Cystic partially differentiated nephroblastoma: Report of a rare case
Introduction: Cystic partially differentiated nephroblastoma(CPDN) is a rare tumour of infancy which is considered to be a low risk malignant neoplasm. It has a favourable outcome as compared to classical Wilms tumor.
Case history: A 10 month old male child presented with abdominal lump of two months duration. Ultrasound abdomen revealed a cystic mass involving most of the left kidney. After nephrectomy we received a specimen of kidney measuring 7x6x5 cms. Multiple cystic areas were seen along with areas of hemorrhage and a narrow rim of normal kidney.On microscopy, multiple cysts of varying sizes were seen lined by cuboidal, flattened, eosinophilic and hobnail cells. The septa were relatively thick containing bland spindle cells with few foci of blastemal cells .A diagnosis of cystic partially differentiated nephroblastoma was made.
Conclusion: CPDN is a rare variant of Wilms tumor with a favorable prognosis. Histopathologic examination helps to differentiate it from other cystic lesions of the kidney and is of therapeutic importance.
Full Text:FULL TEXT PDF
Bindhu J,Imtiaz A, Kumar RV, Thejaswini MDRT. Cystic variant of favorable-histology Wilms’ tumor presenting with osteolytic metastasis to the ribs. J Postgrad Med. 2010,56:28-30.
Eble JN, Bonsib SM. Extensively cystic renal neoplasms: cystic nephroma, cystic partially differentiated nephroblastoma, multilocular cystic renal cell carcinoma and cystic hamartoma of renal pelvis. SeminDiagnPatho. 1998;15:2-20.
Parikh KS, Shukla NS, Shah MP et al. Cystic partially differentiated nephroblastoma. Indian J Med PediatrOnco. 2004; 24:34-7.
Blakely ML, Shamberger RC, Norkool P, et al. Outcome of children with cystic partially differentiated nephroblastomatreated with and without chemotherapy. J Pediatr Surg. 2003; 38: 897-900.
Joshi VV, Beckwith JB. Multilocular cyst of the kidney (cystic nephroma) and cystic partially differentiated nephroblastoma.Terminology and criteria for diagnosis. Cancer. 1989; 64: 466-79.
Singh S, Gupta R, Khurana N. Cystic partially differentiated nephroblastoma: a rare differentiated variant of Wilm’s tumour. J Postgrad Med. 2006; 52:45-6.
Agrons GA, Wagner BJ, Davidson AJ, Suarez ES. Multilocular cystic renal tumor in children: radiologic-pathologic correlation. RadioGraphics. 1995; 115:659-660.
Joshi VV, Banerjee AK, Yadav K, Pathak IC. Cystic partially differentiated nephroblastoma: a clinicopathologic entity in the spectrum of infantile renal neoplasia. Cancer. 1977; 40: 789-795.
Joshi VV, Beckwith JB. Pathologic delineation of the papillonodular type of cystic partially differentiated nephroblastoma. A review of 11 cases. Cancer. 1990; 66:1568-77.
Rajangam K, Narasimhan KL, Trehan A, et al. Partial nephrectomy in cystic partially differentiated nephroblastoma. J Pediatr Surg. 2000; 35:897-900.
Sonia KP, Shilin NS, Pankaj MS, et al. Cystic partially differentiated nephroblastoma. Indian J Med Pediatr Oncol. 2004; 25:34-7.
- There are currently no refbacks.
Annals of Pathology and Laboratory Medicine (APALM) is licensed under a Creative Commons Attribution 4.0 International License. Based on a work at APALM