View source for SMARCB1-deficient sinonasal carcinoma

'''SMARCB1-deficient sinonasal carcinoma''' (also '''INI-1-deficient carcinomas of the sinonasal tract''') is a rare malignant tumour of the [[head and neck pathology|head and neck]] with loss of [[SMARCB1]] staining.<ref name=pmid28291122>{{Cite journal  | last1 = Agaimy | first1 = A. | last2 = Hartmann | first2 = A. | last3 = Antonescu | first3 = CR. | last4 = Chiosea | first4 = SI. | last5 = El-Mofty | first5 = SK. | last6 = Geddert | first6 = H. | last7 = Iro | first7 = H. | last8 = Lewis | first8 = JS. | last9 = Märkl | first9 = B. | title = SMARCB1 (INI-1)-deficient Sinonasal Carcinoma: A Series of 39 Cases Expanding the Morphologic and Clinicopathologic Spectrum of a Recently Described Entity. | journal = Am J Surg Pathol | volume = 41 | issue = 4 | pages = 458-471 | month = Apr | year = 2017 | doi = 10.1097/PAS.0000000000000797 | PMID = 28291122 }}</ref>

==General==
*Rare - large series from 2017 has 39 cases.<ref name=pmid28291122/>
*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:<ref name=pmid28291122/>
*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.<ref name=pmid28291122/>

==See also==
*[[Head and neck pathology]].

==References==
{{Reflist|1}}

[[Category:Diagnosis]]