adrenocortical carcinoma ( acc ) is a very rare entity in the pediatric population , and is rarely associated with virilizing symptoms .
the gland is grossly enlarged and the phenotypic features overlap with an adrenocortical adenoma , which need to be evaluated carefully , so as to distinguish it from the latter , as complete surgical excision is curative in an adenoma unlike in acc which may need adjuvant therapy and follow - up .
a two - year - old male child presented with features of virilization , which included growth of facial and pubic hair , deepening of voice , and penile growth for five months [ figure 1 ] .
the features of cushing syndrome ( moon face , pot belly , skin stria ) were not present .
ultrasonography showed a complex echogenic mass in the left paravertebral region . the child 's testosterone and dehydroepiandrostenidione levels were elevated and the serum cortisol level was within normal limits [ table 1 ] .
a provisional diagnosis of adrenocortical carcinoma was made , an adrenalectomy was done , and a histopathological diagnosis of adrenocortical carcinoma was made .
two - year - old male child with features of virilization ( pubic hair and penile growth ) preoperative and postoperative ( after two months ) hormonal profile grossly , the adrenalectomy specimen measured 11  9  7 cm and the weighed 240 g. the external surface was unremarkable .
[ figure 2 ] cut section of tumor showing reddish brown areas with areas of hemorrhage .
a narrow strip of normal adrenal was seen compressed to the periphery microscopy revealed large cells with mild - to - moderate pleomorphism .
the cells were arranged in sheets and trabaculae [ figure 3 ] ; > 75% of the cells had abundant eosinophilic cytoplasm .
a majority of the tumor had a diffuse architecture , interspersed with areas of hemorrhage ; there was no evidence of atypical mitosis or vascular and capsular invasion .
( h and e  100 ) photomicrograph showing cells having abundant eosinophilic cytoplasm and hyperchromatic nucleus with one - to - two nucleoli .
( h and e  400 ) based on the weight , size of the tumor , and microscopic features , and considering the criteria proposed by weiss , a diagnosis of adrenocortical carcinoma was made .
after the operation , the manifestations of virilization disappeared gradually , except for the lower voice .
grossly , the adrenalectomy specimen measured 11  9  7 cm and the weighed 240 g. the external surface was unremarkable .
[ figure 2 ] cut section of tumor showing reddish brown areas with areas of hemorrhage .
a narrow strip of normal adrenal was seen compressed to the periphery microscopy revealed large cells with mild - to - moderate pleomorphism .
the cells were arranged in sheets and trabaculae [ figure 3 ] ; > 75% of the cells had abundant eosinophilic cytoplasm .
a majority of the tumor had a diffuse architecture , interspersed with areas of hemorrhage ; there was no evidence of atypical mitosis or vascular and capsular invasion .
( h and e  100 ) photomicrograph showing cells having abundant eosinophilic cytoplasm and hyperchromatic nucleus with one - to - two nucleoli .
( h and e  400 ) based on the weight , size of the tumor , and microscopic features , and considering the criteria proposed by weiss , a diagnosis of adrenocortical carcinoma was made .
after the operation , the manifestations of virilization disappeared gradually , except for the lower voice .
acc and adrenocortical adenoma are the two ends of a spectrum that need to be differentiated .
these carcinomas are also seen with increased frequency in children with the li - fraumeni syndrome , beckwith - wiedermann syndrome , and congenital adrenal hyperplasia . a slight preponderance in the female population has been noted .
acc can be associated with cushing 's syndrome and minerlocorticoid production , although > 75% do not have any hormone overproduction .
grossly , carcinomas , as compared to adenomas , are large and weigh more than 100 g. it has been stated that any adrenal tumor weighing more than 250 g , age > 3.5 years at diagnosis , and associated with symptoms of marked hormone overproduction , is to be considered malignant with poor prognosis .
pure virilizing carcinomas , in general , appear to have a better prognosis than other adrenal carcinomas . in acc ,
the cut section of the gland is pink to yellow tan , with areas of hemorrhage and necrosis .
microscopically , the tumor has different patterns of growth and as the distinction between benign and malignant neoplasms is difficult , the weiss criteria , which require histopathological findings , are employed to distinguish the benign from the malignant tumors .
this classification is based on the mitotic index , confluent necrosis , atypical mitosis , and nuclear grade , and a tumor is rendered as malignant when the score is three or more than three .
our case has a score of 3 , although the weiss criteria is not useful in the pediatric population . hence , in our case , considering the weight and size of the gland , coupled with the microscopy , elevated hormonal levels , and the clinical symptoms , a diagnosis of acc was made . in our case ,
good prognostic factors were , weight less than 250 g , age less than 3.5 years , and a pure virilizing form of carcinoma .
all adrenocortical carcinomas are highly malignant tumors , and hence , they need to be resected .
mitotane , an insecticide derivative that produces adrenocortical necrosis , has been extensively used in adults with acc , but its efficacy in children who highlight a case of virilizing adrenocortical carcinoma , a very aggressive tumor , which is very rare in the pediatric age group and requires surgery , is not known . in this communication
 adrenocortical carcinomas are rare tumors with an incidence of one to two cases per million population and are still more rarer in the pediatric age group . 
 adrenocortical carcinomas can be functional or may be unassociated with syndromes of hormone overproduction . 
 it is very important to differentiate an adrenocortical adenoma from a carcinoma , as both share a large number of phenotypic features , and assess their prognosis , as adrenocortical carcinoma may need an adjuvant therapy . in this communication 
 , we describe the case of a two - year - old boy , who presented with iso - sexual precocious puberty , having features of virilization , which included growth of facial and pubic hair , deepening of voice , and penile growth . 
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