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Year : 2010 | Volume
: 21
| Issue : 4 | Page : 728-731 |
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Intrarenal epidermoid cyst presented as an enlarged multicystic kidney |
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Asmaa Gaber Abdou, Nancy Youssef Asaad
Department of Pathology, Faculty of Medicine, Menofiya University, Shebein Elkom, Egypt
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Date of Web Publication | 26-Jun-2010 |
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Abstract | | |
This report describes a case of large multiple and multilocular epidermoid cysts affecting the left kidney of a 67 old male. The condition was accidentally discovered during the ultrasonography for left loin pain and revealed enlarged left kidney with picture suggestive of hydronephrosis. Grossly, the kidney was distorted cysts that were filled with a cheesy material. The histologic picture of an end stage kidney disease was apparent in the compressed renal parenchyma by these cysts that were identical to an epidermoid cyst elsewhere. In conclusion, although rare, epidermoid cyst of the kidney could cause serious kidney damage leading to an end stage kidney disease. Epidermoid cyst of the kidney could be multiple producing a picture similar to hydronephrosis andbroadening the differential diagnosis of multicystic disease of the kidney.
How to cite this article: Abdou AG, Asaad NY. Intrarenal epidermoid cyst presented as an enlarged multicystic kidney. Saudi J Kidney Dis Transpl 2010;21:728-31 |
How to cite this URL: Abdou AG, Asaad NY. Intrarenal epidermoid cyst presented as an enlarged multicystic kidney. Saudi J Kidney Dis Transpl [serial online] 2010 [cited 2021 Apr 18];21:728-31. Available from: https://www.sjkdt.org/text.asp?2010/21/4/728/64661 |
Introduction | |  |
Epidermal inclusion cyst refers to those cysts that arise from the implantation of epidermal elements in the dermis. However, many cysts originate from the infundibular portion of the hair follicle, and the more general term, epidermoid cyst, is favored. The typical epidermoid cyst is lined with stratified squamous epithelium that contains a granular layer and is filled with keratinous material that is often in a laminated arrangement. Intrarenal epidermoid cyst is a very rare condition with few reported cases in the English literature [1],[2],[3],[4] [Table 1]. It is also reported to arise in the renal pelvis and upper ureter with only four cases mentioned in the literature. [5],[6],[7],[8]
In this report we present an accidentally discovered case of extensive left kidney destruction replaced by boggy multilocular cysts pathologically proved to be an epidermoid cyst.
Case Report | |  |
A 67-year-old male patient suffered from left loin pain after previous blunt trauma. No previous history of upper or lower urinary tract stones. Ultrasonography revealed markedly enlarged hydronephrotic left kidney with evidence of rupture that necessitated left nephrectomy. Gross examination revealed that the kidney was enlarged, distorted and measured 20 Χ 15 Χ 10 cm. Its cut section showed replacement of kidney tissue by multiple pockets & cavities filled with firm cheesy material and absence of grossly identified stones [Figure 1]. Microscopically, the renal parenchyma was compressed by these multiple and multilocular cysts that were lined by keratinized stratified squamous epithelium with granular cell layer and filled with laminated layers of keratin [Figure 2] and [Figure 3]. No evidence of skin appendages either pilosebaceous units or eccrine sweat glands were noticed. The surrounding renal parenchyma showed signs of atrophy in the form of decreased number of glomeruli that appeared mostly sclerosed and hyalinized. In addition to the atrophy of the tubules that appear small in size and filled with colloid like casts (tubular thyroidization) [Figure 4]. This atrophic parenchyma was either seen directly beneath the cyst wall or separated from it by areas of fat or fibrosis. Considerable foreign body granulomatous reaction was also recognized. The pelviureteric junction was lined by normal transitional urothelium without any evidence of squamous metaplasia.
Discussion | |  |
In our patient, the left kidney was studded by multiple cysts replacing and compressing the whole renal parenchyma that was diagnosed as hydronephrosis by ultrasound. The definitive diagnosis was reached by the microscopic identification of the skin layered filled cysts that lacked skin appendages. On the other hand, in the previous described cases, the intrarenal epidermoid cyst was single and affecting one pole of the kidney that was suspected to be a renal neoplasm preoperatively. [1],[2],[3],[4] In our case as well as the previous reported ones, the diagnosis could not be made preoperatively and not even suspected.
