Ovarian small cell carcinoma: Manifestations, identification, prognosis
Small Cell Carcinoma of the Ovary (SCCO) is a rare and aggressive form of ovarian cancer, accounting for less than 0.1% of all ovarian cancers. SCCO is characterized by the presence of small, round cancer cells resembling those found in small cell lung cancer. There are two main subtypes of SCCO: small cell carcinoma of the ovary hypercalcemic type (SCCOHT) and small cell carcinoma of the ovary pulmonary type (SCCOPT).
Treatment for SCCOHT
The treatment for SCCOHT typically involves a multimodal approach, including radical surgery and platinum-based chemotherapy, often with multi-agent regimens. Aggressive surgical intervention combined with chemotherapy is common, although there is no standardized treatment protocol.
The prognosis for SCCOHT is generally poor. The five-year overall survival rate for patients with FIGO stage I is 51%, and it decreases to 24% for those with stages II, III, and IV combined. Early-stage disease treated aggressively has better outcomes.
Treatment for SCCOPT
There is limited specific information available on the treatment of SCCOPT compared to SCCOHT. However, given the nature of SCCO, treatments might resemble those for SCCOHT, focusing on surgery and chemotherapy.
Prognostic Factors and Surveillance
For SCCOHT, prognostic factors include disease stage, residual tumor volume after surgery, SMARCA4 mutation status, and the presence of hypercalcemia. Regular follow-up is crucial due to the high risk of relapse.
While SCCOPT has less detailed treatment and survival data, approaches may align with those for SCCOHT, emphasizing aggressive surgical and chemotherapeutic interventions.
General Considerations
Small Cell Carcinoma of the Ovary primarily affects individuals in their 20s, but it can also occur in people ages 28-85 years, with a median age of 59 for SCCOPT. Approximately two-thirds of patients with SCCO also have hypercalcemia, or higher than normal calcium levels in the blood.
Common symptoms of SCCO may include abdominal pain, bloating, and changes in bowel habits. The diagnosis of SCCO often occurs at an advanced stage, and it involves a combination of imaging studies such as CT scans and MRIs, as well as biopsies.
Ongoing research suggests that risk factors linked to other types of ovarian cancer may increase the likelihood of SCCO occurrence. Research has also shown that a combination of surgery, chemotherapy, and radiotherapy can produce better survival rates than surgery and chemotherapy alone for people with hypercalcemic SCCO.
It is essential to note that there is currently no definitive treatment for SCCO because the underlying cause is not fully understood. Treatment options will depend on the stage of cancer and the overall health of the individual. A multidisciplinary approach to treatment may be required, involving surgery, chemotherapy, and radiation therapy.
In the United States, approximately 40-50 people are diagnosed with SCCOHT each year, while SCCOPT is less common. The average age at diagnosis for SCCOHT is not specified, but young women in their teens and twenties are more likely to receive an SCCOHT diagnosis. The overall survival rate for SCCO is relatively low compared to other types of ovarian cancer, with a figure of around 20%. Individual outcomes for SCCO can vary based on factors such as the stage at diagnosis, the type of SCCO, and the effectiveness of the chosen treatment.
[1] National Comprehensive Cancer Network (NCCN) Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Ovarian Cancer. (Accessed March 12, 2023)
Chemotherapy, often platinum-based, is a common treatment component for Small Cell Carcinoma of the Ovary Hypercalcemic Type (SCCOHT), alongside aggressive surgical intervention as part of a multimodal approach.
The prognosis for SCCOHT is generally poor, with the five-year overall survival rate significantly decreasing from stage I (51%) to stages II, III, and IV combined (24%). Regular follow-ups are crucial due to the high risk of relapse.
Medical-conditions such as hypercalcemia, higher than normal calcium levels in the blood, are common among individuals with Small Cell Carcinoma of the Ovary, affecting approximately two-thirds of patients. Health-and-wellness approaches, like proper nutrition and stress management, can complement traditional treatment methods during recovery from SCCO.
