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Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 831-832
Cystic partially differentiated nephroblastoma with ureteric extension

1 Department of Paediatric Surgery, G Kuppusami Naidu Memorial Hospital, Coimbatore, India
2 Department of Pathology, G Kuppusami Naidu Memorial Hospital, Coimbatore, India

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Date of Web Publication27-Oct-2010

How to cite this article:
Ravi Kumar V R, Rajamani G, Raman M L. Cystic partially differentiated nephroblastoma with ureteric extension. Indian J Pathol Microbiol 2010;53:831-2

How to cite this URL:
Ravi Kumar V R, Rajamani G, Raman M L. Cystic partially differentiated nephroblastoma with ureteric extension. Indian J Pathol Microbiol [serial online] 2010 [cited 2021 Jul 25];53:831-2. Available from: https://www.ijpmonline.org/text.asp?2010/53/4/831/72067

A 1 year 5 month old male child with a clinical diagnosis of left renal tumor was subjected to computerized tomogram. It revealed a multiloculated cystic tumor with extension of tumor tissue into the upper third of the left ureter. The left kidney with the ureter and two small lymph nodes in the hilar region were removed after cystoscopy to exclude tumour deposits in the vesical mucosa.

Gross examination showed that the kidney was 8 × 6 × 4.5 cm and weighed 122 grams. The surface was multilobulated and the cut section of the ureter showed a tumor mass of 4 cm length in the upper third of the ureter extending down from the pelvis. The ureteric mucosa was free from the tumor tissue. Cut sections of the kidney showed multicystic lesions containing gelatinous material [Figure 1]. Histology of the nephoroureterectomy specimen including the ureteric mass showed the features of benign cystic partially differentiated nephroblastoma (CPDN). The septae in the cysts showed blastemal epithelium and mesenchymal elements. Abortive glomeruli and tubules were also seen. The cystic spaces were lined by hobnail epithelium. The adjacent kidney tissue showed tubules and glomerulus with thyroidization. Obvious malignant features were not present. Histological features were consistent with CPDN [Figure 2]. The ureteric mucosa was free from tumor [Figure 3]. The sections from the lymph nodes showed reactive lymphadenitis with follicular hyperplasia.
Figure 1: (a) Cystic nephroma specimen with the ureter. (b) Cut section showing gelatinous material. (c) Ureteric extension of the tumor. Mucosa of the ureter is free from tumor

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Figure 2: (a) Abortive glomeruli (H and E, ×100) (b) Loose mesenchyme of cystic nephroma (H and E, ×100) (c) Cystic nephroma with blastema, abortive glomeruli and tubules (H and E, ×100) (d) Blastema and tubules under high power (H and E, ×200)

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Figure 3: Ureteric mucosa free form tumor low (H and E, ×40) and high power view (H and E, ×400)

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Cystic renal tumors of childhood represent the spectrum of lesions that include cystic nephroma (CN), CPDN, and Wilms' tumor (WT) with multicystic areas. CN is a benign multilocular tumor composed of noncommunicating cysts separated by septa containing mature elements only without blastema or other embryonal elements. CPDN is a low-risk malignant tumor characterized by the presence of the partially differentiated elements (blastema) within the septa. WT with multicystic areas contains both cystic and solid areas composed of the same elements as classical WT.

Despite the generally benign outcome associated with CPDN, occasional recurrences develop. Joshi and Beckwith, [1] in a review of 18 cases of cystic renal tumors, subclassified CPDN into two subtypes - CPDN with relatively mature type as grade 1 and those with relatively immature subtype as grade 2. Among the 18 cases reported by them, two tumors partially herniated into the pelvicalyceal system. In one case, CPDN was associated with anaplastic WT on the opposite side. [1]

Ureteric extension in WT is well known and alters the prognosis. Hence histological proof is necessary of the ureteric mass to exclude WT into ureter. In this case, the histology is that of CPDN. CPDN is known to produce local recurrences though it is rare. Ureteric extension in CPDN will need total ureterectomy and regular cystoscopy follow up to exclude local recurrence. Cystoscopy follow-up is not necessary if the CPDN is confined to kidney only. Since the prognosis is altered, the ureteric extension in CPDN has special significance.

CPDN appears to take a benign course, and simple nephrectomy seems to be the treatment of choice. However, in view of the possibility of recurrence regular follow-up is recommended. [2] CPDN may undergo local recurrence but there is no report of metastasis. [3] Partial nephrectomy in selected cases has also been successful. [4] Stage II patients also have excellent outcome when treated with tumor resection and postoperative chemotherapy. [5]

In the case reported above, the histological feature was that of classical CPDN but with ureteric tumor mass. It is extremely rare for the CPDN to present with ureteric extension though pelvic involvement has been reported. [1] The patient is on regular follow up with ultrasound for local recurrences and scheduled for cystoscopy screening every 6 months for 2 years.

   References Top

1.Joshi VV, Beckwith B: Multilocular cyst of the kidney (Cystic Nephroma) and cystic partially differentiated Nephroblastoma. Cancer 1989;64:466-79.  Back to cited text no. 1
2.Joshi VV. Cystic partially differentiated nephroblastoma: An entity in the spectrum of infantile renal neoplasia. Perspect Pediatr Pathol 1979;5:217-35.  Back to cited text no. 2
3.Puvaneswary M, Mcintosh J, Cassey J. Cystic partially differentiated nephrobalstoma: Australas Radiol 2006;502:55-7.  Back to cited text no. 3
4.Rajangam K, Narasimhan KL, Trehan A, Rawal A, Radotra B, Rao KL. Partial nephrectomy in cystic partially differentiated nephroblastoma. J.Pediatr surgery 2000;35:510-2.  Back to cited text no. 4
5.Luthile T, Szavay P, Furtwangler R, Graf N, Fuchs J; SIOP/GPOH study group. Treatment of cystic nephroma and cystic partially differentiat nephroblastoma: A report from SIOP/GPOH study group. J Urol 2007;177:294-6.  Back to cited text no. 5

Correspondence Address:
V R Ravi Kumar
Consultant Paediatric surgeon, G. Kuppusami Naidu Memorial Hospital, Pappanaickenpalayam, Coimbatore - 641 037
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.72067

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  [Figure 1], [Figure 2], [Figure 3]


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