Ovarian Cancer

Alternative Names

  • Ovarian Cancer, Epithelial
  • Suppressor of Tumorigenicity 8
  • ST8
  • Ovarian Cancer, Familial
  • Ovarian Tumor
  • Peritoneal Ovarian Carcinomatosis
  • Neonatal Ovarian Cyst
  • Fetal Ovarian Cyst
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WHO-ICD-10 version:2010

Congenital malformations, deformations and chromosomal abnormalities

Congenital malformations of genital organs

OMIM Number

167000

Mode of Inheritance

Autosomal dominant

Gene Map Locus

3p22.1, 3q26.32,6q26,11q25, 14q32.33,16q22.1

Description

Ovarian cysts are fluid filled sacs on the surface of the ovary. Fetal and neonatal ovarian cysts have previously been considered uncommon. With the development of prenatal and postnatal ultrasonography, however, the detection of cysts has increased. Ovarian cysts usually become clinically apparent only during childhood adolescence. Unilateral cysts are more common than bilateral, and unilocular cysts, are more common than multilocular cysts. An ovarian cyst should be suspected when a female has a cystic intra-abdominal mass which is separate from the organs of the urinary and gastrointestinal tract. Any cystic ovarian enlargement is almost always benign, but complications occur such as torsion of the ovarian cyst leading to signs of an acute abdomen and small bowel obstruction. This may be the first clinical sign of the cyst if it had not yet been detected by prenatal or postnatal ultrasonography. However, larger cysts carry a greater risk of torsion with subsequent loss of the ovary, and hence a surgical intervention is indicated.

Familial ovarian cancer shows an autosomal dominant inheritance pattern. Structural and numerical chromosomal abnormalities are usually observed in the ovarian tumor tissues. Multiple loci in chromosome 2, 6, 9, 11, and 17 are known to be associated with ovarian cancer. Studies have also observed a small percent of early-onset ovarian cancer patients with germline mutations in MLH1, MSH2, and tumor suppressor genes BRCA1 and BRCA2.

Epidemiology in the Arab World

View Map
Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
167000.1LebanonFemaleYes Ovarian carcinomaNM_000059.4:c.4342_4343delHeterozygousEl-Khoury et al. 2019 Mother and older sis...
167000.2LebanonFemale Ovarian neoplasm; Breast carcinomaNM_181523.3:c.1346T>CHomozygousAutosomal, RecessiveMoussa et al, 2020

Other Reports

Bahrain

Al-Ojaimi (2005) reported a 32-year-old Bahraini lady with a large fetal intra-abdominal cyst detected antenatally on ultrasound examination at 16 weeks of gestation. There was no significant family or previous medical history. The cyst was simple anechoic, increasing in size and causing progressive displacement of the fetal thoracic organs. A successful intrauterine needle aspiration was carried out under ultrasound guidance at 30 weeks gestation without maternal or fetal morbidity. Cytology of the cyst fluid showed luteinized granulosa cells and biochemistry demonstrated high concentrations of estradiol, progesterone, and testosterone that confirmed the etiology of the cyst as ovarian. Amniocentesis resulted in a normal female karyotype (46, XX). There was no evidence of recurrence following aspiration and no further need for postnatal surgery.

Kuwait

Junaid (1996) reviewed and classified 301 cases of primary ovarian neoplasms encountered in four Kuwaiti hospitals between 1984 and 1989. The most common tumor was benign cystic teratoma. Although germ cell tumors were more prevalent than epithelial tumors, the majority (76%) of malignant tumors were epithelial. Intriguingly, the percentage of epithelial tumors in patients <20-year-old was higher in Kuwait than what is recorded internationally.

[Junaid, HTA. Ovarian tumors in Kuwait. Med Princ Pract. 1996; 5(4):192-7.]

Oman

Fatima et al. (2000) reported an uncommon presentation of ovarian tumor with deep venous thrombosis in a 45-year old female. The patient presented with left femoral vein thrombosis, and while on anticoagulant therapy, developed extensive right popliteal vein thrombosis, confirmed by Doppler flow studies. Clinically, she had tense swelling of both legs, and abdominal examination revealed an intraperitoneal mass (7x8 cm) arising from pelvis, which was firm, non tender, with restricted movement. Ultrasound scan showed the mass to be rising from the right ovary, while the left ovary and uterus were normal. Tumor marker CA125 was raised (153.5), and CT abdomen and pelvis confirmed the ultrasound findings. There were no lymph nodes and the liver and spleen were normal. The thrombophilic profile was normal. As she was started on heparin infusion and antibiotics, she developed pulmonary embolism and became tachycardic, with poor air entry on the right side. ABG revealed O2 of 84%, PCO2 of 39% and O2 saturation of 90%. The diagnosis was confirmed by lung perfusion scan which showed segmental perfusion defect in right mid zone and left lower zone. Further emboli were prevented by insertion of an inferior vena cava filter. After stabilizing the patient on heparin infusion, retrograde ureteric catheterization and staging laparotomy with debulking was performed to reveal a large right ovarian tumor (10x12 cm) with an intact capsule and no evidence of peritoneal metastasis. Peritoneal washings for cytology from POD, para colic gutters, and sub diaphragmatic areas were done, and all pelvic and abdominal contents were examined. This was followed by total abdominal hysterectomy with bilateral salpingo-oopherectomy and infracolic omentectomy. The histological diagnosis of the tumor was a clear cell adenocarcinoma stage 1 A (FIGO). Postoperatively, the limb swelling subsided and the patient was discharged after 12 days on warfarin for three months and to be followed up with CA125 tumor marker and CT scans.

[Fatima A, Kuruvilla M, Sharma U. Uncommon presentation of malignant ovarian tumor. Oman Med J. 2000; 17(2):57-8.]

Qatar

Ejeckam et al. (1994) indicated that gynecological malignancies constitute 6.88% (144 of 2092) of all malignant lesions in Qatar over a 15 year period (1979-1993). Cancer of the cervix was the most common with 54.86% followed by ovarian cancer 22.91%, endometrium 15.97%, vulva and vagina 2.75%, and 2.08% respectively. As in some other developing countries, cervical and ovarian neoplasms tend to occur in the younger age group. Increasing number of pregnancies seems to protect against ovarian cancer.

Saudi Arabia

Jawad et al. (1998) reported a 38-week-gestation female fetus with an abdominal cystic mass. The physical examination revealed a mobile abdominal mass. Post-delivery ultrasound confirmed the diagnosis of a right-sided cystic pelvic mass with echogenic findings. On repeat ultrasound at the ages of 3 and 5 months, the cystic lesion was reported to be on the left side of the pelvis and had increased in size to 4.2-3.7 cm. The parents consented to laparoscopic examination and possible removal of the lesion, which was achieved successfully. The postoperative course was smooth and the baby was discharged home on the second postoperative day. Histologic examination of the cyst showed extensive necrosis and autolysis with widespread calcifications and no evidence of malignancy.

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