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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
CASE REPORT  
Year : 2011  |  Volume : 22  |  Issue : 4  |  Page : 779-781
Cystic renal neoplasm causing hypertension in a 2-year-old child


1 Department of Medical Oncology, Cancer Institute (W.I.A.), Adyar, Chennai, India
2 Department of Surgical Oncology, Cancer Institute (W.I.A.), Adyar, Chennai, India

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Date of Web Publication9-Jul-2011
 

   Abstract 

Cystic neoplasms of kidney in pediatric age group include a wide variety of tumors, both of benign and malignant pathology. In a child, renal neoplasms with associated hypertension are rare. Here, we present a 2-year-old child who had a multicystic renal tumor with hypertension. She underwent radical nephrectomy subsequent to which hypertension disappeared. The postoperative pathology revealed multicystic nephroma.

How to cite this article:
Babu S, Agarwal R, Narayansamy K, Rajendranath R, Balasubramanian S. Cystic renal neoplasm causing hypertension in a 2-year-old child. Saudi J Kidney Dis Transpl 2011;22:779-81

How to cite this URL:
Babu S, Agarwal R, Narayansamy K, Rajendranath R, Balasubramanian S. Cystic renal neoplasm causing hypertension in a 2-year-old child. Saudi J Kidney Dis Transpl [serial online] 2011 [cited 2020 Jun 5];22:779-81. Available from: http://www.sjkdt.org/text.asp?2011/22/4/779/82692

   Introduction Top


Cystic neoplasms of kidney in pediatric age group include a wide variety of tumors comprising both benign and malignant neoplasms. For cystic neoplasms, the differential diagnoses include simple cysts of kidney, cystic lesions as a part of unilateral autosomal dominant polycystic disease, multicystic nephroma, partially differentiated cystic nephroblastoma (CPDN) and cystic degeneration in malignant tumors of kidney. [1],[2],[3] Hypertension in pre-adolescent age group is most commonly secondary. [4] Among the tumors associated with hypertension in this age group are neuroblastoma, other adrenal tumors including pheochromocytoma, functioning adrenal tumors and Wilms' tumor. Here, we present a case of multicystic tumor of kidney with hypertension in a 2-year-old child.


   Case Report Top


A 2-year-old girl presented with history of fullness in flank of two months duration. She was also detected to have hypertension (170/110 mmHg). She was born out of a non-consanguineous marriage and had attained the milestones normally. There was no history of any congenital malformations. On clinical examination, a firm mass of size 8 × 8 cm was palpable in right hypochondrium and lumbar region. Urinary vanillyl mandelic acid (VMA) level was marginally elevated. There was no evidence of congenital cardiac anomaly including coarctation of aorta. The hypertension was controlled with calcium channel blocker drug, nifedipine. Computed tomography of abdomen revealed a multiloculated cystic mass measuring 7.8 × 7.5 cm in the upper and mid portions of right kidney [Figure 1]. The capsule and the septae were found partially enhancing with the contrast. There was no solid area in the cysts. Patient underwent radical nephrectomy. Grossly, the tumor was well encapsulated with normal appearing renal parenchyma compressed on the posterior aspect. Cut section revealed multiple cysts ranging from 0.5 to 2 cm in size and they were non-communicating [Figure 2]. There was encroachment of cyst into renal pelvis. Histopathology demonstrated multicystic nephroma as the septae of cysts demonstrated fibrous septae with mature renal tubules and absence of blastemal components.
Figure 1: CT scan showing multicystic renal tumor.

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Figure 2: Cut section of tumor showing multiple non-communicating cysts.

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   Discussion Top


Cystic nephroma is a rare benign multicystic renal tumor. This was first reported by Edmunds in 1892 as a "cyst adenoma". [5] In 1989, Joshi and Beckwith proposed a modified terminology and redefined the diagnostic criteria to differentiate cystic nephroma from CPDN and other cystic renal tumors such as Wilms' tumor with cyst formation. [6] Differential diagnosis for cystic nephroma includes CPDN, unilateral polycystic disease of kidney, cystic variant of mesoblastic nephroma, clear cell sarcoma and cystic degeneration in renal cell carcinoma and Wilms' tumor. [1],[2],[3]

There is a biphasic age and sex distribution. Early peak is between three months and two years and the later peak is after third decade, mostly in fourth and fifth decades. In the pediatric populations, male predominance exists (M:F = 2:1), and in the adults, females are more prone (F:M = 8:1). Cystic nephroma in adults is exceptional before the age of 30.

In children, the presenting symptom is mostly a painless enlarging flank mass detected by parents or physician. Meanwhile, in adults, vague flank pain, hematuria, urinary tract infection and rarely hypertension may be the presenting features. [7] To the best of our knowledge, association of hypertension with cystic nephroma in children has not been reported earlier. But our patient had hypertension which needed medication with nifedipine. The pathogenesis of hypertension is unclear. The compression of intra-renal vessels or main renal vessels by massive tumor may be the factor responsible. Once the tumor was resected, the hypertension disappeared in the immediate postoperative period and she is not on any antihypertensive medication now. Other renal tumors associated with hypertension are Wilm's tumor and rarely, renal cell carcinoma.

It is important to differentiate the cystic nephroma from other tumors that are malignant, as management strategies may change. Radiologically, there will be no solid elements in cysts and the septae partially enhance with contrast. [8] It is not possible to differentiate from CPDN radiologically. Histologically, blastemal elements in the septae characterize the CPDN. [7] Association of hypertension with CPDN is unknown. Management of multicystic nephroma is by complete surgical resection. In appropriate situation, partial nephrectomy may be done in cystic nephromas. However, incomplete resection may lead to recurrence. [9] Malignant transformation has not been reported.

 
   References Top

1.Hartman DS, Davis CJ, Sanders RC, Johns TT, Smirniotopoulos J, Goldman SM. The multi-loculated renal mass: considerations and differential features. RadioGraphics 1987;7:29-52.  Back to cited text no. 1
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2.Slywotzky CM, Bosniak MA. Localized cystic disease of the kidney. AJR Am J Roentgenol 2001;176:843-9.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Lowe RE, Cohen MD. Computed tomographic evaluation of Wilms tumour and neuroblastoma. RadioGraphics 1984;4:915-25.  Back to cited text no. 3
    
4.Flynn JT. Evaluation and management of hypertension in childhood. Prog Pediatr Cardiol 2001;12:177-88.  Back to cited text no. 4
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5.Edmunds W. Cystic adenoma of the kidney. Trans Pathol Soc London 1892;43:89-90.  Back to cited text no. 5
    
6.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.  Back to cited text no. 6
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7.John KH, Henry WG, Josephine WA, Steven AB. Cystic nephroma. Radiographics 2004;24: 583-94.  Back to cited text no. 7
    
8.Dalla-Palma L, Pozzi-Mucelli F, di Donna A, Pozzi-Mucelli R. Cystic renal tumors: US and CT findings. Urol Radiol 1990;12:67-73.  Back to cited text no. 8
    
9.Stuart W, Atreya D. Failure of Percutaneous Endoscopic Resection of a Renal Cystic Nephroma on Longer-Term Follow-Up. J Endourol 2008;22:1505-8.  Back to cited text no. 9
    

Top
Correspondence Address:
Satheesan Balasubramanian
Associate Professor, Division of Uro-Oncology, Department of Surgical Oncology, Cancer Institute (W.I.A.), Sardar Patel Road, Adyar, Chennai, Tamil Nadu
India
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PMID: 21743228

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    Abstract
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