Abstract.
Adrenocortical tumors (ACT) are rare in childhood. Determination of malignant potential is difficult. To assess the presentation, clinical behaviour, and histology of these tumors in our center, the records of nine patients with ACTs between 1989 and 2000 were analyzed. The age, sex, clinical presentation, investigations, treatment, follow-up data, and current status were determined. The pathological features were reanalyzed by our pathologist. The endocrine profile of the tumors was also retrieved from the records. There were two males and seven females; the median age at referral was 2.5 years. Two patients (one male, one female) presented with clinical features of Cushing's syndrome with hypertension. Four girls had features of Cushing's syndrome with associated virilization. One 11-year-old girl presented with hypertension, hypokalemia, and pseudoparalysis due to an aldosterone-secreting tumor. One girl presented with precocious pubic hair and clitoromegaly. One male had evidence of true precocious puberty. The endocrine profile was available in eight cases: cortisol levels were raised in four, testosterone in four, and aldosterone in one. One boy had pubertal levels of follicle-stimulating hormone and testosterone. Surgery was the only modality of therapy used. Histopathology showed capsular invasion in four patients and vascular invasion in two. The median tumor weight was 80 g (15–550 g) and the size ranged from 1.5 × 1.5 to 12 × 12 cm. Five patients are alive without tumor recurrence at a median follow–up time of 2 years. Tumor recurrence was seen in two patients, one with capsular rupture during surgery and another with a tumor weight of 16 g with associated capsular and vascular invasion. Atypical modes of presentation like pseudoparalysis must be kept in mind. Histologic criteria for malignancy in ACT are unreliable. Tumor weight may not always be accurate in predicting tumor behaviour. Complete surgical excision remains the only effective and potentially curative treatment.
Similar content being viewed by others
References
Mayer SK, Oligny LL, Deal C, Yasbeek S, Gagne N, Blanchard H (1997). Childhood adrenocortical tumors: case series and reevaluation of prognosis – a 24 year experience. J Pediatr Surg 32: 911–915
Liou LS, Kay R (2000) Adrenocortical carcinoma in children. Urol Clin North Am 27: 403–421
Wolthers OD, Cameron FJ, Schbeimberg I, et al (1980) Androgen secreting adrenocortical tumors. Arch Dis Child 80: 46–50
Teintuier C, Pauchard MS, Brugrieres PF(1999) Clinical and prognostic aspects of adrenocortical neoplasms in childhood. Med Pediatr Oncol 32: 106–111
Baranwal AK, Singhi SC, Narasimhan KL, et al (1999) Aldosterone producing adernocortical adenoma in childhood: a case report. J Pediatr Surg 34: 878–880
Abrams HL, Siegelman SS, Adams DF, et al (1982) Computed tomography versus ultrasound of the adrenal gland: a prospective study. Radiology 143: 121–128
Hanson JA, Weber A, Reznek RH, et al (1996) Magnetic resonance imaging of adrenocortical adenomas in childhood. Correlation with computed tomography and ultrasound. Pediatr Radiol 26: 794–799
Bugg MF, Reibeiro RC, Robertson PK, et al (1994) Correlation of pathologic features with clinical outcome in pediatric adrenocortical neoplasia. A study of Brazilian population. Am J Clin Pathol 101: 625–629
Sandrini R, Ribeiro RC, Delacerda L (1997) Extensive personal experience: childhood adrenocortical tumors. J Clin Endocrinol Metab 82: 2027–2031
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Narasimhan, K.L., Samujh, R., Bhansali, A. et al. Adrenocortical tumors in childhood. Ped Surgery Int 19, 432–435 (2003). https://doi.org/10.1007/s00383-002-0727-1
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00383-002-0727-1