Prolactinomas are the most common pituitary adenomas (approximately 40% of cases), and they represent an important cause of hypogonadism and infertility in both sexes. The magnitude of prolactin (PRL) elevation can be useful in determining the etiology of hyperprolactinemia. Indeed, PRL levels > 250 ng/mL are highly suggestive of the presence of a prolactinoma. In contrast, most patients with stalk dysfunction, drug-induced hyperprolactinemia or systemic diseases present with PRL levels < 100 ng/mL. However, exceptions to these rules are not rare. On the other hand, among patients with macroprolactinomas (MACs), artificially low PRL levels may result from the so-called "hook effect". Patients harboring cystic MACs may also present with a mild PRL elevation. The screening for macroprolactin is mostly indicated for asymptomatic patients and those with apparent idiopathic hyperprolactinemia. Dopamine agonists (DAs) are the treatment of choice for prolactinomas, particularly cabergoline, which is more effective and better tolerated than bromocriptine. After 2 years of successful treatment, DA withdrawal should be considered in all cases of microprolactinomas and in selected cases of MACs. In this publication, the goal of the Neuroendocrinology Department of the Brazilian Society of Endocrinology and Metabolism (SBEM) is to provide a review of the diagnosis and treatment of hyperprolactinemia and prolactinomas, emphasizing controversial issues regarding these topics. This review is based on data published in the literature and the authors' experience.
Persistence of cortisol response (DeltaF > 193 nmol/l) to desmopressin in the early postoperative period can help to identify Cushing's disease patients with initial remission who present risk for later recurrence.
Thymic hyperplasia has been described after the resolution of hypercortisolism from several etiologies, causing great diagnostic dilemmas. We describe a case where the catheterization of the thymic vein was essential for the differential diagnosis of a thymic enlargement in an adrenalectomized patient with ACTH-dependent Cushing's syndrome. The patient was a 48-year-old female with clinical and laboratorial data suggesting Cushing's disease. She underwent a transsphenoidal surgery with no tumor visualization and no remission of the syndrome. Histopathological studies disclosed a normal pituitary. She underwent a bilateral adrenalectomy and 8 months later a chest CT showed an increase of left thymic lobe, which was previously non-existent. After a negative 111 In-pentetreotide scintigraphy, the patient underwent simultaneous and bilateral catheterism of the petrosus sinuses and catheterization of the thymic and inominate veins and no ACTH gradient was shown among the sites of collection. She did not undergo thoracotomy and a follow-up was established. During the evolution, there was a spontaneous regression of the thymic lesion 38 months after the diagnosis. The ACTH gradient during the catheterization of thymic vein was essential for the differential diagnosis of the thymic enlargement tumor after hypercortisolism resolution in ACTH-dependent Cushing's syndrome, especially in this case, where the ACTH source was occult, thus avoiding an invasive surgical procedure for a benign entity with spontaneous resolution.European Journal of Endocrinology 154 807-811
RESUMOCabergolina (CAB, Pharmacia) é um agonista dopaminérgico derivado do ergot com longa ação após administração oral, que já tem demonstrado ser de utilidade para o tratamento da hiperprolactinemia. Quarenta e cinco pacientes (36 mulheres, 9 homens) com prolactinomas (27 micro, 18 macro), intolerantes e/ou resistentes à bromocriptina (BRC) foram tratados com dose semanal de CAB de 0,25 a 7mg (mediana: 1 mg) dividida de 1 a 7 administrações. O tratamento, em compassionate basis, variou de 1 a 38 meses (mediana: 12 meses). Entre os 38 pacientes com intolerância persistente à BRC (sintomas digestivos, n=27; hipotensão postural, n=13; congestão nasal, n=5; manifestações psiquiátricas, n=4; retenção urinaria, n=1), somente 5 permaneceram intolerantes à CAB (sintomas digestivos, n=2; hipotensão postural, n=2; congestão nasal, n=1). Todos aqueles que toleraram bem a BRC também o fizeram com CAB. Onze casos alcançaram normalização da prolactina durante o uso de BRC. Estes pacientes e outros 19 também resolveram a hiperprolactinemia com CAB. Entretanto, somente 3 dos 7 pacientes resistentes (dose diária de BRC de 10 a 25mg) tiveram a prolactina sérica normalizada durante o uso de CAB. Redução tumoral foi documentada por ressonância magnética ou tomografia computadorizada em 7 macroprolactinomas durante o tratamento com CAB. Assim, devido à sua excelente eficácia, tolerabilidade e comodidade posológica, a CAB apresenta-se como importante alternativa no tratamento clínico dos prolactinomas. ABSTRACTCabergoline (CAB, Pharmacia) is a long-acting ergoline dopaminergic agonist for oral administration, already described as a useful drug for hyperprolactinemia therapy. Forty-five patients (36 women, 9 men) with prolactinomas (27 micro, 18 macro) with persistent intolerance and/or resistance to bromocriptine (BRC) were treated with a weekly CAB dose of 0.25-7.0mg (median: 1mg) divided in one to seven administrations along the week. The treatment, in compassionate basis, lasted from 1 to 38 months (median: 12 mo). Among the 38 BRC intolerant patients (digestive symptoms, n=27; orthostatic hypotension, n=13; nasal stuffiness, n=5; psychiatric manifestations, n=4; urinary retention, n=1) only 5 remained persistently intolerant to CAB (digestive symptoms, n=2; arterial hypotension, n=2; nasal stuffiness, n=1). All BRC tolerant patients accepted CAB well. Eleven cases had serum prolactin levels normalized with BRC. These patients and other 19 also reached normal prolactin levels with CAB. However, only 3 out of the 7 BRC resistant patients (daily dose: 10-25mg) had their serum prolactin levels normalized with CAB. Tumor shrinkage was documented by magnetic resonance imaging or computed tomography scanning in 7 macroprolactinoma patients during CAB treatment. Thus, due to its excellent efficacy, tolerability and low frequency administration,
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