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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 4  |  Page : 536-537
Mucinous adenocarcinoma of the renal pelvis with pseudomyxoma peritonei


Department of Pathology, T.N. Medical College and B.Y.L. Nair Hospital, Dr. A.L. Nair Road, Bombay Central, Mumbai 400 008, India

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   Abstract 

Mucinous adenocarcinoma of the renal pelvis is an extremely rare tumor with very few case reports in literature. Pseudomyxoma peritonei is an uncommon condition characterized by the presence of mucinous gelatinous material in the peritoneal cavity. It occurs secondary to primary mucinous neoplasms of particularly the appendix and the ovary. We present a case of a 35-year-old female who had a history of dull aching pain in the right flank since one and a half years. Upon ultrasonography (USG) and computerized tomography (CT) scan, there was a large cystic mass measuring 15x15x12 cm, extending into the right lumbar region. Grossly, the entire kidney was converted into a cystic mass measuring 15x15x12 cm containing gelatinous mucinous material weighing 1 kg. Histologically, the tumor was composed of simple and complex glandular acini together with a superficial resemblance to colonic mucosa with abundant extracellular mucin. Thus, a diagnosis of mucinous adenocarcinoma of the renal pelvis leading to pseudomyxoma peritonei was made.

Keywords: Mucinous adenocarcinoma, pseudomyxoma peritonei, renal pelvis

How to cite this article:
Shah VB, Amonkar GP, Deshpande JR, Bhalekar H. Mucinous adenocarcinoma of the renal pelvis with pseudomyxoma peritonei. Indian J Pathol Microbiol 2008;51:536-7

How to cite this URL:
Shah VB, Amonkar GP, Deshpande JR, Bhalekar H. Mucinous adenocarcinoma of the renal pelvis with pseudomyxoma peritonei. Indian J Pathol Microbiol [serial online] 2008 [cited 2014 Oct 31];51:536-7. Available from: http://www.ijpmonline.org/text.asp?2008/51/4/536/43753



   Introduction Top


Mucinous adenocarcinoma of the renal pelvis is an extremely rare tumor with very few case reports in literature. [1],[2],[3],[4],[5] Pseudomyxoma peritonei is also an uncommon condition. It is characterized by the presence of mucinous gelatinous material in the peritoneal cavity. Primary mucinous neoplasms, especially of the appendix and the ovary, are the known antecedents of pseudomyxoma peritonei. [6],[7] We present an extremely rare case of mucinous adenocarcinoma of the renal pelvis in which surgical excision of the primary tumor was followed by pseudomyxoma peritonei at a later date. To the best of our knowledge, such a combined occurrence has not yet been reported in literature.


   Case Report Top


A 35-year-old female presented with a history of a dull aching pain in the right flank since one and a half years. There was a history of right-sided pyelolithotomy 10 years prior. Upon ultrasonography (USG) and computerized tomography (CT) scan, there was a large cystic mass measuring 15x15x12 cms, extending into the right lumbar region. The right kidney could not be visualized separately but both the ovaries and the appendix were normal on USG and CT scan. Surgical excision of the mass was performed. Grossly, the entire kidney was converted into a cystic mass measuring 15x15x12 cm containing gelatinous mucinous material weighing 1 kg [Figure 1]. Histologically, the tumor was composed of simple and complex glandular acini together with a superficial resemblance to colonic mucosa with abundant extracellular mucin [Figure 2]. The multiple sections taken for analysis revealed small foci of renal parenchyma showing atrophy with chronic pyelonephritis and granular calculous material. Considering the histomorphological features, a diagnosis of mucinous adenocarcinoma of the renal pelvis was made. Four months later, the patient developed massive peritoneal deposits and a biopsy of these lesions showed similar tumor cells with abundant mucin. Upon USG and CT scan both the ovaries and the appendix were normal. A diagnosis of mucinous adenocarcinoma of the renal pelvis leading to pseudomyxoma peritonei was reported.


