Dysgerminoma of the ovary in a patient with triple-X syndrome

A 24-year-old patient with dysgerminoma of the right ovary and hypoplastic left ovary was screened for chromosomal aberration. Triple-X syndrome was found in the oral epithelium and lymphocyte culture, while chromosomal in situ hybridization of the tumor itself showed a normal XX karyotype. Although trisomy X and dysgerminoma are coincidental findings in our case report, women with malignant germ cell tumors and abnormal genitalia should be checked for chromosomal aberrations to exclude anomalies carrying a Y chromosome.