SMARCB1-deficient sinonasal carcinoma: Difference between revisions
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'''SMARCB1-deficient sinonasal carcinoma''' 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> | '''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== | ==Microscopic== | ||
Features:<ref name=pmid28291122/> | Features:<ref name=pmid28291122/> | ||
*Pagetoid spread. | *Pagetoid spread of plasmacytoid or [[rhabdoid]] cells. | ||
*Cyst/pseudocyst like spaces. | |||
*Lack squamous dysplasia. | |||
*Lack of carcinoma-in-situ. | |||
DDx: | DDx: | ||
*[[ | *[[Sinonasal undifferentiated carcinoma]] (SNUC). | ||
*[[Squamous cell carcinoma of the head and neck]]. | *[[Squamous cell carcinoma of the head and neck]]. | ||
*[[NUT carcinoma]]. | |||
==IHC== | ==IHC== | ||
*SMARCB1 (INI1) -ve.<ref name=pmid28291122/> | *SMARCB1 (INI1) -ve (definitional).<ref name=pmid28291122/> | ||
*Pankeratin +ve ~ 97% +ve.<ref name=pmid28291122/> | |||
*CK5 +ve ~ 64% +ve.<ref name=pmid28291122/> | |||
*P63 +ve ~ 55% +ve.<ref name=pmid28291122/> | |||
*CK7 -ve/+ve ~ 48% +ve.<ref name=pmid28291122/> | |||
*NUT -ve.<ref name=pmid28291122/> | |||
==See also== | ==See also== | ||