   Discussion Top


Mucinous adenocarcinoma of the renal pelvis is an extremely rare entity. [1],[2],[3],[4],[5] Tumors of the renal pelvis are themselves uncommon with transitional cell carcinoma being the most frequently occurring type. Only approximately 100 cases of mucinous adenocarcinoma of the renal pelvis have been reported. These tumors occur as a result of metaplasia of the transitional epithelium of the calyces and pelvis into glandular epithelium, which then undergoes a malignant transformation. [1],[2],[3],[4],[5] There are also reports of this tumor occurring simultaneously with transitional renal cell carcinoma of the renal pelvis and bladder. [3],[4] Presence of chronic infection and calculi are commonly associated with these tumors. [1],[2],[3],[4],[5] In our case, the renal parenchyma showed chronic pyelonephritis and granular calculous material. Grossly, abundant mucin is seen in most cases as was evident in our case. However, sometimes they may present as a small occult tumor in a case of renal calculi. Hence, it is very important to look carefully at the pelvic mucosa of the kidney in cases of calculi where mucin is identified.

Pseudomyxoma peritonei is a rare clinical syndrome characterized by intraperitoneal accumulation of mucin produced by neoplastic tumors especially of the appendix and the ovaries. [6],[7] Pseudomyxoma peritonei in women may be ovarian or appendicial in origin with a majority of the cases being related to the appendix. When both the ovaries and the appendix are involved, the appendicial tumor is probably primary and the ovarian tumor secondary. [6] However, mucinous tumors of other organs like the intestine and pancreas have been implicated in the development of pseudomyxoma peritonei. [8],[9] Mucinous adenocarcinoma of the renal pelvis leading to pseudomyxoma peritonei has not been documented in literature and hence we have presented this case to highlight this rare occurrence.

 
   References Top

1.Aufderheide AC, Streitz JM. Mucinous adenocarcinoma of the renal pelvis. Cancer 1974;33:167-73.  Back to cited text no. 1  [PUBMED]  
2.Kakkar N, Joshi K, Mandal AK. Mucinous adenocarcinoma of the renal pelvis: A case report. Indian J Pathol Microbiol 1997;40:393-5.  Back to cited text no. 2    
3.Takehara K, Nomata K, Eguchi J, Hisamatsu H, Maruta S, Hayashi T, et al. Mucinous adenocarcinoma of the renal pelvis associated with transitional cell carcinoma in the renal pelvis and the bladder. Int J Urol 2004;11:1016-8.  Back to cited text no. 3    
4.Stein A, Sova Y, Lurci M, Lurci A. Adenocarcinoma of the renal pelvis: A report of two cases, one with simultaneous transitional carcinoma of the bladder. Urol Int 1988;43:299-301.  Back to cited text no. 4    
5.Terris MK, Anderson RU. Mucinous adenocarcinoma of the renal pelvis in natives of India. Urol Int 1997;58:121-3.  Back to cited text no. 5  [PUBMED]  
6.Young RH, Gilks CB, Scully RE. Mucinous tumours of the appendix associated with mucinous tumours of the ovary and pseudomyxoma peritonei: A clinicopathological analysis of 22 cases supporting an origin in the appendix. J Surg Pathol 1991;15:415-29.  Back to cited text no. 6    
7.Prayson RA, Hart WK, Petras RE. Pseudomyxoma peritonei: Clinicopathological study of 19 cases with emphasis on site of origin and nature of associated ovarian tumours. Am J Surg Pathol 1994;18:591-603.  Back to cited text no. 7    
8.Mizuta Y, Akazawa Y, Shiozawa K, Ohara H, Ohba K, Ohnita K, et al. Pseudomyxoma peritonei accompanied by intraductal papillary mucinous neoplasm of the pancreas. Pancreatology 2005;5:470-4.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Varona JF, Guerra JM, Salamanca J, Colina F, Lopez G, Morales M. Pseudomyxoma peritonei: A clinicopathologic analysis and follow up of 21 patients. Hepatogastroenterology 2005;52:812-6.  Back to cited text no. 9  [PUBMED]  

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Correspondence Address:
Gayathri P Amonkar
P, 778, Homi Villa, Parsi Colony, Tilak Road, Dadar (E), Mumbai 400 014, Maharashtra
India
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DOI: 10.4103/0377-4929.43753

PMID: 19008588

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    Figures

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