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Difference between revisions of "Biphenotypic sinonasal sarcoma"
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Biphenotypic sinonasal sarcoma (view source)
Revision as of 14:20, 19 November 2018
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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''' | 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. | *Rare - largest series published (as of 2012) 28 patients.<ref name=pmid22301502/> | ||
*Female predominant. | *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: | |||
*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 | *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== | ||