Hereditary Ovarian Cancer: Not Only BRCA 1 and 2 Genes

Table 1

Different types of ovarian cancer with their clinicopathologic features and behavior.

	Type 1	Type 2
Prevalence	About 30%	About 70%
Histotype	Serous, endometrioid, mucinous, and clear-cell tumors	Serous, mixed malignant mesodermal tumors carcinosarcomas, and undifferentiated tumors
Grade	Low and borderline	High
Mutations	PTEN, KRAS, BRAF, PIK3CA, ERBB2, CTNNB1, ARID1A, PPP2R1A, and microsatellite instability	TP53; BRCA 1/2
Clinical behavior	Typically large cystic mass confined to the ovary, relatively indolent course	Diagnosed at advanced stages and aggressive behavior