• Clinical science

Ovarian tumors

Abstract

The ovaries consist of different kinds of tissue (epithelial, germ cells, and sex cord tissue), which may give rise to benign or malignant tumors. Symptoms depend on the type of tissue affected and range from local abdominal discomfort to endocrinological phenomena caused by hormone-producing tumors. Metastases of other tumors and lymphomas may also affect the ovaries. The most common malignant tumor of the ovaries is serous ovarian cancer, which (with the exception of those who are genetically predisposed) mostly affects older women. The lack of early symptoms of ovarian cancers often delays diagnosis, resulting in an unfavorable prognosis. Ovarian cancers primarily metastasize intraperitoneally and later become noticeable mostly due to increasing abdominal girth caused by malignancy-related ascites. Treatment generally involves radical surgical removal of the tumor and chemotherapy.

Epidemiology

  • Lifetime prevalence of malignant ovarian cancer: 1–2%
  • Peak incidence: 60–70 years
  • Genetic predisposition may play a role in familial incidence and in younger patients (< 30 years) developing tumors.
  • Epithelial ovarian carcinomas account for 70% of all ovarian malignancies.

References:[1][2][3]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

Risk factors

References:[4][2][3]

Overview of ovarian tumors

Epithelial Tumors

Epithelial ovarian tumors may be benign, malignant, or borderline!

Germ cell tumors

Sex cord-stromal tumors of the ovary

Mesenchymal tumors

  • ∼ 4% of all ovarian tumors
  • Malignant fibrosarcoma

Metastasis

References:[4][2][3][5][6][7]

Clinical features

General symptoms

The first symptom is often increasing abdominal girth (clothes no longer fit at the waist)!

Specific symptoms

Metastatic dissemination

  • Lymphatic spread: especially along the pelvic and para-aortic lymph nodes; less often along the inguinal lymph nodes
  • Hematogenous spread: rare; metastases can occur in the lungs, liver, bones, and CNS
  • Spread by continuity: peritoneal carcinomatosis with intraperitoneal spread and superficial metastases.; commonly spreads to to the omentum (omental caking)

References:[1][2][3][5][6][8]

Stages

TNM Classification of malignant tumors and FIGO Cancer Staging

TNM FIGO Anatomic extension
T1 I Tumor limited to one (T1a) or both ovaries (T1b) (with intact or ruptured (T1c) capsule)
T2 II Tumor involving one or both ovaries with extension to the pelvis or peritoneal cancer
T3 III Tumor involving one or both ovaries with extension outside the pelvis
N1 Metastasis to regional lymph nodes
M1 IV Distant metastasis (excluding peritoneal metastasis)

References:[9]

Diagnostics

Fine needle aspiration cytology is absolutely contraindicated in ovarian tumors because it increases the risk of spreading tumor cells to the peritoneum!

Call your Ex and Grandparents! – Call-Exner bodies are characteristic of Granulosa cell tumors.

Ultrasound workup of ovarian masses
Benign Malignant
Internal structure Uniform, thin walls Irregularly thickened septa
Margins Smooth Indistinct borders; papillary projections
Echogenicity Anechoic Hypoechoic, anechoic, and hyperechoic components
Content Cystic Cystic or solid components
Vascularization Unremarkable Possible central vascularization
Pouch of Douglas Unremarkable Possible free fluid (ascites)

References:[5][6][10]

Differential diagnoses

The differential diagnoses listed here are not exhaustive.

Treatment

  • Surgery
    • Frozen section and histology positive for carcinoma: radical surgical staging
      • Removal of the greater omentum
      • Lymphadenectomy (pelvic and para-aortic)
      • Hysterectomy with bilateral salpingo-oophorectomy (removal of uterus, ovaries, tubes, and supporting structures)
      • The prognosis is directly correlated with the extensiveness of the radical tumor removal.
      • Exception: A fertility-sparing surgery is possible in cases of verified stage FIGO IA and of grade G1 (i.e., tissue is well differentiated)
      • Appendectomy if involvement is suspected during surgery
      • Biopsy from all noticeable locations/adhesions (peritoneal biopsy and washing cytology
    • Frozen section negative for carcinoma: tumor resection, but no surgical staging
    • Second-look surgery
  • Chemotherapy

Malignant germ cell tumors respond particularly well to polychemotherapy because they are highly aggressive!

References:[5][6]

Prognosis

Ovarian carcinoma

  • Very poor overall prognosis as a result of late diagnosis
  • 5-year survival rate of all ovarian carcinomas: ∼ 30–40%
FIGO Anatomic extension 5-year survival rate
I Limited to one or both ovaries 80–90%
II Infiltration of lesser pelvis 60–70%
III Extension outside pelvis 30–50%
IV Distant metastases 10–20%

References:[5]

Prevention

References:[11]

Special patient groups

Pregnant women

  • Pregnancy luteoma
    • Definition: : rare benign tumors that develop specifically during pregnancy.
    • Clinical features
      • Majority of patients are asymptomatic
      • When clinically apparent, it presents typically with symptoms of virilization during pregnancy
    • Diagnostics: tumor often diagnosed incidentally (during cesarean delivery)
      • Apparent as solid adnexal masses (unilateral or bilateral; usually nodular)
      • The size of the tumor is usually < 10 cm in diameter.
    • Treatment: observation only; regresses spontaneously post-partum
  • Theca lutein cysts
    • Definition: a type of benign enlargement of the ovaries. It is a functional ovarian cyst that is thought to originate due to excessive amounts of circulating gonadotropins such as beta-hCG.
    • Diagnostics: Ultrasonography shows bilateral enlarged, multilocular, cystic masses of the ovaries.
    • Treatment

If surgical removal of an ovarian tumor is indicated during pregnancy, surgery should, if possible, be scheduled for after the 10th week of gestation, as the secretion of progesterone by the corpus luteum is essential for the maintenance of the pregnancy. The placenta takes over this function from approximately the 10th week of pregnancy onwards.

References:[12][13][14][15]