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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 3  |  Page : 400-402
Renal mucinous tubular and spindle cell carcinoma


Department of Pathology, SGPGIMS, Lucknow, India

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Date of Web Publication12-Aug-2009
 

   Abstract 

Mucinous tubular and spindle cell carcinoma is a rare variant of renal cell carcinoma, which has recently been described. It has a low malignant potential and is usually confined to the kidney. These are thought to be of the loop of Henle or distal nephron origin. We report a 65-year-old male who presented with flank pain, hematuria and a well-defined renal mass that was diagnosed as mucinous tubular and spindle cell tumor.

Keywords: Mucinous, renal tumor, variant

How to cite this article:
Kumari N, Chhabra P, Dewan U, Jain M. Renal mucinous tubular and spindle cell carcinoma. Indian J Pathol Microbiol 2009;52:400-2

How to cite this URL:
Kumari N, Chhabra P, Dewan U, Jain M. Renal mucinous tubular and spindle cell carcinoma. Indian J Pathol Microbiol [serial online] 2009 [cited 2019 Oct 24];52:400-2. Available from: http://www.ijpmonline.org/text.asp?2009/52/3/400/55007



   Introduction Top


In the last few years, many new variants of renal cell tumors have been described. There have been great advances in molecular genetics, cytogenetics and characterization of various morphological features as a result of which newer categories of renal cell tumors are being described. Still, approximately 6-7% of renal tumors remain unclassified. [1],[2] The mucinous tubular and spindle cell carcinoma variant of renal cell carcinoma is a newly diagnosed entity with low grade and low stage, which is thought to be of the loop of Henle or distal nephron origin. There are just a couple of case reports about the morphological and immunohistochemical profiles of this entity, which shows that there is considerable overlap between papillary renal cell carcinoma and mucinous tubular and spindle cell carcinoma of the kidney. [3],[4] We describe a rare case report of a 65-year-old male diagnosed as mucinous tubular and spindle cell carcinoma of kidney for the first time in our institute.


   Case Report Top


The patient was an elderly male who had a complaint of mild to moderate right flank pain for 1 year duration. He also had one episode of hematuria 1.5 years back. There was no history of fever, jaundice, tuberculosis, hypertension and diabetes. The patient's general physical examination and routine biochemical and hematological investigations were within the normal range. Ultrasonography showed a hypovascular right renal mass measuring 4.5 cm 4.5 cm at the upper pole. The left kidney, urinary bladder and prostate were unremarkable. Contrast enhanced computed tomography (CECT) confirmed the mass to be located in the right upper and middle portion of the right kidney.

Right laparoscopic transperitoneal radical nephrectomy was performed, including removal of the adrenal gland. On cut surface, the tumor measured 6 cm 6 cm, was a well-circumscribed solid, grayish white in appearance, causing compression of the renal pelvicalyceal system. The adjacent renal parenchyma was grossly unremarkable. There was no invasion in the surrounding perinephric fat or Gerota's fascia or in the renal vein in the hilum. Microscopically, the tumor was well circumscribed and revealed the epithelial component in the form of foci of round to compressed tubules and cords lined by cuboidal cells with no nuclear atypia [Figure 1]. The cuboidal epithelial cells had basally located mildly anisomorphic nuclei, inconspicuous nucleoli and a moderate amount of cytoplasm [Figure 2]. These epithelial elements were separated by short fascicles of spindle cells, suggesting a mesenchymal component. The tumor was rich in inflammatory cell infiltrate composed of plasma cells and lymphocytes. Mucinous change was not evident easily on hematoxylin and eosin stain; however, mucin stain (Alcian blue) highlighted small foci of mucinous change. No mitosis or necrosis was evident. Immunohistochemistry showed positivity for EMA, CK (pancytokeratin) and CK7 in the tubular epithelial component and vimentin in the spindle cell component. Vimentin was also weakly seen in the epithelial component. Desmin, SMA, CEA, S-100, CD15 and CD34 were negative.


