ADRENOCORTICAL ADENOMA AS A CAUSE OF HYPERANDROGENISM SYNDROME IN A GIRL: THE PATH TO DIAGNOSIS

Abstract

Introduction. Hyperandrogenism syndrome in girls is a symptom complex that develops in the female body due to an excess of androgens or an increase in their effects in target tissues. With hyperandrogenism, which began in early childhood, clinical manifestations are possible in the form of accelerated growth and rates of ossification, premature adrenarche, acne. One of the causes
of hyperandrogenism syndrome is a tumor of the adrenal cortex (OCN). The level of hormonal activity of OCN in children is 90 % compared to 50 % in adults. While adults usually have the  secretion of one hormone, children often have the secretion of several hormones from different layers of the adrenal cortex. Objective: to conduct a comprehensive laboratory and instrumental examination in a preschool-age girl with hyperandrogenism syndrome to clarify its cause and optimize therapy. Description of the clinical case. The girl M., aged 4 years and 7 months, was admitted to the St. Petersburg State Medical University Clinic due to a large increase in weight (4 kg in 3 months), acne rashes on the skin of the forehead and wings of the nose, hair loss of the labia majora and pubic area, hypertrophy of the clitoris up to 2.0–2.5 cm; at the same time, separate entrances to the urethra and vagina were visualized. The sexual formula according to Tanner: A 1–2 P3 Ma1 Iu(–). Subcutaneous fat is excessively developed in the cheeks and abdomen (kushingoid features of the phenotype).  Karyotype 46XX. Average height 106 cm (–0.38 SDS), weight 19 kg, BMI =16.9 kg/m2 (+1.03 SDS). The clinical symptom complex was associated with severe hyperandrogenemia, represented by high levels of testosterone, androstenedione and 17-OH progesterone, and moderately elevated levels of DHEA sulfate. At the same time, a violation of the circadian rhythm of cortisol attracted attention. Ultrasound examination revealed a formation in the left adrenal gland. 6,6 × 7,2 × 7,0 cm of cystic solid type, heterogeneous echostructure, with perinodular blood flow, without a clearly defined capsule. It was impossible to rule out a tumor of the left adrenal gland, the secret. Conclusion. In the case of patient M. with hyperandrogenism syndrome, the final diagnosis of "adrenocortical adenoma" was established after 5 months after the appearance of the first clinical manifestations of virilization, which coincides with the literature data. The diagnostic search and radical surgical treatment in this case took 20 calendar days. This clinical case is an example of a diagnostic search for the cause of hyperandrogenism syndrome in young girls. To verify the hormone-producing formation of the adrenal gland as the cause of hyperandrogenism syndrome, it is diagnostically significant to conduct a comprehensive examination, including biochemical, hormonal, instrumental examination methods with the examination of specialized specialists.