ACTA ENDOCRINOLOGICA (BUC)

Background. Patients with acromegaly (caused by growth-hormone-secreting pituitary adenomas) are at increased risk of hypopituitarism, in particular hypogonadotropic hypogonadism, before and after multimodal therapy. In affected women of reproductive age, fertility is impaired and complex fertility treatments are needed to achieve conception. C ase presentation. We present the case of a young woman with acromegaly caused by a GH-secreting macroadenoma with suprasellar and bilateral cavernous sinus extension; hypogonadotropic hypogonadism and secondary hypothyroidism were present from the initial evaluation. Neurosurgical intervention was repeatedly recommended but the patient refused it initially; also she was non-compliant to the medical treatment of acromegaly. Transsphenoidal tumor debulking with adjuvant gamma-knife radiotherapy was eventually performed. Following treatment persistent active acromegaly and hypogonadotropic hypogonadism were diagnosed. Under chronic estroprogestative replacement therapy, the patient conceived and delivered a full-term healthy newborn without any complications. Possible mechanisms are discussed. Conclusions. Secondary hypogonadotropic hypogonadism in pituitary patients, even when considered permanent (after surgery and radiotherapy), can exceptionally allow spontaneous conception and normal course of pregnancy.

Background. Craniopharyngiomas are benign but locally invasive tumours of the sellar region that arise from ectopic embryonic remnants of Rathke's pouch, affecting both children (adamantinomatous type -aCP) and adults (papillary type -pCP) and associated with significant morbidity. Objective. To study the clinical presentation of CRF as well as the posttreatment evolution of craniopharyngioma in children versus adults in a large mixed cohort. Material and methods. We performed a retrospective review of CRF patients evaluated in the National Institute of Endocrinology in Bucharest between 1990 and 2016. Results. A total of 107 patients (72 adults, 35 children) with a mean follow-up of 6.2 years were included. The presenting symptoms were mostly headache, visual impairment, symptoms of hypopituitarism, diabetes insipidus. Some symptoms or hormonal abnormalities were significantly more prevalent in the children group (p<0.05): nausea/ vomiting (47.8% vs 16.7%), photophobia (21.7% vs 5.6%), diabetes insipidus(28.5% vs 8.3%), GH deficiency (68.8% vs 17.1%). Impaired visual acuity (67.6%of cases) or visual fields (71.4%) were more frequent in adults compared to children (44.1%; 51.6%). The tumor dimensions were similar in both groups (3.05± 1.05 cm in children; 2.7± 1.07 cm in adults). Massive suprasellar extension reaching the third ventricle was frequently present in all cases. All cases underwent surgery but only a minority of those not cured received postoperative adjuvant radiotherapy. Frequent postoperative complications were: aggravation of the endocrine deficit (>80% of cases in both groups needed chronic replacement therapy), central diabetes insipidus (68.2% children, 34.3% of adults). Conclusions. Despite similar tumor dimensions and extension compared to adults, craniopharyngioma in children is more frequently associated with signs of intracranial pressure. The results and complications of treatment are similar in adults and children

for non-functioning pituitary macroadenomas (NFMAs) is still under debate. Aim. To appreciate the best timing for postoperative high voltage radiotherapy (hRT) in different type of NFMAs (classified using immunohistochemistry (IHC)). Subjects and Methods. Of 97 patients with a remnant (>1 cm) and IHC for anterior pituitary hormones, 41 patients (groups A & B) were submitted to hRT and followed up at least 5 years. RT was performed in 20/41 patients (Group A) within the first year after surgery, 21/41 patients (Group B) afterwards while in control group C, 56 patients were followed up without hRT. The progression of postoperative remnant was defined as a change of minimum of 25% of any diameter (transversal or vertical) by serial imaging studies. Results. The IHC of NFMAs revealed the following: 38 (39%) null cell, 29 (30%) gonadotropinomas, 12 (12%) silent plurihormonal, 11 (11%) silent corticotroph and 7 (7%) silent GH/PRL adenomas. Immunoreactive adenomas have relapse rate higher than null cell adenomas (ACTH> GH/ PRL> FSH/ LH> null cell) with a significant rate for silent ACTH 6/8 (75%). The null cell adenoma relapse rate was 6/23 (26%), p<0.009 in group without hRT. The relapse rate was significantly lower in group AB with hRT than in group without RT (p=0.025), at five years. Immediate hRT (applied within in first years) improved the control of the tumour growth in 90% (18/20) cases. In the group A, 10 % (2/20) patients relapsed than 24% (5/21) patients in group B and 39% (22/56) patients, Group C. Conclusion. An optimal time for radiotherapy is within the first year after the partial surgical removal of NFMAs, particularly if a large amount of residual tumour remains. Patients with silent corticotroph adenoma require special attention.