Adrenocortical tumours in children: our experience with nine cases

Abstract

Nine cases of adrenocortical tumor are presented, six were males. Four were less than three years and five were between 5 and 10 years of age. Clinical virilization was found in 8 children, one had only signs of hypercortisolism and another showed signs of virilization and hypercortisolism simultaneously. Urinary 17-KS and 17-OHCS were high in all patients. Plasma levels of testosterone and of the other adrenal androgens were high in all the cases tested. Plasma level of cortisol was elevated only in few cases. In two out of five cases steroids were only partially suppressed by dexamethasone. Computed tomography and abdominal sonography have been useful tods for the localization of the tumour. The resection of the tumour, indipendent of hystopathological diagnosis, led to a complete normalization of the clinical and hormonal picture in eight cases evaluated at a distance of 2 months 10 years after surgery. In one case a hepatic metastasis was observed and removed three years after surgery. Adrenocortical tumours are rare (Javadpour et al., 1980) and the vast majority of them are hormone secreting. Although cases have been described which showed feminilization and hyperaldosteronism (Bacon & Lowrey, 1965; Bhettay & Bormici, 1977; Crane et al., 1961; Gauguly et al., 1980), virilization and hypercortisolism are most commonly found (Hayles et al., 1966). This paper illustrates our experience with nine cases of adrenocortical tumour, eight of whom showed inappropriate virilization with or without hypercortisolism and one of whom was a classic case of Cushing's syndrome.

Pseudo-precocious Puberty Associated with an Adrenocortical Tumor in a Young Child

“…A literature review of case reports revealed that virilization was considered a good clue for suspecting ACT, similar to the presentation in this case [11][12][13]. Razavi reported a 10-monthold boy diagnosed with ACT, who presented with sexual pubic hair, acne, and seborrhea dermatitis [3].…”

“…ACT can be benign or malignant [3]. The most common clinical presentation of ACT in children is pseudo-precocious puberty, which is observed in approximately 50%-84.2% of cases or as Cushing's syndrome in the remaining cases [12][13]. However, malignancy should not be underestimated in this age group [2].…”

Combination chemotherapy for adrenal carcinoma: Response in a 5 1/2‐year‐old male

“…Adrenocortical carcinoma is a rare tumour of childhood [ 11. Primary surgical resection may be curative [2], but prognosis is dependent on tumour size and location [3-51. Although current reports in the paediatric literature on outcome are more optimistic [6], occurrence of metastatic disease still carries on almost uniformly fatal outcome.…”