Difference between revisions of "SMARCB1-deficient sinonasal carcinoma"
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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>{{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== | |||
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 (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== | ||
Latest revision as of 15:42, 3 January 2020
SMARCB1-deficient sinonasal carcinoma (also INI-1-deficient carcinomas of the sinonasal tract) 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 (definitional).[1]
- Pankeratin +ve ~ 97% +ve.[1]
- CK5 +ve ~ 64% +ve.[1]
- P63 +ve ~ 55% +ve.[1]
- CK7 -ve/+ve ~ 48% +ve.[1]
- NUT -ve.[1]
See also
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 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.