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. Author manuscript; available in PMC: 2018 May 1.
Published in final edited form as: Histopathology. 2017 Feb 21;70(6):861–868. doi: 10.1111/his.13143

Table 1.

Clinicopathological characteristics of laryngeal NUT carcinomas.

No cases Author (Ref) Year Age Sex Location Initial diagnosis Basis for diagnosis and genetics Immunohistochemistry Treatment Died of Disease (months)
1 Vargas et al (13) 2001 13 F Epiglottis PDC FISH
BDR4-NUT
Trisomy 8, t(15;19)(q13;p13.1)
(+):CK QRT+Surgery (Radical neck dissection).
Palliative QT.
9
(−): AFP, CD3, CD20, CD30, CEA, Chromogranin A, Desmin, EMA, HMB-45, LCA, MSA, PLAP, SMA, S-100, EBER-ISH
2 Stelow et al (15) 2008 78 F Supraglottis UC FISH
BRD4-NUT
*(+): panCK, p63. QRT+Surgery (Laryngectomy+ neck dissection) 8
*(−): synaptophysin, chromogranin, LCA, CD19, CD30, CD15, S100, HMB45, tyrosinase, ALK, desmin, SMA, EMA, lysozyme, CD43.
3 Kundra et al (25) 2016 39 M Supraglottis HGMN NUT IHC (+ focal):CK5/6, p40, CAM 5.2, and AE-1/AE-3. NA NA
(−):CD10, CD20, CD43, CD3, CD23, BCL-2, BCL-6, MUM-1, S100, MELAN-1, MART-1, HMB-45, actin, desmin, myogenin, FLI-1, chromogranin, synaptophysin
4 Hospital for Sick Children, Toronto, Canada 2012 5 F Larynx/ base of tongue NUT Midline Carcinoma NUT IHC, fusion unknown (+)
p63, p53 (high expression)

60% loss p27
80–90% loss p21

Increased Mib-1
Uniformly NUT protein positive
(−)
p16
ISH for EBV and EBER Negative

Both T and B cells present with no clear evidence of significant antigen loss in T cells, orlight-chain restricted B cells
Tracheotomy and induction chemotherapy with HDAC inhibitor followed by bilateral neck dissections and postoperative radiationtherapy. Eventually required total laryngectomy for recurrence. 7
5 University of Chicago, Chicago, IL 2014 41 M Supraglottis Invasive basaloid SCC FISH
NUT IHC
BRD4-NUT
NUT Partial laryngectomy & neck dissection; 6.5 cycles adjuvant QRT 11
6 Auckland City Hospital, Auckland, New Zealand 2016 17 M Supraglottis NUT midline carcinoma FISH
NUT IHC
BRD4-NUT
Karyotype: 47,XY,+8,i(8)(p10),t(15;19)(q14;p13.1)[16]/46,XY[4]
(+)
p63, p40, Ki-67 (high)

(+ in 50% of cells)
p16

(+ focal)
CKAE1/AE3, High Molecular Weight CK, CK56, CD30

(−)

- CD56, Synaptophysin, Chromogranin, Calcitonin

– S100, MelanA, Sox10

– CD20, CD3, LCA(CD45)

– PLAP, CD117, SALL4, OCT ¾, HCG, AFP

– TTF1, CK7, CK20, PAX8

– Desmin, myogenin, CD34

EBER ISH was negative.
Transoral CO2 Laser Microsurgical resection; with staged Bilateral neck dissections (5 days later) – ipsilateral extended radical Neck Dissection and contralateral modified radical neck dissection with pec major flap to ipsilateral neck AWD
7 Portuguese Institute of Oncology Porto, Portugal 2015 47 M Larynx/ base of tongue Squamous cell carcinoma, poorly differentiated NUT IHC fusion unknown NUT Palliative chemotherapy with taxol and carboplatin 3 months

PDC: Poorly differentiated carcinoma, UC: Undifferentiated carcinoma, HGMN: High-Grade malignant neoplasm, IHC: Immunohistochemistry, NA: Not available, AWD: Alive with disease, QT: Chemotherapy, QRT: Chemoradiotherapy.