Rare case of remission of a patient with small cell carcinoma of the ovary, hypercalcaemic type (SCCOHT) stage IV: Case report

•Small cell carcinoma of the ovary is a very rare, highly undifferentiated, aggressive malignancy that affects young women and linked to a poor prognosis (especially women under 40 years of age). We present a 22 years old African woman with complete remission 30 months after diagnosis despite a conservative surgery and only 3 cycles of chemotherapy.•SCCOHT seems to be a monogenic disease with mutations in the tumour suppressor gene, a chromatin remodeler: SMARCA4 and can involve germline or somatic lines. Mutation can also be a de novo sequence. Research for new treatments includes target therapy.•Because of the rareness of SCCOHT (about 300 cases in the world) no standard treatment guidelines for SCCOHT are established, but combination of surgical debulking and chemotherapy based on cisplatin, are common practice.•Autologous stem cell transplant after high-dose adjuvant chemotherapy seems to lead to the best survival rates, because it allows perform doses 2- to 10-fold higher than standard chemotherapy doses.