[Sertoli-Leydig cell tumour (arrhenoblastoma) in a patient with polycystic ovary syndrome: clinical, ultrasonographic, hormonal and histopathological evaluation].
Nowadays, the polycystic ovary syndrome is considered the most common endocrinopathy in women. A patient referred for oligomenorrhea or amenorrhea, will probably be classified as suffering from this syndrome if at least one of the three characteristics of the syndrome is present: 1. polycystic ovaries (ultrasonographic finding); 2. hirsutism, and 3. hyperandrogenism. However, differential diagnosis of PCOS must also include patients with menstrual disorders and hirsutism, suffering from disorders of the pituitary gland (hyperprolactinaemia or acromegaly), disorders of adrenal glands (congenital classical or non-classical adrenal hyperplasia) or androgen secreting ovarian tumours.


METHODS
A PCOS patient with a very high serum testosterone level -8.2 nmol/L (normal concentrations 0.7-3.47 nmol/L), and clinical signs of hyperandrogenism, which had been deteriorating during the monitoring period of approximately two years, is presented. The first ultrasound examination diagnosed polycystic ovaries, having a volume at the upper limit of normal range (7.9 cml). After one year ultrasound showed an asymmetrical enlargement of the right ovary (volume 25.34 cm3). Three months later a tumour of the right ovary was diagnosed (Figure 1). Salpingo-oophorectomy was performed. Microscopic analysis indicated without doubt the presence of a Sertoli-Leydig cell tumour of intermediate differentiation (Figure 2).


CONCLUSIONS
In adolescence, congenital adrenal hyperplasia and adrenal tumours may be a source of elevated androgen concentrations. Adrenal tumours usually also cause excess secretion of dehidroepiandrosteron-sulfate (DHEAS). However, rare cases of adrenal tumours with excess testosterone secretion, but with normal or even low DHEAS levels, have also been reported. Adrenal carcinoma can cause virilization, however, in this case excess cortizole secretion is also frequently present. In young patients, the most common state accompanied by hyperandrogenism is PCOS. Hyperthecosis is also accompanied by high testosterone levels, but an ultrasound finding of cystic ovaries is not typical for this condition. Androgen-secreting ovarian tumours are a more common cause of hyperandrogenism than hyperthecosis. Sertoli-Leydig cell tumours are tumours of sexual cords and ovarian stroma. They present as unilateral, solid tumours (bilateral in < 3% of cases). In all cases when they are histologically well or intermediately differentiated, as in our patient, they are limited to the ovary. The percentage of Sertoli-Leydig cell tumours, manifesting endocrine activity has not been established, but it most probably amounts to about 50%, while virilization develops in about 1/3 of patients. In tumours showing hormonal activity the testosterone level is elevated, while the DHEAS level is within the normal range. Analysis of our patient confirmed the immense importance of detailed anamnesis, physical examination and regular control check-ups. Asymmetrical ovarian volume, especially if accompanied by unusually high testosterone concentrations, very probably points to an ovarian tumour, and when dealing with a patient with progressive hirsutism and/or virilization, even with a negative ultrasound finding, the clinician should remain suspicious and careful and request control examinations.
Puzigaća
S
S
Prelević
G
G
Svetenović
Z
Z
Djuricić
S
S
Kokan
Dj
Dj
Radivojević
U
U
D000293:Adolescent; D005260:Female; D006801:Humans; D010051:Ovarian Neoplasms / Q000150:complications* / Q000175:diagnosis; D011085:Polycystic Ovary Syndrome / Q000150:complications* / Q000175:diagnosis; D018310:Sertoli-Leydig Cell Tumor / Q000150:complications* / Q000175:diagnosis