Paediatric renal cell carcinoma

Definition

This is a rare provisional subtype occurring in patients between 5 and 30 years of age, without an underlying predisposing syndrome.

Clinical features

These patients do not have von Hippel Lindau syndrome or tuberous sclerosis. There is a possible association with neuroblastoma².

Radiology

Macroscopic appearances

Tumours often present at an advanced stage.

Histopathology

Cells form both nests and papillae. There is voluminous cytoplasm, clear to granular. Calcification is common.

Immunohistochemistry

	Cytokeratins	negative or weak
	EMA	negative or weak

Cytogenetics

Abnormalities involving chromosomes 1 and X are common. There may be two subtypes:

• t(1;X), fusing the TFE3 gene on chromosome X and the PRCC gene on chromosome 1.

• t(17;X), fusing the TFE3 gene on chromosome X and the ASPS gene on chromosome 17.

Differential diagnosis

In the absence of von Hippel Lindau syndrome or tuberous sclerosis, other renal cell carcinomas are even rarer between the ages of 5 and 20 years.

• Conventional renal cell carcinoma

Prognosis

This is an aggressive tumour.

References

¹Renshaw, A. A. (2002). "Subclassification of renal cell neoplasms: an update for the practising pathologist." Histopathology 41(4): 283-300.

²Medeiros, L. J., G. Palmedo, et al. (1999). "Oncocytoid renal cell carcinoma after neuroblastoma: a report of four cases of a distinct clinicopathologic entity." Am J Surg Pathol 23(7): 772-80.

This page last revised 6.1.2004.

©SMUHT/PW Bishop