Ovarian small cell carcinoma of the hypercalcemic type

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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 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:

Images

www:

IHC

Features:[4]

See also

References

  1. 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. 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. 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.
  4. 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.