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Journal of Indian Association of Pediatric Surgeons
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Table of Contents   
CASE REPORT
Year : 2020  |  Volume : 25  |  Issue : 6  |  Page : 408-410
 

Renal pelvic cholesteatoma: An uncommon finding in children


1 Department of Pediatric Surgery, Chacha Nehru Bal Chikitsalaya, Delhi, India
2 Department of Pathology, Chacha Nehru Bal Chikitsalaya, Delhi, India

Date of Submission18-Jan-2020
Date of Decision08-May-2020
Date of Acceptance21-Jul-2020
Date of Web Publication27-Oct-2020

Correspondence Address:
Dr. Chhabi Ranu Gupta
Department of Pediatric Surgery, Chacha Nehru Bal Chikitsalaya, Geeta Colony, Delhi - 110 031
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.JIAPS_20_20

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   Abstract 


Renal cholesteatoma or keratinizing desquamative squamous metaplasia is infrequent in adults and rare in children. We report a case of renal cholesteatoma in a 4-year-old male child who was referred to us as a case of multiple renal calculi with hydronephrosis. We also discuss his management with a review of relevant literature.


Keywords: Cholesteatoma, keratinizing desquamative squamous metaplasia, renal, pediatric


How to cite this article:
Gupta CR, Rao SP, Mahajan N, Singh L. Renal pelvic cholesteatoma: An uncommon finding in children. J Indian Assoc Pediatr Surg 2020;25:408-10

How to cite this URL:
Gupta CR, Rao SP, Mahajan N, Singh L. Renal pelvic cholesteatoma: An uncommon finding in children. J Indian Assoc Pediatr Surg [serial online] 2020 [cited 2020 Nov 28];25:408-10. Available from: https://www.jiaps.com/text.asp?2020/25/6/408/299199





   Introduction Top


Cholesteatoma of the upper urinary tract is an uncommon benign condition. Flank pain, the passage of cornified material in the urine, and a filling defect on intravenous pyelography (IVP) constitute the characteristic triad. Earlier, most cases were managed by extensive surgery due to its presumed association with squamous cell carcinoma but now a conservative approach is being followed.. We report a case of renal pelvic cholesteatoma that was referred to us as a case with multiple renal calculi. This is an uncommon condition in children with very few cases reported in literature till date.


   Case Report Top


Our patient, a 4-year-old male, presented with flank pain and pain during passing urine. The patient had these complaints for the past 6 months and was referred to our hospital after an ultrasound report suggested gross right hydronephrosis with multiple calculi in dilated renal pelvis and calyces. There was no history of hematuria. Physical examination was unremarkable. Laboratory data revealed a normal complete blood count, blood urea, creatinine, electrolytes, calcium, and phosphorus. Urine culture revealed no growth and routine urine examination was also insignificant. Intravenous urography showed multiple filling defects in the right renal pelvis [Figure 1]. The left kidney was normal. A Tc 99m DTPA renogram revealed a compromised cortical function of the right kidney with obstruction to outflow at the level of pelviureteric junction (PUJ). The patient was then planned for surgery with a preoperative diagnosis of multiple calculi in the right renal pelvis obstructing at the PUJ. Intraoperatively, a soft growth was encountered at the PUJ and on opening the renal pelvis, soft pultaceous material extruded out [Figure 2]. It was sent for histopathological examination and the surgery was concluded with a right dismembered pyeloplasty over a 3.5 Fr DJ stent. The postoperative course was uneventful. The patient was discharged with advice to return after 6 weeks for DJ stent removal. Histopathological examination showed an inflamed renal pelvis lined by hyperplastic urothelium. The subepithelium showed extensive edema and moderate-to-dense lymphoplasmacytic infiltrate. The cheesy material sent for biopsy showed abundant acellular proteinaceous material along with keratin flakes which were suggestive of cholesteatoma [Figure 3].
Figure 1: Excretory urography showing filling defects in dilated right pelvicalyceal system

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Figure 2: Extracted pultaceous material from the renal pelvis and calyces

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Figure 3: (a) Photomicrograph showing anucleated squames and keratinous debris (H and E, ×100). (b) Photomicrograph showing keratin debris, fibrin, abundant calcification, minimal inflammation, and focal foreign-body giant cell

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   Discussion Top


Cholesteatoma most commonly occurs in the ear. There are few case reports of upper urinary tract cholesteatomas or keratinizing desquamative squamous metaplasia (KDSM) in adults, but we could find only two case reports of renal cholesteatoma in children in English literature.[1],[2]

Histologically, there is “squamous metaplasia of transitional epithelium, keratinization and subsequent desquamation of the keratinized layers. Inflammatory infiltration, parakeratosis, rete-peg formation, and atrophy or dysplasia of the renal parenchyma may also be seen.” The basal layer of the squamous epithelium KDSM is always normal, emphasizing its benign nature.[3] No difference has been found in the histological picture of acquired cholesteatoma in adult and children.[4]

