Difference between revisions of "Biphenotypic sinonasal sarcoma"

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*S-100 +ve - strong (28 +ve of 28 cases<ref name=pmid22301502/>).
*S-100 +ve - strong (28 +ve of 28 cases<ref name=pmid22301502/>).
*Actin +ve (27 +ve of 28 cases<ref name=pmid22301502/>).
*Actin +ve (27 +ve of 28 cases<ref name=pmid22301502/>).
*Beta-catenin - nuclear +ve.<ref>http://dx.doi.org/10.1016/j.humpath.2016.04.009
</ref><reF>{{Cite journal  | last1 = Powers | first1 = KA. | last2 = Han | first2 = LM. | last3 = Chiu | first3 = AG. | last4 = Aly | first4 = FZ. | title = Low-grade sinonasal sarcoma with neural and myogenic features--diagnostic challenge and pathogenic insight. | journal = Oral Surg Oral Med Oral Pathol Oral Radiol | volume = 119 | issue = 5 | pages = e265-9 | month = May | year = 2015 | doi = 10.1016/j.oooo.2014.10.007 | PMID = 25488011 }}</ref>


==Molecular==
==Molecular==

Revision as of 17:49, 1 May 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 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:

Gross

  • Usually ethmoid sinus and/or nasal cavity.[3]

Microscopic

Features:

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

Note:

  • May have rhabdomyoblastic differentiation - case report.[4]

DDx:

IHC

Features:

  • S-100 +ve - strong (28 +ve of 28 cases[3]).
  • Actin +ve (27 +ve of 28 cases[3]).
  • Beta-catenin - nuclear +ve.[5][6]

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.
  2. ↑ 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. ↑ 3.0 3.1 3.2 3.3 3.4 3.5 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.
  4. ↑ Huang, SC.; Ghossein, RA.; Bishop, JA.; Zhang, L.; Chen, TC.; Huang, HY.; Antonescu, CR. (Jan 2016). "Novel PAX3-NCOA1 Fusions in Biphenotypic Sinonasal Sarcoma With Focal Rhabdomyoblastic Differentiation.". Am J Surg Pathol 40 (1): 51-9. doi:10.1097/PAS.0000000000000492. PMID 26371783.
  5. ↑ http://dx.doi.org/10.1016/j.humpath.2016.04.009
  6. ↑ 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.
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