Small cell carcinoma of the ovary hypercalcemic type (SCCOHT): About three case reports

This is the authors' abstract. We don't add key points for this paper.

• Diagnostic Challenges: Preoperative diagnosis of SCCOHT is difficult, often mimicking mucinous cystadenocarcinoma on imaging or other kind of ovarian tumors. Hypercalcemia isn’t always present. • Intensive Treatment Matters: Intensive multimodal therapy, including surgery, chemotherapy, radiotherapy, and stem cell transplantation, is crucial for better outcomes. • Genetic Insights: SMARCA4 mutations are common in SCCOHT and offer potential therapeutic targets (CDK-4/6 inhibitors, EZH2 inhibitors, or PD-L1 inhibitors). Genetic testing can be considered, though its value for at-risk relatives is debated. • Improved Survival: With intensive multimodal treatment, patients with stage I SCCOHT do not systematically have a poor prognosis, showing improved survival outcomes compared to historical data. Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT), is an extremely rare and aggressive ovarian cancer affecting young women, typically associated with poor survival. We present three cases of SCCOHT and review the current literature regarding clinical features, diagnosis, management, and survival. Three patients aged 16, 29, and 39 years were treated for ovarian masses. None presented with hypercalcemia. In case 1, MRI findings mimicked a mucinous cystadenocarcinoma, while the other two cases appeared as benign ovarian tumors. Following initial surgery and histological diagnosis of SCCOHT, all patients received comprehensive radical surgery, multi-agent chemotherapy, radiotherapy, and autologous stem cell transplantation. Two patients remain recurrence-free at 5 and 20 years, while the third has a 10-month follow-up. Genetic testing was performed in two patients, revealing no constitutional mutations in SMARCA4 or related genes. These cases highlight the difficulty of establishing a preoperative diagnosis of SCCOHT. Unlike previous reports, our data suggest that intensive multimodal therapy can result in favourable outcomes for stage I SCCOHT. Further studies are needed to define optimal treatment strategies.