Benign metastasizing leiomyomas (BMLs) occur predominantly in women during reproductive years. The condition is characterized by uterine leiomyomas associated with the development, typically years later, of slow-growing metastatic lesions. The most commonly affected organs are the lungs, but BMLs have been reported in lymph nodes, deep soft tissues, mesentery, bones, the central nervous system, and the heart. In many cases, these lesions have an indolent course and are discovered rather incidentally. However, occasionally they can present with debilitating symptoms or even life-threatening complications. The presence of estrogen and progester- U TERINE LEIOMYOMAS HAVE been reported in as-sociation with extrauterine benign-appearing smooth muscle tumors. This condition has been described mainly in women of reproductive age but can occur at any age. It has been referred to in the literature as benign metastasizing leiomyoma (BML). This term was introduced in 1939 by Steiner (1) in his report of a case of a 36-yr-old woman who died after massive pulmonary and hilar lymphatic metastases of a uterine leiomyoma. The interest of this condition to endocrinologists is its susceptibility to the hormonal milieu. Knowledge of this susceptibility may lead to effective clinical control of this metastasizing tumor by hormonal manipulation. We report two cases of BML in which hormonal manipulation with LHRH agonists, antiestrogens, and aromatase inhibitors (AIs) caused alleviation of symptoms and regression of the metastatic lesions. Case 1A 47-yr-old Caucasian woman consulted because of recurrent left subscapular pain that presented every few weeks with spontaneous resolution within a few days. Her complaint was initially attributed to cervical arthrosis and treated with analgesics. Three years later she developed numbness over her left hand, and a firm mass of about 5 cm was noticed in the left supraclavicular area (Fig. 1). A magnetic resonance image (MRI) revealed a large paraspinal tumor involving the brachial plexus and extending into the vertebral canal (Fig. 2). C5-T1 laminectomies, debulking of the extraspinal, and near complete removal of the intraspinal component was carried out. Pathological evaluation revealed a well-differentiated leiomyosarcoma [1-3 mitoses per 10 high-power fields (HPFs) and no necrosis] with bony involvement (Fig. 3).Radiotherapy was planned but the patient refused it. Shortly afterward she began experiencing cervical pain unrelieved by opioid analgesia, with a similar cyclical pattern to her previous pain; this prompted consultation to endocrinology. Her past medical history was remarkable for a total hysterectomy at age 36 yr for a 6-cm uterine leiomyoma. The histological blocks were retrieved and reexamined; the new report described a tumor with ill-defined infiltrative borders, areas of coagulative necrosis, mitotic rate of 4/10 HPFs, and few pathological mitotic figures but no significant cytological atypia. The conclusion was smooth muscle tumor of low malignant potential (2). The histol...

No abstract

Ergot-derived dopamine receptor agonists, especially pergolide and cabergoline, have been associated with an increased risk of valvular heart disease in patients treated for Parkinson's disease. Cabergoline at lower doses than those employed in Parkinson's disease is widely used in patients with prolactinomas, because of its high efficacy and tolerability; however, its safety with regard to cardiac valve disease is unknown. In order to assess the prevalence of cardiac valve regurgitation in patients with prolactinomas treated with long-term cabergoline, we performed a prospective and multicentric study including four university centers in the province of Quebec. A transthoracic echocardiogram was performed in 70 patients with prolactinomas treated with cabergoline for at least 1 year (duration of treatment, 55 +/- 22 months; cumulative dose 282 +/- 271 mg, mean +/- SD) and 70 control subjects matched for age and sex. Valvular regurgitation was graded according to the American Society of Echocardiography recommendations as mild, moderate, or severe. Moderate valvular regurgitation was found in four patients (5.7%) and five control subjects (7.1%) (P = 0.73). No patient had severe valvular regurgitation. There was no correlation between the presence of significant heart-valve regurgitation and cabergoline cumulative dose, duration of cabergoline treatment, prior use of bromocriptine, age, adenoma size, or prolactin levels. Our results show that low doses of cabergoline seem to be a safe treatment of hyperprolactinemic patients. However, in patients with prolonged cabergoline treatment, we suggest that echocardiographic surveillance may be warranted.

No abstract

There was an increase over time of referral for acromegaly management with female predilection. Initial higher IGF-1, but not GH levels, were predictive of co-morbidities and persistent active disease after treatment. Disease remission or control was attained in 70% of patients utilizing multimodal therapy.