Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT): a challenge for clinicopathological diagnosis

Ran Li; Ting Zhou; Shaohua Chen; Nan Li; Zhaogen Cai; Yunzhi Ling; Zhenzhong Feng

Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT): a challenge for clinicopathological diagnosis

Int J Clin Exp Pathol. 2019 Jun 1;12(6):2166-2172. eCollection 2019.

Authors

Ran Li¹, Ting Zhou¹, Shaohua Chen¹, Nan Li¹, Zhaogen Cai¹, Yunzhi Ling², Zhenzhong Feng¹

Affiliations

¹ Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu Medical College Bengbu, Anhui, P. R. China.
² Department of Anesthesiology, The First Affiliated Hospital of Bengbu Medical College, Bengbu Medical College Bengbu, Anhui, P. R. China.

PMID: 31934039
PMCID: PMC6949608

Abstract

Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is an extremely aggressive ovarian tumor, with a poor prognosis and high mortality for young women. This paper aims to inform clinical physicians of new clinical improvements and further understanding of SCCOHT. Two cases diagnosed with SCCOHT from our medical database were reconfirmed and immunohistochemically stained with vimentin, CK, EMA, S-100, ER, PR, and SMARCA4. Diffuse small, round cells with scant cytoplasms, small nucleoli, hyperchromatic nuclei, and active nuclear divisions were detected in the microscopy. The immunohistochemical markers indicated minor positive but notably were SMARCA4 negative, which led to the final diagnosis. SCCOHT is a rare and lethal ovarian tumor in young women. The loss of SMARCA4 or the presence of SMARCA2 is a specific marker for the disease. Susceptibility to CDK4/6 inhibitors associated with downregulation of SMARCA4 targeted cyclin D1 may be a probable therapeutical mechanism for the disease.

Keywords: ATRT; CDK4/6; PD1; SCCOHT; SMARCA4.