Primitive neuro-ectodermal tumour of kidney in adult : report of four consecutive cases and review of the literature

Abstract EN

Background : PNET of kidney is a rare entity and its diagnosis is complicated by the presence of a number of differential diagnoses.

The disease is most commonly seen in young adults.

Radical nephrectomy and adjuvant chemotherapy is the standard treatment.

However, the patients have a modest survival and often develop distant metastasis.

We herein report four cases of renal PNET (rPNET).

Methodology : We retrospectively retrieved treatment chart of four cases of rPNET.

Results : Median age was 29 years.

Radical nephrectomy was performed in three cases.

All four cases received multiagent chemotherapy.

VAC alternating with IE was the commonest regimen.

Compliance and tolerance to treatment was excellent.

At the last follow up two patients were in complete remission whereas the remaining two cases had systemic metastasis and alive with disease.

Conclusion : Multimodality approach is required in rPNET.

Patient with localized disease appears to have better disease control and survival.