Biphenotypic sinonasal sarcoma

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Biphenotypic sinonasal sarcoma
Diagnosis in short

Synonyms low-grade sinonasal sarcoma with neural and myogenic features

LM bland hypercellular stroma typically without many mitoses, invaginating glandular epithelium
LM DDx solitary fibrous tumour, schwannoma, sinonasal-type hemangiopericytoma, benign nasal polyp
IHC S-100 +ve, actin +ve, beta-catenin +ve (nuclear)
Molecular PAX3-MAML3 fusion - t(2;4)(q35;q31.1)
Site head and neck - ethmoid sinus and/or nasal cavity

Clinical history female predominant (3:1)
Prevalence very rare ~ large series 28 individual as of 2012

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][2]

Others:[2]

  • Desmin -ve.
  • AE1/AE3 -ve.
  • Vimentin +ve.

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. 2.0 2.1 2.2 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