Endocrine Abstracts

Introduction

Acromegaly is an uncommon chronic disease with an insidious course. Due to the high morbimortality it causes, its early diagnosis and treatment are priority. The three therapeutic pillars are surgery, pharmacological treatment and radiotherapy, alone or in combination.

Objectives

To clinically, biochemically and histologically characterize patients with acromegaly under follow-up in a tertiary hospital in Madrid since 2000.

Methods

Retrospective study including 97 adult patients diagnosed with acromegaly under follow-up in the Neuroendocrinology Unit of the Hospital Universitario La Paz, Madrid, during the period 2000-2020. Data were obtained by review of medical records. Continuous variables were expressed as mean and standard deviation and categorical variables as absolute values and percentages.

Results

Of the 97 patients with acromegaly, 96.7% (n=94) had a pituitary origin and 3.3% (n=3) had an ectopic one (bronchial, pancreatic and adrenal, 33% in each case). Mutations in GNAS and MEN1 genes were found, 1% in each case. 55% were women, age 61.53±16.5years, BMI 28.67±4.2kg/m2, annular circumference 20.4±1.5cm, GH 21.2±36.5µg/L and IGF-1 754.7±544.8µg/L. Only 58.6% were diagnosed during the first 5 years of disease. 76.3% were diagnosed by clinical manifestations secondary to hormonal hypersecretion, 15.8% by compressive clinical manifestations and 7.9% incidentally. 57.6% had pituitary macroadenoma, 36.5% had microadenoma, 2.4% had empty sella turcica and 1.2% had normal pituitary. 66.7% of the adenomas were densely granular, 58% somatropinomas, and 34.5% and 88.9% were positive for p53 and Ki67, respectively. 5.7% had pre-surgery hormonal deficits (4 patients with secondary hypothyroidism and 3 hypogonadotropic hypogonadism) and 48.9% had post-surgery hormonal deficits (22 patients with secondary adrenal insufficiency, 19 hypogonadotropic hypogonadism, 16 secondary hypothyroidism, 8 transient diabetes insipidus, 3 permanent diabetes insipidus, 2 transient SIADH, 2 biphasic response, 1 adult GH deficiency). Transsphenoidal surgery was the first line of treatment in 88.7% (n=86) of patients, achieving a cure rate of 53.4% (n=46). 13.3% were surgically reintervened and 26.5% also received RT. Of the 51 patients not cured, 94.1% (n=48) received medical treatment, achieving disease control in 92% of cases. The main comorbidities were: dysglycosis (53.6%), nodular thyroid disease (57.7%), left ventricular hypertrophy (69%), arterial hypertension (39.4%), osteopenia (34%) and obstructive sleep apnea-hypopnea syndrome (25%).

Conclusions

Timely diagnosis of early stage acromegaly remains a challenge in routine clinical practice. The best biochemical marker is IGF-1. In the hands of expert neurosurgeons, the treatment of choice remains transsphenoidal surgery. It is essential to integral approach associated comorbidities.