THERAPY OF ENDOCRINE DISEASE: The challenges in managing giant prolactinomas

摘要: Giant prolactinomas are rare tumours, representing only 2-3% of all prolactin (PRL)-secreting tumours and raising special diagnostic therapeutic challenges. Based on several considerations developed in this review, their definition should be restricted to pituitary adenomas with a diameter 40  mm or more, significant extrasellar extension, very high PRL concentrations (usually above 1000  μg/l) no concomitant GH ACTH secretion. much more frequent young middle-aged men than women, male female ratio about 9:1. Endocrine symptoms often present but overlooked for long period time, diagnosis is eventually made when neurologic complications arise from massive extension into the surrounding structures, leading cranial nerve palsies, hydrocephalus, temporal epilepsy exophthalmos. usually range 1000-100,000  μg/l, may underestimated by so-called 'high-dose hook effect'. As every prolactinoma, dopamine agonists first-line treatment allowing rapid alleviation majority cases, reduction tumour size three-fourths patients normalization 60-70%. These extensive not completely resectable neurosurgery has morbidity mortality. It therefore acute such as apoplexy leakage cerebrospinal fluid (often induced medical treatment) insufficient tumoural response progression. Irradiation temozolomide useful adjuvant therapies subset aggressive/invasive which controlled despite combined surgical treatments. Because these various challenges, we advocate multidisciplinary management giant expert centres.