Renal KDSM or cholesteatomatous leukoplakia is usually located in the renal pelvis and calyces. It is more common in adults (97.5% of described cases), with a male-to-female ratio being 3:2.[5] This condition is thought to arise due to chronic urothelial inflammation, though hypotheses of embryological anomaly or even spontaneous transformation of urothelium into squamous epithelium have also been suggested.[3],[6]

Diagnosing this condition is difficult. The clinical and radiological picture can mimic renal stones, renal mass, blood clot, tuberculosis, or papillary necrosis; all of which are more common than KDSM. Elimination of flake-like keratinized material in the urine is considered to be the most characteristic sign.[3] However, this was not present in our case may be due to associated PUJ obstruction. The degree of calcification of the lesion is variable. Cases with minimal calcification may not be detectable on plain X-ray, while those having a significant amount of calcification may appear like calculi. Special studies such as excretory urography and retrograde pyelography, and computed tomography urography can be helpful, but a urothelial tumor must always be included in the differential diagnosis.[3] In our case, the plain X-ray was inconclusive, but excretory urography showed filling defects in the renal pelvicalyceal area and obstruction at the PUJ. DTPA renogram showed decreased cortical function with obstruction at the PUJ. This condition may be associated with renal stones.[7],[8]

In literature, there are reports of nephrectomy being done in cases with renal KDSM due to a mistaken diagnosis of malignancy.[2],[9] Despite some suggestions of its possible progression to squamous carcinoma, there have been no reports of progression to malignancy. Hence, more conservative approaches have been used in recent times. Managing these cases via the percutaneous and transureteroscopic route, or even clinical follow-up, has been described.[10],[11] Some authors have suggested Vitamin A supplementation as a curative measure in selected cases of auditory cholesteatomas.[12] This may be extrapolated to cases with urinary tract involvement. Removal of cholesteatoma and pyeloplasty was done in our case. The child is doing well at 2-year follow-up without any recurrence.


   Conclusion Top


Renal cholesteatoma is an uncommon benign condition. It should be kept in mind when dealing with suspicious renal pelvic masses or when a patient presents with complaints of passing white flakes in the urine. Minimally invasive interventions or clinical follow-up should be advised as per the patient's symptoms after confirmation of diagnosis. Vitamin A supplementation can be tried for treatment and preventing recurrence. Patients should be followed up for possible recurrence and malignancy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Ross RR Jr., Lewis HY, Brough AJ. Renal cholesteatoma in a child. J Urol 1970;104:184-8.  Back to cited text no. 1
    
2.
Jain P, Mishra A, Misra DS. Keratinizing squamous metaplasia of the upper urinary tract in a child with a solitary kidney. Indian J Urol 2014;30:230-2.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Hertle L, Androulakakis P. Keratinizing desquamative squamous metaplasia of the upper urinary tract: Leukoplakia-cholesteatoma. J Urol 1982;127:631-5.  Back to cited text no. 3
    
4.
Dornelles C, Meurer L, Selaimen da Costa S, Schweiger C. Histologic description of acquired cholesteatomas: Comparison between children and adults. Braz J Otorhinolaryngol 2006;72:641-8.  Back to cited text no. 4
    
5.
Lima DX, Rabelo EA, Salles PG. Cholesteatoma of the upper urinary tract. Int Braz J Urol 2004;30:494-5.  Back to cited text no. 5
    
6.
Weiner DP, Koss DP, Sablay LG. The prevalence and significanceof Brunn's nestscystis cystic and squamous metaplasia in normal bladders. J Urol 1979;122:317.  Back to cited text no. 6
    
7.
Sarlis JN, Malakates SK, Papadimitriou D. Cholesteatoma of the renal pelvis. J Urol 1977;118:468-9.  Back to cited text no. 7
    
8.
Sharma S, Pai K, Valiathan M, Phadnis P, Chawla A. Renal cholesteatoma: A case report of keratinising desquamative squamous metaplasia of the upper urinary tract. Int J Health Sci Res 2016;6:419-23.  Back to cited text no. 8
    
9.
Boswell PD, Fugitt B, Kane CJ. Keratinizing desquamative squamous metaplasia of the kidney mimicking transitional cell carcinoma. Urology 1998;52:512-3.  Back to cited text no. 9
    
10.
Houston W. Renal cholesteatoma. Br J Urol 1983;55:239-40.  Back to cited text no. 10
    
11.
Neerhut G, Politis G, Alpert L, Griffith DP. Cholesteatoma of the renal pelvis: Endoscopic management. J Urol 1988;139:1032-4.  Back to cited text no. 11
    
12.
Vishal Rao US, Srinivas DR, Humbarwadi RS, Malhotra BK. Role of Vitamin A in the evolution of cholesteatoma. Indian J Otolaryngol Head Neck Surg 2009;61:150-2.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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