Dysgerminoma : Intro & Gross
Image Description
Clinical: Most dysgerminomas are diagnosed in second to third decade of life (median age 22 years). They are rare in premenarchal or postmenopausal women. The usual presentation is with a rapidly growing abdominal mass and pelvic pain. Symptoms may simulate pregnancy. About 10-20% manifest during pregnancy. They can rupture intraperitoneally or undergo torsion. Majority of the cases (two-thirds) are Stage IA at presentation (tumor limited to one ovary with intact capsule or one fallopian tube surface; no malignant cells in ascites or peritoneal washings). Involvement of the contralateral ovary is seen in 20% of cases (grossly 10% and microscopically in another 10% of cases), usually in the setting of gonadal dysgenesis.
About 5% of dysgerminomas arise in phenotypically female individuals with abnormal gonads - either pure gonadal dysgenesis (46XY, bilateral streak gonads), mixed gonadal dysgenesis (45/46XY, unilateral streak gonads, contralateral testis) or androgen insensitivity syndrome (46XY, testicular feminization). In such cases, the tumor is often discovered during work-up for amenorrhea.
Serum Tumor Markers: LDH levels are usually elevated. About 3-5% of dysgerminomas produce hCG causing estrogenic symptoms (irregular menstruation, isosexual pseudoprecocity, pseudopregnancy) or rarely androgenic symptoms. Serum levels of CA-125, PLAP, inhibin and NSE may be elevated. Some patients develop paraneoplastic hypercalcemia.
Gross: Dysgerminomas are large (average size 15 cm), lobulated, solid, soft fleshy tumors with yellow-tan or cream color appearance. Areas of cystic change, hemorrhage and necrosis may be present but are not common. The tumors arising in a background of gonadoblastoma often show calcification.
The image shows dysgerminoma of the ovary in a 39 y/o female. Image copyright: pathorama.ch
About 5% of dysgerminomas arise in phenotypically female individuals with abnormal gonads - either pure gonadal dysgenesis (46XY, bilateral streak gonads), mixed gonadal dysgenesis (45/46XY, unilateral streak gonads, contralateral testis) or androgen insensitivity syndrome (46XY, testicular feminization). In such cases, the tumor is often discovered during work-up for amenorrhea.
Serum Tumor Markers: LDH levels are usually elevated. About 3-5% of dysgerminomas produce hCG causing estrogenic symptoms (irregular menstruation, isosexual pseudoprecocity, pseudopregnancy) or rarely androgenic symptoms. Serum levels of CA-125, PLAP, inhibin and NSE may be elevated. Some patients develop paraneoplastic hypercalcemia.
Gross: Dysgerminomas are large (average size 15 cm), lobulated, solid, soft fleshy tumors with yellow-tan or cream color appearance. Areas of cystic change, hemorrhage and necrosis may be present but are not common. The tumors arising in a background of gonadoblastoma often show calcification.
The image shows dysgerminoma of the ovary in a 39 y/o female. Image copyright: pathorama.ch