SMARCB1-deficient sinonasal carcinoma
SMARCB1-deficient sinonasal carcinoma is a rare malignant tumour of the head and neck with loss of SMARCB1 staining.[1]
General
- Rare - large series from 2017 has 39 cases.[1]
- Large age range (19-89 years old).
- Often present at high stage T3 or T4.
- Aggressive clinical course.
Gross
- Most often in ethmoid sinus +/- nasal cavity.
Microscopic
Features:[1]
- Pagetoid spread of plasmacytoid or rhabdoid cells.
- Cyst/pseudocyst like spaces.
- Lack squamous dysplasia.
- Lack of carcinoma-in-situ.
DDx:
- Sinonasal undifferentiated carcinoma (SNUC).
- Squamous cell carcinoma of the head and neck.
- NUT carcinoma.
IHC
- SMARCB1 (INI1) -ve.[1]
See also
References
- ↑ 1.0 1.1 1.2 1.3 Agaimy, A.; Hartmann, A.; Antonescu, CR.; Chiosea, SI.; El-Mofty, SK.; Geddert, H.; Iro, H.; Lewis, JS. et al. (Apr 2017). "SMARCB1 (INI-1)-deficient Sinonasal Carcinoma: A Series of 39 Cases Expanding the Morphologic and Clinicopathologic Spectrum of a Recently Described Entity.". Am J Surg Pathol 41 (4): 458-471. doi:10.1097/PAS.0000000000000797. PMID 28291122.