Difference between revisions of "Biphenotypic sinonasal sarcoma"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Biphenotypic Sinonasal Sarcoma LDRT 12255.tif
| Width      =
| Caption    = [[Micrograph]] showing a biphenotypic sinonasal sarcoma. [[H&E stain]]. (WC/LESTERTHEINVESTOR)
| Synonyms  = low-grade sinonasal sarcoma with neural and myogenic features
| Micro      = bland hypercellular stroma typically without many mitoses, invaginating glandular epithelium
| Subtypes  =
| LMDDx      = [[solitary fibrous tumour]], [[schwannoma]], [[sinonasal-type hemangiopericytoma]], benign [[nasal polyp]]
| Stains    =
| IHC        = S-100 +ve, actin +ve, beta-catenin +ve (nuclear)
| EM        =
| Molecular  = PAX3-MAML3 fusion - t(2;4)(q35;q31.1)
| IF        =
| Gross      =
| Grossing  =
| Staging    =
| Site      = [[head and neck pathology|head and neck]] - ethmoid sinus and/or nasal cavity
| Assdx      =
| Syndromes  =
| Clinicalhx = female predominant (3:1)
| Signs      =
| Symptoms  =
| Prevalence = very rare ~ large series 28 individuals as of 2012
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
| Tx        =
}}
'''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]].
'''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 sinonasal sarcoma with neural and myogenic features'''.<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>
It is also known as '''low-grade sinonasal sarcoma with neural and myogenic features'''.<ref name=pmid25488011>{{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>


==General==
==General==
*Rare - largest series published (as of 2012) 28 patients.<ref name=pmid22301502/>
*Rare - largest series published (as of 2012) 28 patients.<ref name=pmid22301502/>
*Female predominant - (female:male = 3:1).<ref name=pmid22301502>{{Cite journal  | last1 = Lewis | first1 = JT. | last2 = Oliveira | first2 = AM. | last3 = Nascimento | first3 = AG. | last4 = Schembri-Wismayer | first4 = D. | last5 = Moore | first5 = EA. | last6 = Olsen | first6 = KD. | last7 = Garcia | first7 = JG. | last8 = Lonzo | first8 = ML. | last9 = Lewis | first9 = JE. | title = Low-grade sinonasal sarcoma with neural and myogenic features: a clinicopathologic analysis of 28 cases. | journal = Am J Surg Pathol | volume = 36 | issue = 4 | pages = 517-25 | month = Apr | year = 2012 | doi = 10.1097/PAS.0b013e3182426886 | PMID = 22301502 }}</ref>
*Female predominant - (female:male = 3:1<ref name=pmid22301502>{{Cite journal  | last1 = Lewis | first1 = JT. | last2 = Oliveira | first2 = AM. | last3 = Nascimento | first3 = AG. | last4 = Schembri-Wismayer | first4 = D. | last5 = Moore | first5 = EA. | last6 = Olsen | first6 = KD. | last7 = Garcia | first7 = JG. | last8 = Lonzo | first8 = ML. | last9 = Lewis | first9 = JE. | title = Low-grade sinonasal sarcoma with neural and myogenic features: a clinicopathologic analysis of 28 cases. | journal = Am J Surg Pathol | volume = 36 | issue = 4 | pages = 517-25 | month = Apr | year = 2012 | doi = 10.1097/PAS.0b013e3182426886 | PMID = 22301502 }}</ref>).


Prognosis:
Prognosis:
*No (far away) [[metastasis]] of death in a series of 28 patients.<ref name=pmid22301502/>
*No (far away) [[metastasis]] or death in a series of 28 patients.<ref name=pmid22301502/>
 
==Gross==
*Usually ethmoid sinus and/or nasal cavity.<ref name=pmid22301502/>


==Microscopic==
==Microscopic==
Features;
Features:
*Bland hypercellular stroma typically without many mitoses.
*Bland hypercellular stroma typically without many mitoses.
*Invaginating glandular epithelium.  
*Invaginating glandular epithelium.  
Note:
*May have rhabdomyoblastic differentiation - case report.<ref name=pmid26371783>{{Cite journal  | last1 = Huang | first1 = SC. | last2 = Ghossein | first2 = RA. | last3 = Bishop | first3 = JA. | last4 = Zhang | first4 = L. | last5 = Chen | first5 = TC. | last6 = Huang | first6 = HY. | last7 = Antonescu | first7 = CR. | title = Novel PAX3-NCOA1 Fusions in Biphenotypic Sinonasal Sarcoma With Focal Rhabdomyoblastic Differentiation. | journal = Am J Surg Pathol | volume = 40 | issue = 1 | pages = 51-9 | month = Jan | year = 2016 | doi = 10.1097/PAS.0000000000000492 | PMID = 26371783 }}</ref>


DDx:
DDx:
*[[Solitary fibrous tumour]].
*[[Solitary fibrous tumour]].
*[[Schwannoma]].
*[[Schwannoma]].
*[[hemangiopericytoma]].
*[[Sinonasal-type hemangiopericytoma]].
*Benign [[nasal polyp]].
 
===Images===
<gallery>
Image: Biphenotypic Sinonasal Sarcoma LDRT 12255.tif | BSS. (WC/LESTERTHEINVESTOR)
Image: Biphenotypic Sinonasal Sarcoma LDRT 12256.tif | BSS. (WC/LESTERTHEINVESTOR)
</gallery>


==IHC==
==IHC==
*Actin +ve.
Features:
*S-100 +ve (strong).
*S-100 +ve - strong (28 +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 name=pmid25488011>{{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>
 
Others:<ref name=pmid25488011/>
*Desmin -ve.
*[[AE1/AE3]] -ve.
*Vimentin +ve.
 
===Images===
<gallery>
Image: Biphenotypic Sinonasal Sarcoma LDRT 12255 S100.tif | BSS S-100. (WC/LESTERTHEINVESTOR)
Image: Biphenotypic Sinonasal Sarcoma LDRT 12255 SMA.tif | BSS - SMA. (WC/LESTERTHEINVESTOR)
</gallery>


==Molecular==
==Molecular==
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==See also==
==See also==
*[[Head and neck pathology]].
*[[Head and neck pathology]].
*[[HPV-related multiphenotypic sinonasal carcinoma]].


==References==
==References==

Latest revision as of 14:20, 19 November 2018

Biphenotypic sinonasal sarcoma
Diagnosis in short

Micrograph showing a biphenotypic sinonasal sarcoma. H&E stain. (WC/LESTERTHEINVESTOR)
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 individuals 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:

Images

  • BSS. (WC/LESTERTHEINVESTOR)

  • BSS. (WC/LESTERTHEINVESTOR)

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.

Images

  • BSS S-100. (WC/LESTERTHEINVESTOR)

  • BSS - SMA. (WC/LESTERTHEINVESTOR)

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
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