Virilising ovarian tumour: a case associating a Sertoli-Leydig cell tumour and a Brenner tumour.

Ovarian Sertoli-Leydig cell tumours (SLCT), also termed arrhenoblastomas, are the most frequent virilising tumours in women of reproductive age. Very rare secretory Brenner tumours (BT) have been described, generally after the menopause. A 31-year-old woman sought medical advice for secondary amenorrhoea, progressive hirsutism and a 5-year history of virilisation syndrome with clitoromegaly. Testosterone was markedly high (285 ng/dl, N<85) with moderate elevation of delta 4-androstenedione (D4AD) (311 ng/dl, N <270), dehydroepiandrosterone sulfate (DHEAS) (366 µg/dl, N <340) and 17-hydroxyprogesterone (17OHP) (275 ng/dl). LH was 9 IU/l, FSH 4.3 IU/l, estradiol 60 pg/ml and progesterone 314 ng/100 ml. Cortisol was decreased (1.3 µg/dl) after the dexamethasone suppression test. Pelvic MRI showed a 5-cm right ovarian tumour with a 2.5 cm nodular component and cystic areas, and two nodules measuring 11 mm and 15 mm above the right and left ovaries. After right ovariectomy by laparoscopy, pathological examination concluded on a 3-cm SLCT and a 2-cm BT; the nodules above the ovaries were dysembryoplastic cysts. Postoperatively, testosterone level was normal after 24 h (26 ng/dl), estradiol and progesterone rapidly decreased, cyclic secretion then resumed and the patient menstruated at day 27. To our knowledge, this is the first report of an ovarian tumour associating a Sertoli-Leydig cell tumour and a Brenner tumour in a patient with virilisation syndrome which resolved after ovariectomy.

Long-term (up to 18 years) effects on GH/IGF-1 hypersecretion and tumour size of primary somatostatin analogue (SSTa) therapy in patients with GH-secreting pituitary adenoma responsive to SSTa.

CONTEXT: The role of somatostatin analogues (SSTa) in the treatment of acromegaly. OBJECTIVE: To evaluate the antihormonal and antitumour efficacy of long-term (up to 18 years) primary treatment with SSTa in patients with GH-secreting pituitary adenoma responsive to SSTa. DESIGN: An open, prospective, single-centre, clinical study. PATIENTS: Thirty-six acromegalic patients, aged 17-75 years (postoral glucose tolerance test GH > 1 microg/l, increased IGF-1 for age and sex), were monitored in a single centre and treated with SSTa as first-line therapy. The mean pretreatment GH level was 13.5 +/- 3.1 microg/l, and IGF-1 (as a percentage of the value over the normal range) was 302 +/- 26%. The patients had macroadenoma (n = 25), microadenoma (n = 8) or empty sella turcica (n = 3). The mean duration of treatment was 8 years (range 3-18 years). Hormonal and morphological monitoring was undertaken after 6 months, and then the patients were followed annually. RESULTS: After 1 year, the mean GH and IGF-1 levels had reduced considerably (GH: 2.4 +/- 0.3 microg/l; IGF-1; 174 +/- 14%, P < 0.01), and they continued to decrease over 10 years, with a mean GH level of 1.6 +/- 0.1 microg/l and IGF-1 of 123 +/- 18% (P = 0.02). GH < 2 microg/l, normal IGF-1, or both were observed in 25 (70%), 24 (67%) and 21 (58%) patients, respectively. The mean reduction in tumour volume was 43% (range 13-97%) and shrinkage > 20% was obtained in 21 patients (72%). SSTa treatment was well tolerated with few digestive or metabolic side-effects. CONCLUSION: Long-term (up to 18 years) treatment with SSTa used as first-line therapy is effective from both an antihormonal and antitumour perspective, and is well tolerated in acromegalic patients.