Ovarian small cell carcinomas are rare and usually aggressive tumors, with two main subtypes: SCOC (small cell ovarian carcinoma, including hypercalcemic type) and SCCOHT (small cell carcinoma of the ovary hypercalcemic type). They typically present in young to middle-aged women and can cause nonspecific pelvic or abdominal symptoms; prognosis is generally poor, and management often requires multimodal therapy including surgery and adjuvant chemotherapy, with treatment decisions guided by stage and histology. Key points
- Types and features
- SCOCH-type and SCCOHT are the main entities; SCCOHT is the most studied in recent years and is characterized by SMARCA4 (BRG1) inactivation in many cases, with a distinct immunoprofile and aggressive behavior.
* Immunohistochemistry helps differentiate small cell ovarian tumors from other “small round blue cell” ovarian tumors; common patterns include EMA and cytokeratin positivity with variable neuroendocrine markers (e.g., synaptophysin) depending on the case.
- Clinical presentation
- Patients often present with abdominal or pelvic symptoms, rapid tumor growth, and sometimes hypercalcemia (especially in the hypercalcemic subtype).
* Younger patients are frequently affected, but cases span a wider age range depending on subtype.
- Diagnosis and work-up
- Work-up combines imaging, tumor markers, histopathology, and immunohistochemistry; distinguishing SCCOHT/SCOC subtypes is critical because it influences prognosis and treatment choices.
- Treatment and prognosis
- Management generally involves surgical resection, with adjuvant chemotherapy and/or radiotherapy tailored to stage and histology; early-stage disease may be managed more conservatively in selected SCOC cases, but advanced disease often requires aggressive multimodal therapy.
* Overall survival is typically poorer than for many other ovarian cancers, especially for advanced-stage disease; prognosis varies with stage, tumor size, and histological subtype.
What would you like next?
- A concise comparison table of SCOC subtypes (SCOCHT vs SCCOHT) with typical age, markers, prognosis, and recommended therapies.
- A brief patient-friendly summary to share with someone newly diagnosed.
- References and a short bibliography for further reading.
