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 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''' 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==
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DDx:
DDx:
*[[SNUC]].
*[[Sinonasal undifferentiated carcinoma]] (SNUC).
*[[Squamous cell carcinoma of the head and neck]].
*[[Squamous cell carcinoma of the head and neck]].



Revision as of 04:33, 18 March 2019

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:

IHC

  • SMARCB1 (INI1) -ve.[1]

See also

References

  1. 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.