Occult ectopic adrenocorticotropic hormone syndrome.
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ACTH Syndrome, Ectopic↗
Biomedical subjects
A M Makowska
Publications and source records attributed to A M Makowska.
Incidentally found adrenocortical carcinoma. A study of 21 patients.
The frequency of adrenocortical carcinoma was studied in a group of 311 incidentally discovered adrenal tumours. Clinical characteristics were also analysed. Ultrasound scan and computed tomography were the main imaging techniques used. Hormonal examinations were also carried out. The patients with an adrenal tumour diameter greater than or equal to 4.0 cm, and those with excess steroid production were recommended for surgery. Of 131 patients treated with surgery, adrenocortical carcinoma was diagnosed in 21 cases. The diameter of these tumours ranged between 3.2 and 20.0 cm. The majority of these were hormonally inactive, but, in some cases increased corticosteroid secretion was noted. In 17/21 patients, mitotane was administered following surgery, with a good response in 13 cases. These 21 cases were compared with a group of 51 patients with clinically overt adrenocortical carcinoma.
Adolescent↗
Secondary adrenal insufficiency associated with autoimmune disorders: a report of twenty-five cases.
OBJECTIVE: Addison's disease is frequently a component of autoimmune polyendocrinopathies while secondary adrenal insufficiency associated with autoimmune disorders is believed to be a rare event. We present a series of patients with secondary adrenal insufficiency coexisting with autoimmune diseases and/or antithyroid autoantibodies. DESIGN AND PATIENTS: Among a group of 102 patients with secondary adrenal failure of unknown origin diagnosed at the Department of Endocrinology of the Centre for Postgraduate Medical Education (Warsaw, Poland) we have identified a group with associated autoimmune disorders. Thyroid abnormalities occurred most frequently. Other diseases included insulin-dependent diabetes mellitus, pernicious anaemia, vitiligo, premature ovarian failure and autoimmune thrombocytopaenia. There were 23 women and one man aged 17-72 years at the time of investigation. Additionally, we included a woman with Addison's disease in whom the ACTH deficiency appeared 18 years after the onset of primary adrenal hypofunction. MEASUREMENTS: Pituitary-adrenal function tests comprised urinary excretion of 17-hydroxycorticosteroids in basal conditions and during a 2-day tetracosactrin test, plasma concentrations of ACTH and cortisol, and a 2-day metyrapone test (in eight cases). Thyroid function and immunity tests were: TSH, thyroxine, the antithyroglobulin, antimicrosomal and anti-peroxidase autoantibodies. Other endocrine studies included: serum LH, FSH and PRL. RESULTS: The 17-hydroxycorticosteroid values, both basally and during stimulation tests were consistent with a diagnosis of secondary adrenal insufficiency. Serum cortisol and plasma ACTH concentrations were low. In 14 patients primary hypothyroidism was confirmed by low T4 levels. In three patients subclinical primary hypothyroidism was revealed (elevated TSH levels). Three patients who had a past history of Graves' disease were euthyroid at the time of investigation. Twenty-three patients had antibodies against peroxidase. Most patients had gonadotrophins and PRL values within normal limits. CONCLUSIONS: The co-existence of autoimmune disorders with secondary adrenal insufficiency suggests an autoimmune aetiology for the ACTH deficiency.
Adolescent↗
Impact of adjuvant mitotane on the clinical course of patients with adrenocortical cancer. Two years later.
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Adrenal Cortex Neoplasms↗
Adrenocortical carcinoma. A clinical study and treatment results of 52 patients.
BACKGROUND: Adrenocortical carcinoma is a rare tumor with a poor prognosis. This work was aimed at analyzing the clinical outlook and treatment results of 52 patients with this disease. METHODS: This study included patients with adrenocortical carcinoma referred to the Department of Endocrinology at the Center of Postgraduate Medical Education (Warsaw, Poland) during the last 30 years. In 11 patients, the adrenal tumor was found incidentally by ultrasonographic scan. Hormonal examinations made it possible to define the endocrine activity of the tumors, whereas imaging techniques helped to determine their staging. Forty-eight patients underwent surgery, and 36 of them received mitotane. This drug was administered to 26 patients for a range of 10 months to 10 years; 13 patients received mitotane immediately after the operation, and 13 others after a delay. The patients with severe hypercorticism were pretreated before surgery with aminoglutethimide and mitotane. RESULTS: The study comprised 10 men and 42 women; hormonally active tumors were diagnosed in 39 of them. Cushing's syndrome was the most frequent entity. At diagnosis, 17 cases were classified as localized disease, 15 as regional disease, and 20 as distant disease. Pretreatment with the inhibitors of steroidogenesis improved the survival perspectives in the early postoperative period. As of this writing, there were 12 survivors in the group of 26 patients treated by surgery and long term mitotane therapy and only 2 survivors of 7 patients treated with surgery only. CONCLUSIONS: Surgery with immediate adjuvant long term mitotane administration was the most effective form of therapy for patients with adrenocortical carcinoma.
17-Hydroxycorticosteroids↗