SummaryContext 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 µ g/l, increased IGF-1 for age and sex), were monitored in a single centre and treated with SSTa as firstline therapy. The mean pretreatment GH level was 13·5 ± 3·1 µ g/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 µ g/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 µ g/l and IGF-1 of 123 ± 18% ( P = 0·02). GH < 2 µ g/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.
Inflammatory myofibroblastic tumours (IMT), also called inflammatory pseudotumours, nodular lymphoid hyperplasia, plasma-cell granuloma and fibrous xanthoma, are rare soft-tissue lesions characterised by inflammatory cells and a fibrous stroma. Clinically and radiologically, they may look like malignant tumours. They rarely affect the central nervous system and are very rare in the spinal cord. We report an IMT of the spinal cord in a 22-year-old woman presenting with spinal cord compression and a cauda equina syndrome. MRI showed a lesion at T9 with extramedullary and intramedullary components giving low signal on T2-weighted images and enhancing homogeneously. Pial lesions on the lumbar enlargement and thoracic spinal were present 11 months after surgery, when the lesion recurred. We present the radiological, operative and pathological findings and review the literature.
Primary sellar melanocytic tumors are exceptional lesions presenting most often as a tumor syndrome and/or anterior pituitary insufficiency mimicking a non-secreting pituitary macroadenoma. The management of these tumors consists of surgical removal of the tumor. However, surgery is often incomplete and stereotactic fractionated radiotherapy is frequently indicated.
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