Biomedical subjects
Publications and source records attributed to P Limone.
Primary pigmented micronodular disease is a peculiar form of ACTH-independent Cushing's syndrome characterized by the familial occurrence, the frequent association with malformations and the pathological adrenocortical picture consisting in micronodules with cellular deposition of lipofuscinic pigment. We describe here a case occurring in a 14-year-old girl.
Hyperlactinemia is the most commonplace expression of hypophyseal pathology, above all in females, and is one of the most frequent causes of sexual disorders. The present study examined the etiopathogenesis of hyperprolactinemic syndromes and discusses their pathophysiology, paying special attention to neuroendocrine alterations in the presence of either prolactin-secreting adenoma or other forms of hyperprolactinemia. Their clinical manifestations are then described together with a detailed review of therapies, especially for prolactinoma. Both general opinion and the authors' personal point of view are reported regarding the indications for medical and surgical treatment for micro and macro-prolactinomas. Lastly, particular aspects are taken into consideration, such as the relationship between hyperprolactinemia and pregnancy, in the light of the most recent findings on the natural evolution of prolactinemia and the possibility of treatment using dopaminergic drugs.
Prolactin-secreting adenomas are the most common type of pituitary tumors. In this paper the Authors review the therapeutic strategies based on the distinction between micro- and macro-adenomas.
The dopaminergic drugs have a pivotal role in the treatment of prolactin-secreting pituitary tumors. The Authors examine the available drugs and delineate the guidelines for the medical therapy of this neoplasia.
Two cases of pituitary-dependent Cushing's syndrome are described in which the computed tomography (CT) examination was negative; as the hormone dynamic investigations were directed towards the presence of corticotropin (ACTH) secreting pituitary formations, magnetic resonance imaging (MRI) of the pituitary was performed, which evidenced the presence of such lesions; subsequent neurosurgery confirmed in both cases the location indicated by MRI. In conclusion, the higher sensitivity of MRI compared to CT in the diagnosis of ACTH secreting pituitary adenomas can be noted.
A family with Sipple's syndrome was studied over three generations. The syndrome is transmitted as a dominant autosomic hereditary trait with high penetrance and varying expressiveness. Our series include 13 patients of three generations: 1) husband and wife who were 1st degree cousins; 2) 4 sisters and 1 brother; 3) 5 sisters and 1 brother. In the first generation we did not find any sign that could be related to the syndrome, in the second generation 2 thyroid carcinomas and a pheochromocytoma were present, and in the third one 2 sisters had these neoplasias, one pheochromocytoma and the other thyroid cancer, another one had marfanoid habitus and the fourth one had pheochromocytoma. This series includes 11 subjects of the second and third generation; the Sipple's syndrome was present in 7 females. The incidence of the syndrome was 63.8% and present only in female subjects. The high incidence rate could be caused by the strict relation between the grandparents. The diffusion of this pathology may be related to the high penetrance of the gene(s) involved whereas the clinical variations may be interpreted to the variable expressiveness and, even more so, as evidence that the other manifestations of the syndrome may occur later in time.
Adrenal feminizing tumours are rare but frequently malignant. A case of adrenal feminizing tumour in a 35-year-old man, which presented with gynaecomastia and infertility due to azoospermia, without loss of libido and sexual potency is reported in this paper. Our patient represents a relatively unusual case. The long free interval from the excision allows us to consider the patient cured as despite the pathological malignant features of the neoplasm.
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