Small cell carcinoma of the ovary

Small cell carcinoma of the ovary (SCCO), also known as small cell carcinoma of the ovary, hypercalcemic type (SCCOHT), is an extremely rare and aggressive form of ovarian cancer. It most commonly affects young women and adolescents, with the average age at diagnosis being around 24 years old. Despite its name, it is not related to small cell lung cancer. This cancer is now understood to be driven by mutations in a gene called SMARCA4, which normally helps control cell growth. The disease typically presents as a rapidly growing mass in one ovary. One of its hallmark features is high calcium levels in the blood (hypercalcemia), which occurs in about two-thirds of patients and can cause symptoms like excessive thirst, frequent urination, nausea, confusion, and fatigue. Patients often experience abdominal pain, bloating, and a feeling of fullness. The tumor tends to grow quickly and can spread to other parts of the body. Treatment usually involves a combination of surgery to remove the tumor, followed by intensive chemotherapy and sometimes radiation therapy. Because this cancer is so rare and aggressive, there is no single standard treatment protocol that is universally agreed upon. High-dose chemotherapy with stem cell rescue has been used in some cases. Despite treatment, the prognosis has historically been poor, especially for advanced-stage disease, though outcomes are improving with more aggressive multi-modal treatment approaches and emerging targeted therapies. Clinical trials are actively exploring new treatment options, including immune checkpoint inhibitors and drugs targeting the underlying genetic defect.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Bring these to your next appointment

• Q1.What stage is my cancer, and has it spread beyond the ovary?,Should I have genetic testing for SMARCA4, and what would the results mean for my family?,What are my options for preserving fertility before treatment begins?,What treatment plan do you recommend, and is high-dose chemotherapy with stem cell transplant an option for me?,Are there any clinical trials I should consider, and how do I access them?,Should I be treated at a specialized center with experience in this rare cancer?,What is my expected prognosis, and what follow-up monitoring will I need after treatment?

Common questions about Small cell carcinoma of the ovary