Abstract
Rearrangements in the nuclear protein in testis (NUT) gene cause carcinomas that represent a rare but aggressive tumor type that often present at advanced stages in midline structures. Survival rarely exceeds 12 months from the time of diagnosis. There have been no reports of a primary cardiac presentation, and few studies have reported on the numerous treatment strategies. Given their aggressive and invasive nature, NUT midline carcinomas present a therapeutic dilemma. Treatment may include surgical resection, chemotherapy, or radiotherapy, but no consistently successful treatment has been established. Surgical resection is indicated to reduce symptomatic mass effect whenever present. Novel therapies with bromodomain extra-terminal inhibitors may be associated with potential survival benefit. Here, we describe an unusual presentation of this tumor. Literature review with management considerations is underlying.
References
Ball A, Bromley A, Glaze S, French CA, Ghatage P, Köbel M (2012) A rare case of NUT midline carcinoma. Gynecol Oncol Case Rep 3:1–3
Bauer DE, Mitchell CM, Strait KM, Lathan CS, Stelow EB, Lüer SC, Muhammed S, Evans AG, Sholl LM, Rosai J, Giraldi E, Oakley RP, Rodriguez-Galindo C, London WB, Sallan SE, Bradner JE, French CA (2012) Clinicopathologic features and long-term outcomes of NUT midline carcinoma. Clin Cancer Res 18(20):5773–5779
Evans AG, French CA, Cameron MJ, Fletcher CD, Jackman DM, Lathan CS, Sholl LM (2012) Pathologic characteristics of NUT midline carcinoma arising in the mediastinum. Am J Surg Pathol 36(8):1222–1227
French C (2014) NUT midline carcinoma. Nat Rev Cancer 14(3):149–150
French CA, Kutok JL, Faquin WC, Toretsky JA, Antonescu CR, Griffin CA, Nose V, Vargas SO, Moschovi M, Tzortzatou-Stathopoulou F, Miyoshi I, Perez-Atayde AR, Aster JC, Fletcher JA (2004) Midline carcinoma of children and young adults with NUT rearrangement. J Clin Oncol 22(20):4135–4139
Maher OM, Christensen AM, Yedururi S, Bell D, Tarek N (2015) Histone deacetylase inhibitor for NUT midline carcinoma. Pediatr Blood Cancer 62(4):715–717
Marx GA, Moran AM (2008) Cardiac Tumors. In: Allen HD, Driscol DJ, Shaddy RE, Feltes TF (eds) Heart disease in infants, children, and adolescents, including the fetus and young adult. Lippincott Williams and Wilkins, Philadelphia, pp 1549–1564
Nakamura H, Tsuta K, Tsuda H, Katsuya Y, Naka G, Iizuka T, Igari T (2015) NUT midline carcinoma of the mediastinum showing two types of poorly differentiated tumor cells: a case report and a literature review. Pathol Res Pract 211(1):92–98
Nelson BA, Lee EY, French CA, Bauer DE, Vargas SO (2010) BRD4-NUT carcinoma of the mediastinum in a pediatric patient: multidetector computed tomography imaging findings. J Thorac Imaging 25(3):W93–W96
Oh SJ, Yeom SY, Kim KH (2013) Clinical implication of surgical resection for the rare cardiac tumors involving heart and great vessels. J Korean Med Sci 28(5):717–724
Padalino MA, Basso C, Milanesi O, Vida VL, Moreolo GS, Thiene G, Stellin G (2005) Surgically treated primary cardiac tumors in early infancy and childhood. J Thorac Cardiovasc Surg 129(6):1358–1363
Parikh SA, French CA, Costello BA, Marks RS, Dronca RS, Nerby CL, Roden AC, Peddareddigari VG, Hilton J, Shapiro GI, Molina JR (2013) NUT midline carcinoma: an aggressive intrathoracic neoplasm. J Thorac Oncol 8(10):1335–1338
Tensha Therapeutics (2015) Tensha therapeutics: therapeutic program, bromodomain opportunity, TEN-010, clinical program. Tensha Therapeutics. Retrieved 26 July 2015, from http://www.tenshatherapeutics.com/program-ten-010.php
Teo M, Crotty P, O’Sullivan M, French CA, Walshe JM (2011) NUT midline carcinoma in a young woman. J Clin Oncol 29(12):e336–e339
Tutak E, Satar M, Ozbarlas N, Uğuz A, Yapicioğlu H, Narli N, Bayram I (2008) A newborn infant with intrapericardial rhabdomyosarcoma: a case report. Turk J Pediatr 50(2):179–181
U.S. National Institutes of Health (2015) A two part, multicenter, open-label study of TEN-010 given subcutaneously. U.S. National Institutes of Health. Retrieved 26 July 2015, from https://www.clinicaltrials.gov/ct2/show/NCT01987362
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors have no conflict of interest to report.
Rights and permissions
About this article
Cite this article
Samples, S., Gleditsch, K. & Polimenakos, A. Intrapericardial NUT Midline Carcinoma: Unusual Presentation of a Rare Tumor and Literature Review with Management Considerations. Pediatr Cardiol 37, 208–211 (2016). https://doi.org/10.1007/s00246-015-1313-3
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-015-1313-3