Image Description
About 5% of dysgerminomas arise in gonadoblastomas occurring in phenotypic female with abnormal gonads. In Pure Gonadal Dysgenesis, the patients have bilateral streak gonads and 46, XY chromosomes and the risk of malignancy is 28% by age 20 years. Those with Mixed Gonadal Dysgenesis have unilateral streak gonad, contralateral testis, and 45,X/46,XY chromosomes with an estimated 19% risk of malignancy by 20 years of age. Therefore, patients with gonadal dysgenesis must undergo prophylactic removal of gonads at an early age.
The image shows a large dysgerminomas with soft, fleshy, pink-tan cut surface and areas of hemorrhage and necrosis (not a commonly seen feature).
About the Disease
Germ cell tumors (GCT) of the ovary originate from primordial germ cells and make up 30% of all primary ovarian neoplasms. More than 90% of ovarian GCT are mature cystic teratomas which are benign tumors. Remaining 5% are malignant. Malignant GCT make up 3% and 20% of all ovarian cancers in Western countries and Asian/African countries respectively. Patients with malignant GCT are usually between 15-30 yrs. of age. Tumors are large (average size 16 cm) and generally unilateral (except dysgerminomas; 10-20% cases are bilateral). Presenting symptoms are abdominal enlargement and pelvic pain. Serum hCG and AFP levels can give a clue to the diagnosis and are useful in monitoring postoperatively. The treatment consists of fertility-sparing surgery followed by combination chemotherapy. About 60-75% of patients present with Stage I disease which has a cure rate of almost 100%. About 25-30% have Stage III disease at diagnosis and the cure rate is around 75%. REFERENCES: 1. WHO Classification of Tumors. Female Genital Tumors. 5th Edition, 2020; IARC, Lyon, France. 2. Nucci MR, Parra-Herran C (2021). Gynecologic Pathology. 2nd Edition. Philadelphia, PA. Elsevier. 3. Goldblum, J. R. et al (2018). Rosai and Ackerman's Surgical Pathology - Eleventh Edition. Philadelphia, PA. Elsevier.