Adrenal cortical paediatric neoplasms

Epidemiology

Paediatric adrenal cortical neoplasms are rare, most being carcinomas.

Clinical features

In contrast to adults, paediatric tumours are often hormonally active: children often present with virilisation or Cushing's syndrome, rarely with feminisation or Conn's syndrome. Congenital syndromes associated with paediatric adrenocortical neoplasms include hemihypertrophy, urinary tract abnormalities, adrenal cytomegaly, Beckwith-Wiedemann syndrome, Li-Fraumeni and SBLA.

Immunohistochemistry

 

a-Inhibin

100%1

 

Vimentin

84%1

CK5/6

72%1

CK7

27%1

CK20

10%1

AE1/AE3 and CK1

13%1

Cam5.2

10%1

EMA

0%1

CEA

17%1

Melan-A

30%1

Synaptophysin

4%1

Chromogranin

0%1

Oestrogen receptor

3%1

Androgen receptor

3%1

bcl-2

8%1

S-100

0%1

Ki67

37%1

p53

50%1

   

Expression of cytokeratins is generally weak.

Differential diagnosis

The strong positivity for inhibin may be diagnostically useful.

Prognosis

Independent predictors of malignant behavior on multivariate analysis are1:

Other predictors on univariate analysis1:

References

1Wieneke, J. A., L. D. Thompson, et al. (2003). "Adrenal cortical neoplasms in the pediatric population: a clinicopathologic and immunophenotypic analysis of 83 patients." Am J Surg Pathol 27(7): 867-81.

This page last revised 14.1.2004.

©SMUHT/PW Bishop