NUT carcinoma
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| NUT carcinoma | |
|---|---|
| Diagnosis in short | |
 NUT carcinoma. H&E stain. | |
|
| |
| Synonyms | NUT midline carcinoma, carcinoma with t(15;19) translocation |
|
| |
| LM | cohesive malignant cells (poorly differentiated carcinoma), islands of well-differentiated squamous epithelium |
| LM DDx | Carcinoma ex pleomorphic adenoma, squamous cell carcinoma, SMARCB1-deficient sinonasal carcinoma, acute leukemia, poorly differentiated carcinoma, |
| IHC | p63 +ve, CD34 +ve/-ve, EMA +ve, NUT +ve |
| Molecular | t(15;19) |
| Site | head and neck, mediastinum, usu. midline |
|
| |
| Prevalence | very rare |
| Prognosis | very poor |
NUT carcinoma is a rare malignant tumour classically of the head and neck. The WHO calls this tumour carcinoma with t(15;19) translocation.[1] It is also known as NUT midline carcinoma, abbreviated NMC.
General
- Not specific to any tissue type or organ.[2]
- Defined by mutation in NUT gene on chromosome 15.
- NUT = Nuclear protein in testis.[3]
Clinical:
- Usually midline - as the name of the tumour suggests.
- Case report of a NMC in the parotid gland.[4]
- Head, neck and mediastinum.[5]
- Very poor prognosis.[4]
Microscopic
- Poorly differentiated carcinoma.
- Cohesive malignant cells.
- Islands of well-differentiated squamous epithelium - key feature.
DDx:
- Squamous cell carcinoma.
- Carcinoma ex pleomorphic adenoma.
- Poorly differentiated carcinoma.
- SMARCB1-deficient sinonasal carcinoma.[6]
- Acute leukemia - especially as NUT carcinoma may be CD34 positive.[7]
Images
NUT midline carcinoma - high mag. (WC)
NUT midline carcinoma - very high mag. (WC)
Molecular
- Rearrangement of the NUT gene.[2]
IHC
- NUT +ve (key immunostain).
- CD34 +ve seen in ~50% of cases.[9]
- p63 +ve.[10]
- Rarely negative - case report.[11]
- EMA +ve.
- p16 +ve (4 of 4 cases).[12]
- Ki-67 >80% of tumour cells.[citation needed]
- CK7 +ve.[13]
Note:
- p63 +ve with p40 -ve may be suggestive.[citation needed]
See also
References
- ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 145. ISBN 978-0781765275.
- ↑ 2.0 2.1 2.2 French, CA. (Nov 2010). "NUT midline carcinoma.". Cancer Genet Cytogenet 203 (1): 16-20. doi:10.1016/j.cancergencyto.2010.06.007. PMID 20951314.
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 608963
- ↑ 4.0 4.1 den Bakker, MA.; Beverloo, BH.; van den Heuvel-Eibrink, MM.; Meeuwis, CA.; Tan, LM.; Johnson, LA.; French, CA.; van Leenders, GJ. (Aug 2009). "NUT midline carcinoma of the parotid gland with mesenchymal differentiation.". Am J Surg Pathol 33 (8): 1253-8. doi:10.1097/PAS.0b013e3181abe120. PMID 19561446.
- ↑ 5.0 5.1 5.2 French, CA. (Jun 2010). "Demystified molecular pathology of NUT midline carcinomas.". J Clin Pathol 63 (6): 492-6. doi:10.1136/jcp.2007.052902. PMID 18552174.
- ↑ Bishop, JA.; Antonescu, CR.; Westra, WH. (Sep 2014). "SMARCB1 (INI-1)-deficient carcinomas of the sinonasal tract.". Am J Surg Pathol 38 (9): 1282-9. doi:10.1097/PAS.0000000000000285. PMID 25007146.
- ↑ Li, W.; Chastain, K. (Jul 2018). "NUT midline carcinoma with leukemic presentation mimicking CD34-positive acute leukemia.". Blood 132 (4): 456. doi:10.1182/blood-2017-07-796268. PMID 30049733.
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 608749
- ↑ Stelow, EB. (Mar 2011). "A review of NUT midline carcinoma.". Head Neck Pathol 5 (1): 31-5. doi:10.1007/s12105-010-0235-x. PMID 21221870.
- ↑ Bishop, JA.; Westra, WH. (Aug 2012). "NUT midline carcinomas of the sinonasal tract.". Am J Surg Pathol 36 (8): 1216-21. doi:10.1097/PAS.0b013e318254ce54. PMID 22534723.
- ↑ Numakura S, Saito K, Motoi N, Mori T, Saito Y, Yokote F, Kanamoto Y, Asami M, Sakai T, Yamauchi Y, Sakao Y, Uozaki H, Kawamura M (November 2020). "P63-negative pulmonary NUT carcinoma arising in the elderly: a case report". Diagn Pathol 15 (1): 134. doi:10.1186/s13000-020-01053-4. PMC 7657348. PMID 33176817. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7657348/.
- ↑ Salles, PG.; Moura, Rde D.; Menezes, LM.; Bacchi, CE. (Apr 2014). "Expression of P16 in NUT carcinomas with no association with human papillomavirus (HPV).". Appl Immunohistochem Mol Morphol 22 (4): 262-5. doi:10.1097/PAI.0b013e3182a4ef2e. PMID 24185123.
- ↑ Zhu, S.; Schuerch, C.; Hunt, J. (Jan 2015). "Review and updates of immunohistochemistry in selected salivary gland and head and neck tumors.". Arch Pathol Lab Med 139 (1): 55-66. doi:10.5858/arpa.2014-0167-RA. PMID 25549144.