Difference between revisions of "Biphenotypic sinonasal sarcoma"

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'''Biphenotypic sinonasal sarcoma''' is a rare bland appearing [[cancer|malignant]] tumour of the [[head and neck pathology|head and neck]].
'''Biphenotypic sinonasal sarcoma''', abbreviated '''SNS''',<ref name=pmid24859338/> is a rare bland appearing [[cancer|malignant]] tumour of the [[head and neck pathology|head and neck]].


It is also known as '''low grade sinonasla sarcoma with neural and myogenic features''', abbreviated '''LGSSNMF'''.
It is also known as '''low grade sinonasla sarcoma with neural and myogenic features''', abbreviated '''LGSSNMF'''.

Revision as of 16:08, 13 March 2016

Biphenotypic sinonasal sarcoma, abbreviated SNS,[1] is a rare bland appearing malignant tumour of the head and neck.

It is also known as low grade sinonasla sarcoma with neural and myogenic features, abbreviated LGSSNMF.

General

  • Rare.
  • Female predominant.

Microscopic

Features;

  • Bland hypercellular stroma typically without many mitoses.
  • Invaginating glandular epithelium.

DDx:

IHC

  • Actin +ve.
  • S-100 +ve (strong).

Molecular

  • PAX3-MAML3 fusion - t(2;4)(q35;q31.1).[1]

See also

References

  1. 1.0 1.1 Wang, X.; Bledsoe, KL.; Graham, RP.; Asmann, YW.; Viswanatha, DS.; Lewis, JE.; Lewis, JT.; Chou, MM. et al. (Jul 2014). "Recurrent PAX3-MAML3 fusion in biphenotypic sinonasal sarcoma.". Nat Genet 46 (7): 666-8. doi:10.1038/ng.2989. PMID 24859338.