Rare ovarian tumors (germ cell and sex cord-stromal tumors)
Germ cell and sex cord-stromal tumors make up the non-epithelial minority of ovarian neoplasms, occurring in younger patients and adolescents and identified by a characteristic tumor-marker and hormone-secretion pattern that guides fertility-sparing surgery and, for malignant germ cell tumors, cisplatin-based chemotherapy.
- Topic reviewThe full article, organized the way the exam asks it.
- ABIM-format MCQsVignettes with an explanation for every wrong answer.
- Key factsThe numbers and thresholds worth memorizing, up front.
- AI tutorMedBrain answers the question you're stuck on.
What this article covers
- Overview
Non-epithelial ovarian neoplasms: germ cell tumors (from primordial germ cells) and sex cord-stromal tumors (from granulosa, theca, Sertoli, and Leydig cell lineages of the embryonic sex cord and…
- Epidemiology
Mature cystic teratoma (dermoid cyst) is the most common benign ovarian tumor overall and the most common ovarian tumor in young females.
- Risk factors
Age itself is the dominant risk marker: a solid or complex adnexal mass in an adolescent or young woman should raise suspicion for a germ cell tumor before epithelial histology.
- Pathophysiology
Germ cell tumors arise from primordial germ cells and differentiate toward somatic structures (teratoma), extra-embryonic structures (yolk sac tumor, choriocarcinoma), or remain undifferentiated (dysgerminoma).
- Clinical features
Palpable adnexal mass with abdominal fullness or distention is the common presenting sign across subtypes, often found on bimanual pelvic exam.
- Differential diagnosis
Epithelial ovarian cancer (most likely alternative in a solid/complex adnexal mass, especially in a postmenopausal patient): elevated cancer antigen 125 (CA-125), bilateral serous or mucinous…
- Diagnosis
The diagnosis is confirmed by surgical pathology after resection.
- Management
First, perform fertility-sparing surgery whenever feasible: unilateral salpingo-oophorectomy with surgical staging (peritoneal washings, contralateral ovary inspection/biopsy if abnormal, omental…
- Complications
Ovarian torsion with ischemia and necrosis if detorsion is delayed; enlarged masses, especially teratomas, are a recognized risk factor.
- Prognosis
Malignant germ cell tumors overall carry a favorable prognosis relative to epithelial ovarian cancer given their chemosensitivity, particularly dysgerminoma, which is highly responsive to platinum-based chemotherapy and radiosensitive.
- Prevention
No proven population screening test exists for germ cell or sex cord-stromal tumors; transvaginal ultrasound and CA-125 are not recommended for average-risk screening of any ovarian tumor type.
Read the whole article
Subscribing to Internal Medicine opens every article, the full question bank and MedBrain, on the web, iPhone and Android.
Related articles
- Epithelial ovarian cancer
Epithelial ovarian cancer is the most lethal gynecologic malignancy because nonspecific symptoms and the absence of an effective screening test mean most women present with stage III or IV disease. Diagnosis rests on pelvic examination, cancer antigen 125 (CA-125), and transvaginal ultrasonography followed by surgical staging; treatment is cytoreductive surgery plus platinum-taxane chemotherapy, with olaparib maintenance in breast cancer susceptibility gene (BRCA)-mutated disease.
- Evaluation of an ovarian mass
An adnexal mass found on exam or incidental imaging is triaged by menopausal status, ultrasound morphology, and CA-125 into observation, gynecology referral, or urgent gynecologic-oncology referral. Getting this triage right avoids both unnecessary surgery on a physiologic cyst and delayed staging surgery on an early ovarian cancer.
- Hyperthyroidism
Hyperthyroidism is the clinical syndrome of thyroid hormone excess, produced by a range of causes from Graves disease and autonomously functioning nodules to destructive thyroiditis and exogenous hormone ingestion. Diagnosis begins with a suppressed thyroid-stimulating hormone and elevated free thyroxine, followed by radioactive iodine uptake testing or antibody measurement to identify the cause, and first-line treatment is a beta blocker for symptom control while the underlying cause directs thionamide, radioactive iodine ablation, or thyroidectomy therapy.
- Primary ovarian insufficiency
Primary ovarian insufficiency is loss of ovarian follicular function before age 40, defined by amenorrhea with a low estradiol and an elevated FSH on two occasions a month apart. It carries fertility, bone, and cardiovascular consequences that estrogen replacement, continued until the normal age of menopause, is meant to prevent.