnosis was not reported for 11 patients. The mandibular tumor subtype was among the rarest reported ( n = 1; 1.6%). Combination therapy, including surgery and chemoradiation, was the most common treatment (55.2%). The patient case presented here is a novel case of pediatric mandibular NUT carcinoma. Due to the poor overall survival of patients with NUT carcinoma, aggressive upfront resection with 2-cm margins followed by adjuvant chemoradiation is advocated.