Biphenotypic sinonasal sarcoma

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Biphenotypic sinonasal sarcoma 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. ↑ 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.
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