Hipertireoidismo relacionado à síndrome de McCune Albright: relato de dois casos e revisão da literatura

Abstract

McCune-Albright syndrome is a rare disease, combining polyostotic fibrous dysplasia, "café-au-lait" cutaneous spots and hyperfunctional endocrinopathies. Cases with hyperthyroidism are treated initially clinically, but definitive ablation with surgery or radioiodine treatment is the most indicated option.

Thyroid Abnormalities in Children and Adolescents with McCune-Albright Syndrome

“…In our experience, isolated sonographic alterations were prevalent, thus differing from descriptions in the literature showing functional alterations as being predominant in MAS [5,6]. Mastorakos et al [6] and Sallum et al [7] describe respectively 63 cases (52 in hyperthyroidism, 11 in euthyroidism) and 85 cases (55 with hyperthyroidism, 12 with subclinical hyperthyroidism, 18 in euthyroidism) of thyreopathy from 1936 to 1997, but they do not specify prevalence of this endocrinopathy in MAS. By contrast, in our group hyperthyroidism, although present in 11.11% as the second endocrinopathy, is less frequent, and far from precocious puberty that characterized 62% of patients.…”

“…Various therapeutic approaches to hyperthyroidism have been utilized such as medical treatment, surgery and radioactive iodine therapy. A medical approach with thionamides (methimazole or propylthiouracil) is usually chosen as the first treatment, as we did [5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26]. Nevertheless, medical treatment does not provide a definitive solution, and hematological and hepatic complications of thioamides may occur, especially for propylthiouracil [32,33].…”

“…Mastorakos et al [6] reviewed 63 cases of thyroid abnormalities from 1936 to 1997, including 19 nodular (14 with and 5 without hyperthyroidism) and 23 diffuse goiters (20 with and 3 without hyperthyroidism) and 18 cases of hyperthyroidism without goiter; Sallum et al [7] mentioned 85 cases of thyroid abnormalities, up to 2007 (55 with hyperthyroidism, 12 with subclinical hyperthyroidism, 18 without hyperthyroidism).…”

Spinal polyostotic fibrous dysplasia in two adults: Does only biopsy unravel the mystery?

“…It can be subclinical, overt or rarely present with thyrotoxic crisis. [1617] In a study from India six of the 17 adults had endocrinopathy, the most common being acromegaly in five and hyperthyroidism in one. [8] These patients require definitive form of therapy for hyperthyroidism as the disease invariably recurs after anti-thyroid drugs are stopped.…”

Radioiodine treatment in McCune-Albright syndrome with hyperthyroidism

“…The association of hyperthyroidism with polyostotic FD is uncommon. [4] We report another rare case who had FD and precocious puberty with hyperthyroidism and was treated with radioiodine, adding to the existing literature[5] that documents only six instances of radioablation in MAS.…”