Ovarian stromal tumors
What is it?
Stromal tumors of the ovary are considered rare tumors, accounting for 3-5% of all ovarian neoplasms, with an incidence of 2.1/1 000 000. This category includes generally benign "pure stromal tumors" (theca cell tumor, steroid cell tumor, Leydig cell tumor), "sex cord tumors" (adult-type granulosa cell tumor, juvenile-type granulosa cell tumor, Sertoli cell tumor, sex cord tumor with annular tubules) and mixed "stromal-sex cord" tumors (Sertoli-Leydig tumor). The age of onset is extremely broad and ranges from pediatric and adolescent to adult.
Which are the symptoms?
The most frequent symptoms are abdominal and/or pelvic pain, sense of abdominal distension, irregularity of the menstrual cycle, appearance of abnormal vaginal bleeding in children/girls in pre-pubescent age and in post-menopausal women, increase of hair on the body surface (hirsutism). However, it is not uncommon for this type of tumor not to show clinical signs and to be diagnosed accidentally during a gynecological ultrasound performed for other reasons.
- abdominal distension
- abdominal-pelvic pain
- menstrual irregularity
- postmenopausal bleeding
- hirsutism
- early pseudopuberty
How is it diagnosed?
Diagnosis requires:
- gynaecological ultrasound
- CT scan of the chest and abdomen
- blood tests for the dosage of some markers (alpha-fetoprotein, beta-HCG, CA125, inhibin B, AMH)
In patients with Sertoli-Leydig cell tumor, a genetic analysis will be performed to search for the DICER1 gene mutation.
Suggested exams
How is it treated?
Treatment is surgical and varies according to the extent of the disease and the age of the patient. The standard of care involves the removal of the uterus, tubes, ovaries, omentum and biopsies of the peritoneum and can be performed laparotomically (surgical opening of the abdomen) or laparoscopically (by means of small incisions in the abdomen and navel). In young patients wishing to preserve fertility, in selected cases, it is possible to preserve the healthy uterus and ovary. In some types of stromal ovarian cancer and in case of spread of disease, chemotherapy may be necessary. After the diagnosis and treatment of a stromal tumor of the ovary, it is necessary to perform regular and periodic gynecological, instrumental and laboratory checks with a frequency and duration that varies according to the type of tumor and the stage of disease.
Where do we treat it?
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