Biphenotypic sinonasal sarcoma
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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 sinonasal sarcoma with neural and myogenic features.[2]
General
- Rare - largest series published (as of 2012) 28 patients.[3]
- Female predominant - (female:male = 3:1).[3]
Prognosis:
- No (far away) metastasis of death in a series of 28 patients.[3]
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
- ↑ 1.0 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.
- ↑ Powers, KA.; Han, LM.; Chiu, AG.; Aly, FZ. (May 2015). "Low-grade sinonasal sarcoma with neural and myogenic features--diagnostic challenge and pathogenic insight.". Oral Surg Oral Med Oral Pathol Oral Radiol 119 (5): e265-9. doi:10.1016/j.oooo.2014.10.007. PMID 25488011.
- ↑ 3.0 3.1 3.2 Lewis, JT.; Oliveira, AM.; Nascimento, AG.; Schembri-Wismayer, D.; Moore, EA.; Olsen, KD.; Garcia, JG.; Lonzo, ML. et al. (Apr 2012). "Low-grade sinonasal sarcoma with neural and myogenic features: a clinicopathologic analysis of 28 cases.". Am J Surg Pathol 36 (4): 517-25. doi:10.1097/PAS.0b013e3182426886. PMID 22301502.