Need for Comprehensive Hormonal Workup in the Management of Adrenocortical Tumors in Children

Abstract

Ob­jec­ti­ve: Clinical findings do not reflect the excess hormonal status in adrenocortical tumors (ACTs) in children. Identification of abnormal hormone secretion may help provide the tumor marker and delineate those patients with a risk of adrenal suppression following tumor removal. To analyze the impact of complete hormonal assessment regardless of the clinical presentation in hormone-secreting ACTs in childhood. Methods: Association of hormonal workup at diagnosis with the clinical findings and frequency of adrenal suppression postoperatively were analyzed in 18 children with ACT.Results: Seventeen of the 18 patients had functional ACT. Clinical findings suggested isolated virilization and isolated Cushing’s syndrome in 38.8% and 17.6% of patients, respectively. Hormonal workup revealed a frequency of 83.3% for hyperandrogenism. The majority of the tumors (50%) had mixed type hormonal secretion. Hypercortisolism existed in 28.5% of children with isolated virilization and hyperandrogenism was found in 2/3 of children with isolated Cushing’s syndrome. Various androgens other than dehydroepiandrosterone sulfate were also determined to be high in hyperandrogenism. Increased testosterone was a highly prevalent tumor marker. Nine patients (3 with no signs of hypercortisolism) had adrenal suppression following tumor removal which lasted 1-24 months.Conclusion: Complete hormonal workup showed the predominance of mixed hormone-secreting type of tumor in the patients who lacked the appropriate clinical findings and also showed that patients lacking signs of Cushing’s syndrome could have postoperative adrenal suppression. Clinical findings may not reflect the abnormal hormone secretion in all cases and tumor markers as well as risk of postoperative adrenal suppression can best be determined by complete hormonal evaluation at the time of diagnosis.

IGF2 and IGF1R in pediatric adrenocortical tumors: roles in metastasis and steroidogenesis

“…Adrenocortical tumors are frequently characterized by hormonal secretion, inducing clinical symptoms of the disease or revealing tumor recurrence during followup (Gönç et al 2014). As expected, most of the children evaluated presented pure androgen or mixed (androgen and cortisol) secreting tumors, while only two patients presented cortisol secretion alone.…”

Çocuk Ve Adolesanlarda Hashi̇moto Ti̇roi̇di̇ti̇ / Hashimoto Thyroiditis in Children and Adolescents

“…Hem erkekte hem kadınla yaşla insidans artmaktadır. Beyazlarda ve Asyalılarda, Afrikalı Amerikanlara göre daha sık saptanmaktadır (2)(3)(4)7).…”

“…Diğer nonspesifik semptomlar araştırılırken ya da herhangi bir semptom yok iken rutin tetkiklerinde tiroid fonksiyon testlerinde bozukluk saptanabilir. Hastalarda ötiroidi, subklinik hipotiroidi, aşikar hipotiroidi, aşikar hipertiroidi ve subklinik hipertiroidi bildirilmiştir (2,18).…”

“…HT'nin erken döneminde Graves Hastalığı ile karışabilen hashitoksikoz kliniği görülebilir. Eşlik edebilecek diğer otoimmün hastalıklar addison hastalığı, alopesia areata veya totalis, pernisiyöz anemi (otoimmün gastrit), kronik aktif hepatit, idiopatik hipoparatiroidizm, primer bilier siroz, primer ovariyan veya testikuler yetmezlik, romatoid artrit, sjögren sendromu, sistemik lupus eritematozus, skleroderma, tip 1 diyabet ve vitiligo'dur (2,18,19).…”

“…Dört yaş altında nadir olmakla birlikte süt çocukluğu döneminde de bildirilmiştir. Tiroid hormonlarının santral sinir sistemi, büyüme gelişme, puberte ve kemik metabolizması üzerine etkileri bilinmektedir ve her yaşta erken tanı ve tedavisi çok önemlidir (2)(3)(4).…”