Difference between revisions of "Ovarian small cell carcinoma of the hypercalcemic type"
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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]]. | |||
''Small cell carcinoma, hypercalcemic type'', and ''small cell carcinoma of the ovary, hypercalcemic type'' redirect here. | |||
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== | |||
*Hypercalcemia.<ref name=pmid7943531>{{Cite journal | last1 = Young | first1 = RH. | last2 = Oliva | first2 = E. | last3 = Scully | first3 = RE. | title = Small cell carcinoma of the ovary, hypercalcemic type. A clinicopathological analysis of 150 cases. | journal = Am J Surg Pathol | volume = 18 | issue = 11 | pages = 1102-16 | month = Nov | year = 1994 | doi = | PMID = 7943531 }}</ref> | |||
*Very rare. | |||
*Most common undifferentiated ovarian malignancy in individuals less than 40 years old.<ref name=pmid24658002/> | |||
*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== | |||
Features:<ref name=pmid7943531/> | |||
*Small cell carcinoma - small blue cells with a high [[NC ratio]] and nuclear moulding. | |||
*Follicle-like spaces. | |||
DDx: | |||
*[[Small cell carcinoma of the lung]]. | |||
*[[Small cell carcinoma of the cervix]]. | |||
*[[Sertoli-Leydig cell tumour]]. | |||
*[[Juvenile granulosa cell tumour]]. | |||
===Images=== | |||
www: | |||
*[http://webpathology.com/image.asp?n=6&Case=531 OSSCHT - low mag. (webpathology.com)]. | |||
*[http://webpathology.com/image.asp?case=531&n=5 OSSCHT - high mag. (webpathology.com)]. | |||
<gallery> | |||
Image:Small_cell_carcinoma_of_the_ovary_hypercalcemic_type_-_low_mag.jpg | OSSCHT - low mag. (WC) | |||
Image:Small_cell_carcinoma_of_the_ovary_hypercalcemic_type_-_intermed_mag.jpg | OSSCHT - intermed. mag. (WC) | |||
Image:Small_cell_carcinoma_of_the_ovary_hypercalcemic_type_-_high_mag.jpg | OSSCHT - high mag. (WC) | |||
Image:Small_cell_carcinoma_of_the_ovary_hypercalcemic_type_-_very_high_mag.jpg | OSSCHT - very high mag. (WC) | |||
</gallery> | |||
==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> | |||
*[[WT-1]] +ve. | |||
*[[TTF-1]] -ve. | |||
*HPV -ve. | |||
*[[p16]] +ve/-ve. | |||
==See also== | |||
*[[Miscellaneous ovarian tumours]]. | |||
*[[Small cell carcinoma]]. | |||
*[[Malignant rhabdoid tumour]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Ovarian tumours]] |
Latest revision as of 15:35, 8 September 2019
Ovarian small cell carcinoma of the hypercalcemic type | |
---|---|
Diagnosis in short | |
Small cell carcinoma of the ovary hypercalcemic type. H&E stain. | |
| |
Synonyms | malignant rhabdoid tumour of the ovary |
| |
LM | small cell carcinoma (small blue cells with a high NC ratio and nuclear moulding), follicle-like spaces |
LM DDx | 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 |
IHC | WT-1 +ve, TTF-1 -ve, HPV -ve, p16 +ve/-ve |
Site | ovary - see ovarian tumours |
| |
Clinical history | typically less than 40 years old |
Prevalence | very rare |
Blood work | hypercalcemia |
Prognosis | poor |
Clin. DDx | other ovarian tumours |
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.
A proposed name for this entity is malignant rhabdoid tumour of the ovary, abbreviated MRTO.[1]
General
- Hypercalcemia.[2]
- Very rare.
- Most common undifferentiated ovarian malignancy in individuals less than 40 years old.[3]
- Associated with SMARCA4 (BRG1) mutations - may be familial.[3]
Microscopic
Features:[2]
- Small cell carcinoma - small blue cells with a high NC ratio and nuclear moulding.
- Follicle-like spaces.
DDx:
- Small cell carcinoma of the lung.
- Small cell carcinoma of the cervix.
- Sertoli-Leydig cell tumour.
- Juvenile granulosa cell tumour.
Images
www:
IHC
Features:[4]
See also
References
- ↑ Foulkes, WD.; Clarke, BA.; Hasselblatt, M.; Majewski, J.; Albrecht, S.; McCluggage, WG. (Jul 2014). "No small surprise - small cell carcinoma of the ovary, hypercalcaemic type, is a malignant rhabdoid tumour.". J Pathol 233 (3): 209-14. doi:10.1002/path.4362. PMID 24752781.
- ↑ 2.0 2.1 Young, RH.; Oliva, E.; Scully, RE. (Nov 1994). "Small cell carcinoma of the ovary, hypercalcemic type. A clinicopathological analysis of 150 cases.". Am J Surg Pathol 18 (11): 1102-16. PMID 7943531.
- ↑ 3.0 3.1 Witkowski L, Carrot-Zhang J, Albrecht S, et al. (May 2014). "Germline and somatic SMARCA4 mutations characterize small cell carcinoma of the ovary, hypercalcemic type". Nat. Genet. 46 (5): 438–43. doi:10.1038/ng.2931. PMID 24658002.
- ↑ Carlson, JW.; Nucci, MR.; Brodsky, J.; Crum, CP.; Hirsch, MS. (Sep 2007). "Biomarker-assisted diagnosis of ovarian, cervical and pulmonary small cell carcinomas: the role of TTF-1, WT-1 and HPV analysis.". Histopathology 51 (3): 305-12. doi:10.1111/j.1365-2559.2007.02790.x. PMID 17727473.