Ovarian small cell carcinoma of the hypercalcemic type
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Ovarian small cell carcinoma of the hypercalcemic type | |
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Diagnosis in short | |
![]() Small cell carcinoma of the ovary hypercalcemic type. H&E stain. | |
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Synonyms | malignant rhabdoid tumour of the ovary |
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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 |
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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.