What is SCCOHT?
Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT) is a rare and aggressive form of ovarian cancer. It primarily affects young women and is characterized by small, undifferentiated cells and elevated levels of calcium in the blood. Despite its rarity, SCCOHT is notable for its rapid progression and poor prognosis.
Causes and Risk Factors
The exact
causes of SCCOHT are not well understood. However, recent research indicates that genetic mutations play a significant role. Specifically, mutations in the
SMARCA4 gene have been frequently observed in SCCOHT cases. These mutations can disrupt normal cellular functions, leading to uncontrolled cell growth.
Symptoms
The
symptoms of SCCOHT can be quite varied but typically include abdominal pain, a palpable mass in the abdomen, and sometimes symptoms of hypercalcemia such as nausea, vomiting, and confusion. Due to the rapid growth of the tumor, symptoms can quickly become severe.
Diagnosis
Given its rarity,
diagnosing SCCOHT can be challenging. Diagnosis usually involves a combination of imaging studies like ultrasound or CT scans, blood tests to check calcium levels, and a biopsy for histological examination. Genetic testing for SMARCA4 mutations can also be helpful in confirming the diagnosis.
Treatment Options
The treatment for SCCOHT is complex and often involves multiple modalities.
Surgery is usually the first step to remove as much of the tumor as possible. This is often followed by
chemotherapy and sometimes radiation therapy. Due to the aggressive nature of the disease, high-dose chemotherapy with stem cell rescue may be considered in some cases.
Prognosis
Unfortunately, the
prognosis for SCCOHT is generally poor. The aggressive nature of the tumor and its tendency to recur even after treatment contribute to the low survival rates. However, early diagnosis and aggressive treatment can improve outcomes for some patients.
Current Research
Ongoing
research is focused on understanding the genetic and molecular basis of SCCOHT. Studies are exploring new therapeutic targets and treatments, including targeted therapies that specifically address the genetic mutations involved. Clinical trials are also underway to evaluate the efficacy of these new approaches.
