Small cell is a rare type of ovarian cancer that predominantly affects young women, with an average age at diagnosis of 24 years old, and often has a poor prognosis.
It represents less than 1% of all ovarian cancer diagnoses, with fewer than 300 cases reported in the literature so far.
Recently, there has been major a breakthrough in the understanding of small cell cancer of the ovary with the discovery that inherited mutations in the SMARCA4 gene explain many of the familial cases of the small cell ovarian cancer of hypercalcaemic type (SCCOHT). The gene is somatically mutated in the majority of the SCCOHT cancers.
This is the first step to targeted drug development for small cell ovarian cancer, similar to the development of PARP inhibitors for BRCA-related cancers.