Cystic poorly differentiated nephroblastoma: A case report and review of literature

Abstract

Background: Cystic poorly differentiated nephroblastoma (CPDN) is a rare variant of nephroblastoma which follows a benign clinical course.
Case diagnosis/treatment: In this report, we document a case of CPDN in a 2 year old boy who presented with recurrent gross painless hematuria and progressive abdominal distension. Abdominal ultrasound showed a multicystic lesion and CT scan features of Stage III Wilms tumour. Nephrectomy was done after two cycles of chemotherapy according to the SIOP Nephroblastoma therapeutic protocols. Histology showed blastemal cells in the wall of only one of the cysts, with no solid expansile nodules. The patient had to have five more cycles of chemotherapy and also radiotherapy for residual tumour.
Conclusions: Surgery is curative in Stage I CPDN and adjuvant therapy is not required. Adequate sampling is critical to ensure accurate diagnosis and appropriate management. We suggest that a minimum of 2–3 tissue sections should be taken per centimetre of tumour diameter. Related entities including cystic nephroma, cystic Wilms tumour and completely necrotic nephroblastoma are discussed in the differential diagnosis.

KEYWORDS: Cystic poorly/partially differentiated nephroblastoma;Diagnosis; Clinical management; Cystic  nephroma; Cystic Wilms tumour; Completely necrotic nephroblastoma