A clinicopathologic profile of adrenocortical tumors.
OBJECTIVE
To study the clinical, biochemical, hormonal, radiological and histopathological profile of adrenocortical tumors in children; to assess the clinicopathological correlations and note the future outcome.


METHODS
Retrospective and prospective study.


METHODS
Hospital based; Endocrine Service of our institution and other institution based services.


METHODS
14 children (Females = 11, Males = 3) with adrenocortical tumor, aged 8 months to 13 years (mean age 5.1 +/- 3.42 years), seen over a period of 9 years.


RESULTS
Females predominated (F:M = 3.7:1). Majority (64%) had a mixed picture with cushingoid features and virilization, whereas 36% presented only for virilization. Elevated serum cortisol levels with loss of diurnal variation was noted only in those with mixed clinical presentation. Adrenal androgen elevation was noted in majority of cases as virilization was common to all. CT confirmed the diagnosis of tumor, 7 on either side. Thirteen cases were operated. Histopathologic diagnosis was carcinoma in 7 and adenoma in 6 cases. Three of the seven with carcinoma died within 3 months to 2 years but two of these with small tumours (weight 60-65 g and diameter < 6 cm) were well at 2 and 5 years, while as one of the six with a large adenoma had recurrence and metastasis after three years.


CONCLUSIONS
Female preponderance was marked (4 times), 43% of tumors had occurred by 3 years of age and 64% by 6 years. Neither the hormonal parameters nor the histopathology correlated well with the biological behavior and outcome. Prolonged and vigilant follow up is essential.
Upadhye
P S
PS
Bai Jerbai Wadia Hospital for Children, Parel, Mumbai.
Desai
M P
MP
Colaco
M P
MP
Desai
P B
PB
Deshpande
R K
RK
Mokal
R A
RA
D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D018246:Adrenocortical Adenoma / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D018268:Adrenocortical Carcinoma / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015994:Incidence; D007194:India / Q000453:epidemiology; D007223:Infant; D008297:Male; D011379:Prognosis; D011446:Prospective Studies; D012189:Retrospective Studies; D012307:Risk Factors; D017678:Sex Distribution; D015996:Survival Rate