Granulosa cell tumour of the ovary

Granulosa cell tumours of the ovary are a type of sex cord / stromal ovarian tumour.

It can be divided into two broad subtypes:

• adult granulosa cell tumour of the ovary: much more common and accounts for ~95% of cases ⁵

• juvenile granulosa cell tumour of the ovary

Epidemiology

Sex cord-stromal tumours represent approximately 8% of all ovarian tumours and are believed to arise from and/or to contain combinations of the sex cord and stromal components of the developing gonad. Granulosa cell tumours of the ovary comprise a distinct subset of ovarian cancers that account for approximately 5% of all ovarian malignancies ⁶. They occur most commonly in perimenopausal and postmenopausal women (peak incidence at age 50) ⁷.

Associations

• the hyperoestrogenemia may produce

  • endometrial hyperplasia

  • endometrial polyps

  • endometrial carcinoma: associated in ~15% (range 3-25%) of cases ⁵

• Maffucci syndrome: with juvenile granulosa cell tumour

Clinical presentation

Patients may present with nonspecific symptoms such as abdominal pain, distention, or bloating. In a majority of cases, there may be hormonal manifestations due to oestrogen activity of the tumour ⁶.

Pathology

Granulosa cell tumours are thought to arise from normal proliferating granulosa cells of the late preovulatory follicle and exhibit many morphological and biochemical features of these cells. They are distinct from other ovarian carcinomas in their hormonal activity; their ability to secrete oestrogen, inhibin, and Müllerian inhibiting substance accounts for some of the clinical manifestations of the disease and also provides useful tumour markers for disease surveillance ⁶.

Radiographic features

They are slow-growing, predominantly solid masses with variable amounts of cystic change and intratumoural haemorrhage. Bilaterality is rare.

Oestrogenic effects on the uterus may manifest as uterine enlargement or as endometrial thickening or haemorrhage ⁶.

Ultrasound

• appearance varies widely: may appear anywhere from a solid mass to a multiloculated solid and cystic mass, to a purely cystic lesion

• varying degrees of haemorrhage or fibrosis

• less likely to have intracystic papillary projections than epithelial ovarian tumours

CT

The CT appearance of a granulosa cell tumour is usually that of a large, well-defined low-attenuation ovarian mass.

Treatment and prognosis

It is suggested that stage I granulosa cell tumours should go for fertility-sparing surgery; unilateral salpingo-oophorectomy appears to be the most appropriate course of action ⁶.

See also

• ovarian tumours