B24. Gonadal ovarian tumors

Definition and epidemiology

By gonadal ovarian tumours, I’m pretty certain they mean ovarian sex cord-stromal tumours.

Ovarian sex cord-stromal tumours (SCSTs) are a group of ovarian tumours. Some of them are benign, some of them are malignant. These tumours arise from sex cord cells, like Sertoli or granulosa cells, or from stromal cells, like fibroblasts and gonadal stroma.

In contrast to epithelial ovarian cancers, malignant SCSTs are often diagnosed at an early stage, and so the prognosis is good. SCSTs account for < 5% of all ovarian tumours.

Classification

WHO classifies SCSTs as the following:

  • Pure stromal tumours
    • Fibroma
    • Thecoma
    • Fibrosarcoma
    • Leydig cell tumour
  • Pure sex cord tumours
    • Granulosa cell tumour
    • Sertoli cell tumour
  • Mixed sex cord-stromal tumours
    • Sertoli-Leydig cell tumour

We can also classify them according to their dignity:

  • Benign tumours
    • Fibroma
    • Thecoma
  • Malignant tumours
    • Fibrosarcoma
    • Granulosa cell tumour
  • May be benign or malignant
    • Sertoli-Leydig cell tumour (adrenoblastoma)
    • Sertoli cell tumour

Most types are most prevalent in postmenopausal women. The only exception is Sertoli-Leydig cell tumour, which primarily affects 30 – 40 year old women.

Fibroma is the most common benign SCST, while granulosa cell tumour is the most common malignant SCST.

Clinical features

Fibromas may present as Meigs syndrome, which is the presence of ascites and/or hydrothorax simultaneously as a fibroma.

Granuloma cell tumour produce oestrogens. Thecomas are often mixed with granulosa cells and can therefore also be oestrogen-secreting. The symptoms of this depends on the age of the patient:

  • Before puberty – causes precocious puberty
  • Reproductive age – causes bleeding disorder (metrorrhagia, hypermenorrhoea)
  • After menopause – causes postmenopausal bleeding

Sertoli-Leydig cell tumours produce androgens, which may cause virilisation, hirsutism, acne, etc.

SCSTs present similarly as epithelial or germ cell ovarian tumours, with some exceptions.

General symptoms of ovarian cancer include (from topic A11):

  • Asymptomatic
  • Abdominal enlargement
    • Sometimes due to ascites
  • Symptoms of pressure on surrounding organs
    • Dysuria
    • Constipation
    • UTI
  • Symptoms relating to complications of the tumour (usually acute)
    • Torsion – acute pain and vomiting
    • Rupture – generalised abdominal pain
    • Haemorrhage – abdominal pain and haemorrhagic shock

Diagnosis and evaluation

Diagnosis and evaluation are the same as for other ovarian cancers, except that hormones should be measured depending on the symptoms of the patient.

From topic A11:

Physical examination should be performed for an adnexal mass as well as inguinal and cervical lymphadenopathy. Ultrasound of the adnexal mass can reveal a solid mass, ascites. Chest, abdominal, and pelvic CT or MRi are used to look for ascites and diseases spread.

There are some tumour markers which may be elevated in ovarian cancer. These are mainly used for follow-up rather than for diagnosis:

  • CA-125
  • HE4
  • β-hCG
  • AFP

The diagnosis of ovarian cancer is histological and is generally made based on the excised tumour after exploratory laparotomy. Biopsy causes tumour seeding and is not performed, so histology can only be performed after surgical excision.

Treatment

The treatment of malignant SCSTs is generally the same as for other ovarian cancers and includes bilateral salpingo-oophorectomy (adnexectomy) and total hysterectomy.

Adjuvant chemotherapy may be used in some cases.

Surgical removal of the tumour generally causes resolution of the tumours effects. In patients with Meigs syndrome, surgical removal of the fibroma leads to complete resolution of the symptoms. In patient with hormonal changes, these changes are reversed after surgery.


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B23. Gestational throphoblast neoplasia (invasive mole, choriocarcinoma); diagnosis and therapy

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B25. Tumor markers of malignant ovarian cancer. Diagnosis and treatment of borderline ovarian cancer.

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