The presence of stratified squamous epithelium in renal lesions is considered as a rare condition which may have different causes. For example, extension of squamous metaplastic changes from the upper ureter to the pelvicalyceal system in prolonged obstructive conditions induced mostly by stones is considered as one of the commonest source of squamous epithelium in the kidney. [9] This metaplastic epithelium may be a precursor for the very rare primary renal squamous cell carcinoma. [10] Squamous epithelium could also be a part of other renal lesions such as teratoid variant of Wilm's tumor, [11] teratoma [12] and dermoid cyst. [13]
In the current study, the absence of renal calculi and metaplastic changes in the pelviureteric junction could differentiate our case from the squamous metaplasia of the pelvicalyceal system due to the obstructive and irritative effects of stones. Furthermore, the confinement of this cystic structure to the epidermal lineage with the absence of other lineages of differentiation together with the lack of other cutaneous adenexal structures helped in differentiating from teratoma and dermoid cyst.
Different theories had been suggested for the presence of epidermoid cyst in extraordinary sites such as internal solid organs including kidney, spleen, brain and testis. If the implantation, sequestration or inclusion could be suitable explanations for epidermoid cyst arising in hairy and in non hairy areas, [4] it would not be acceptable in internal organs except after exclusion of trauma. In kidney, it is suggested that this type of cyst could originate from the embryonic remnant of Wolffian ducts and this hypothesis is considered as the most convenient one. [4] Inclusion of surface mesothelium in splenic parenchyma could be the source of epidermoid cyst in the spleen. [14] (Epidermoid cyst could arise in brain or cavernous sinus from subpopulation of neural crest cells that remain entrapped within the meninges in uncomplicated stage of maturation. [15] Epidermoid cyst could also develop in testis as an epidermal differentiation of monodermal teratoma referring to its germ cell origin. [16] Squamous metaplasia of the rete testis could be another explanatory pathway for its development in testis. [16]
In conclusion, epidermoid cyst of the kidney is a rare condition causing severe kidney damage leading to an end stage kidney disease. Epidermoid cyst of the kidney could be multiple producing a picture similar to hydronephrosis. Awareness of the occurrence of this cyst would broaden the differential diagnosis in unilateral cystic diseases of the kidney.
References | |  |
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2. | Duprat G, Filiatrault D, Michaud J. Intrarenal epidermoid cyst. Pediatr Radiol 1986;16:73-5. |
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4. | Lim SC, Kim CS. Intrarenal epidermal cyst. Pathol Int 2003;53:574-8. [PUBMED] [FULLTEXT] |
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9. | Boswell PD, Fugitt B, Kane CJ. Keratinizing desquamative squamous metaplasia of the kidney mimicking transitional cell carcinoma. Urology 1998;52:512-3. [PUBMED] [FULLTEXT] |
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11. | Inoue M, Uchida K, Kohei O, et al. Teratoid Wilms' tumor: a case report with literature review. J Pediatr Surg 2006;41:1759-63. [PUBMED] [FULLTEXT] |
12. | Mochizuki K, Ohno Y, Tokai Y, et al. Congenital intrarenal teratoma arising from a horseshoe kidney. J Pediatr Surg 2006;41:1313-5. [PUBMED] [FULLTEXT] |
13. | Otani M, Tsujimoto S, Miura M, Nagashima Y. Intrarenal mature cystic teratoma associated with renal dysplasia: case report and literature review. Pathol Int 2001;51:560-4. [PUBMED] [FULLTEXT] |
14. | Maskey P, Rupakheti S, Regmi R, Adhikary S, Agrawal CS. Splenic epidermoid cyst. Kathmandu Univ Med J 2007;18:250-2. |
15. | Tatagiba M, Iaconetta, Samii M. Epidermoid cyst of the cavernous sinus: clinical features, pathogenesis and treatment. Br J Neurosurg 2000;14:571-5. |
16. | Loya AG, Said JW, Grant EG. Epidermoid cyst of the testis: radiologic-pathologic correlation. Radiographics 2004;24(Suppl 1):S243-6. [PUBMED] [FULLTEXT] |

Correspondence Address: Asmaa Gaber Abdou Department of Pathology, Faculty of Medicine, Menofiya University, Shebein Elkom Egypt
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PMID: 20587881 
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1] |
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This article has been cited by | 1 |
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| Go, J.H. and Hong, J.H. and Lee, G. | | International Neurourology Journal. 2012; 16(4): 205-208 | | [Pubmed] | | 2 |
Epidermoid cyst in the kidney |
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| Desai, S. and Thakur, S. and Menon, S. and Desai, S.B. | | Urology. 2011; 78(3): 563-564 | | [Pubmed] | |
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