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 (sarcoma, breast and brain neoplasms, leukaemia, laryngeal and lung carcinoma, adrenocortical carcinoma).

Immunohistochemistry

	a-Inhibin	100% (denominator is about 30, but not stated exactly for each antibody. strong and diffuse positivity)1
	Vimentin	84% (denominator is about 30, but not stated exactly for each antibody)1
	CK5/6	72% (denominator is about 30, but not stated exactly for each antibody)1
	CK7	27% (denominator is about 30, but not stated exactly for each antibody)1
	CK20	10% (denominator is about 30, but not stated exactly for each antibody)1
	AE1/AE3 and CK1	13% (denominator is about 30, but not stated exactly for each antibody)1
	Cam5.2	10% (denominator is about 30, but not stated exactly for each antibody)1
	EMA	0% (denominator is about 30, but not stated exactly for each antibody)1
	CEA	17% (denominator is about 30, but not stated exactly for each antibody)1
	Melan-A	30% (denominator is about 30, but not stated exactly for each antibody)1
	Synaptophysin	4% (denominator is about 30, but not stated exactly for each antibody)1
	Chromogranin	0% (denominator is about 30, but not stated exactly for each antibody)1
	Oestrogen receptor	3% (denominator is about 30, but not stated exactly for each antibody)1
	Androgen receptor	3% (denominator is about 30, but not astute exactly for each antibody)1
	bcl-2	8% (denominator is about 30, but not stated exactly for each antibody)1
	S-100	0% (denominator is about 30, but not stated exactly for each antibody)1
	Ki67	37% (denominator is about 30, but not stated exactly for each antibody)1
	p53	50% (denominator is about 30, but not stated exactly for each antibody)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:

• vena cava invasion
  

• necrosis
  

• mitotic activity >15/20 HPF

Other predictors on univariate analysis¹:

• tumour weight > 400g
  

• tumour size > 10.5 cm
  

• capsular or vascular invasion
  

• extension into periadrenal tissue
  

• severe nuclear atypia
  

• atypical mitotic figures

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