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Difference between revisions of "Ovarian small cell carcinoma of the hypercalcemic type"
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Ovarian small cell carcinoma of the hypercalcemic type (view source)
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Small_cell_carcinoma_of_the_ovary_hypercalcemic_type_-_very_high_mag.jpg | |||
| Width = | |||
| Caption = Small cell carcinoma of the ovary hypercalcemic type. [[H&E stain]]. | |||
| Synonyms = malignant rhabdoid tumour of the ovary | |||
| Micro = small cell carcinoma (small blue cells with a high [[NC ratio]] and nuclear moulding), follicle-like spaces | |||
| Subtypes = | |||
| LMDDx = [[small cell carcinoma of the lung]], [[small cell carcinoma of the cervix]], [[Sertoli-Leydig cell tumour]], [[juvenile granulosa cell tumour]], other [[small round blue cell tumours]] | |||
| Stains = | |||
| IHC = [[WT-1]] +ve, [[TTF-1]] -ve, HPV -ve, [[p16]] +ve/-ve | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Staging = | |||
| Site = [[ovary]] - see ''[[ovarian tumours]]'' | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = typically less than 40 years old | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = very rare | |||
| Bloodwork = hypercalcemia | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = poor | |||
| Other = | |||
| ClinDDx = other [[ovarian tumours]] | |||
| Tx = | |||
}} | |||
'''Ovarian small cell carcinoma of the hypercalcemic type''', abbreviated '''OSSCHT''', is a rare [[malignant]] [[ovarian tumour]]. | '''Ovarian small cell carcinoma of the hypercalcemic type''', abbreviated '''OSSCHT''', is a rare [[malignant]] [[ovarian tumour]]. | ||
''Small cell carcinoma, hypercalcemic type'', and ''small cell carcinoma of the ovary, hypercalcemic type'' redirect here. | ''Small cell carcinoma, hypercalcemic type'', and ''small cell carcinoma of the ovary, hypercalcemic type'' redirect here. | ||
A proposed name | A proposed name for this entity is ''malignant rhabdoid tumour of the ovary'', abbreviated ''MRTO''.<ref name=pmid24752781>{{Cite journal | last1 = Foulkes | first1 = WD. | last2 = Clarke | first2 = BA. | last3 = Hasselblatt | first3 = M. | last4 = Majewski | first4 = J. | last5 = Albrecht | first5 = S. | last6 = McCluggage | first6 = WG. | title = No small surprise - small cell carcinoma of the ovary, hypercalcaemic type, is a malignant rhabdoid tumour. | journal = J Pathol | volume = 233 | issue = 3 | pages = 209-14 | month = Jul | year = 2014 | doi = 10.1002/path.4362 | PMID = 24752781 }}</ref> | ||
==General== | ==General== | ||
| Line 9: | Line 41: | ||
*Very rare. | *Very rare. | ||
*Most common undifferentiated ovarian malignancy in individuals less than 40 years old.<ref name=pmid24658002/> | *Most common undifferentiated ovarian malignancy in individuals less than 40 years old.<ref name=pmid24658002/> | ||
*Associated with SMARCA4 mutations - may be familial.<ref name=pmid24658002>{{cite journal |author=Witkowski L, Carrot-Zhang J, Albrecht S, ''et al.'' |title=Germline and somatic SMARCA4 mutations characterize small cell carcinoma of the ovary, hypercalcemic type |journal=Nat. Genet. |volume=46 |issue=5 |pages=438–43 |year=2014 |month=May |pmid=24658002 |doi=10.1038/ng.2931 |url=}}</ref> | *Associated with SMARCA4 (BRG1) mutations - may be familial.<ref name=pmid24658002>{{cite journal |author=Witkowski L, Carrot-Zhang J, Albrecht S, ''et al.'' |title=Germline and somatic SMARCA4 mutations characterize small cell carcinoma of the ovary, hypercalcemic type |journal=Nat. Genet. |volume=46 |issue=5 |pages=438–43 |year=2014 |month=May |pmid=24658002 |doi=10.1038/ng.2931 |url=}}</ref> | ||
==Microscopic== | ==Microscopic== | ||
Features:<ref name=pmid7943531/> | Features:<ref name=pmid7943531/> | ||
*Small cell carcinoma. | *Small cell carcinoma - small blue cells with a high [[NC ratio]] and nuclear moulding. | ||
*Follicle-like spaces. | *Follicle-like spaces. | ||
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==IHC== | ==IHC== | ||
Features:<ref name=pmid17727473>{{Cite journal | last1 = Carlson | first1 = JW. | last2 = Nucci | first2 = MR. | last3 = Brodsky | first3 = J. | last4 = Crum | first4 = CP. | last5 = Hirsch | first5 = MS. | title = Biomarker-assisted diagnosis of ovarian, cervical and pulmonary small cell carcinomas: the role of TTF-1, WT-1 and HPV analysis. | journal = Histopathology | volume = 51 | issue = 3 | pages = 305-12 | month = Sep | year = 2007 | doi = 10.1111/j.1365-2559.2007.02790.x | PMID = 17727473 }}</ref> | Features:<ref name=pmid17727473>{{Cite journal | last1 = Carlson | first1 = JW. | last2 = Nucci | first2 = MR. | last3 = Brodsky | first3 = J. | last4 = Crum | first4 = CP. | last5 = Hirsch | first5 = MS. | title = Biomarker-assisted diagnosis of ovarian, cervical and pulmonary small cell carcinomas: the role of TTF-1, WT-1 and HPV analysis. | journal = Histopathology | volume = 51 | issue = 3 | pages = 305-12 | month = Sep | year = 2007 | doi = 10.1111/j.1365-2559.2007.02790.x | PMID = 17727473 }}</ref> | ||
*WT-1 +ve. | *[[WT-1]] +ve. | ||
*TTF-1 -ve. | *[[TTF-1]] -ve. | ||
*HPV -ve. | *HPV -ve. | ||
*p16 +ve/-ve. | *[[p16]] +ve/-ve. | ||
==See also== | ==See also== | ||
[[Miscellaneous ovarian tumours]]. | *[[Miscellaneous ovarian tumours]]. | ||
*[[Small cell carcinoma]]. | |||
*[[Malignant rhabdoid tumour]]. | |||
==References= | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Ovarian tumours]] | [[Category:Ovarian tumours]] | ||