   Discussion Top


Mucinous tubular and spindle cell tumor of the kidney is a recently described entity that has a less aggressive behavior in spite of having a spindle cell component. Till date, there are approximately more than 50 cases reported in the literature with few series of cases. [5] Spindle cell or sarcomatoid differentiation in an otherwise conventional renal cell carcinoma carries a much more aggressive behavior. The renal mucinous tubular and spindle cell tumor is more common in females with a wide age range of 17-82 years (average, 53 years), with a male:female ratio of 1:4.[6] These tumors are grossly well circumscribed and are usually located in the medulla. Our case also had well-circumscribed growth on gross and was located in the upper pole and mid region near the medulla. Microscopically, these tumors may contain interconnected tubules, many appearing straight and with slit-like lumina, solid compressed cord-like structures and prominent low-grade spindle cell areas. No papillae or cysts are present. Myxoid change is seen at least focally, which can be brought out well by mucin stains, and is usually accompanied by some inflammatory cell infiltrates. Ultrastructural examination of some of these tumors has shown resemblance to the loop of Henle. [4] The main differential diagnosis of this tumor is lesions that contain spindle cell components like sarcomatoid renal cell carcinoma, type I papillary renal cell carcinoma with spindle cell areas, collecting duct carcinoma and, sometimes, metanephric adenoma. Sarcomatoid renal cell carcinoma displays considerable nuclear pleomorphism and mitotic activity, which is absent in the mucinous tubular and spindle cell carcinomas. Collecting duct carcinoma also shows nuclear pleomorphism in the tubular component, whereas they are of low grade in the former. Metanephric adenoma shows cells having less cytoplasm and appears as a cellular blue tumor on microscopy. They are never diffusely reactive for CK7 and EMA is negative. [1],[7] Our case also had a well-circumscribed tumor composed of round to compressed tubules and cords showing low-grade nuclear morphology. No true papillae with a fibrovascular core were seen and the tumor was rich in inflammatory cell infiltrate composed of plasma cells and lymphocytes. Immunohistochemically, our case showed positivity for CK7 and EMA, which is in contrast to metanephric adenoma. The positivity for CK7 and EMA in mucinous tubular and spindle cell tumor increases the complexity further to differentiate this tumor from renal papillary cell carcinoma. In such cases, demonstration of gains or losses of chromosome 7, 17 and Y would be helpful. [8] These tumors have been shown to behave as low grade without any recurrence and metastasis has been reported in two cases till now in the literature. [5] Our case was asymptomatic after surgery; however, the follow-up till the time of report of the case was available for 12 months only.


   Conclusion Top


Mucinous tubular and spindle cell carcinoma of the kidney is a rare low-grade renal tumor having overlap of morphological and immunohistochemical features with other renal cell neoplasms. Immunohistochemical and cytogenetic studies would be helpful in the diagnosis of this neoplasm because it has to be differentiated from other high-grade renal neoplasms. We report this case for its rarity.

 
   References Top

1.Parwani AV, Husain AN, Epstein JI, Beckwith JB, Argani P. Low-grade myxoid renal epithelial neoplasms with distal nephron differentiation. Hum Pathol 2001;32:506-12.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Reuter VE, Presti JC. Contemporary approach to the classification of renal epithelial tumors. Semin Oncol. 2000;27:124-37.  Back to cited text no. 2    
3.Srigley J, Eble JN, Grignon D, RWJ H. Unusual renal cell carcinoma with prominent spindle cell change possibly related to the loop of Henle. Mod Pathol 1999;12:107A.  Back to cited text no. 3    
4.Srigley JR, Kapusta L, Reuter V, Amin M, Grignon DG, Eble JN, et al . Phenotypic, Molecular and Ultrastructural Studies of a Novel Low Grade Renal Epithelial Neoplasm possibly related to the Loop of Henle. Mod Pathol 2002;12:182A.  Back to cited text no. 4    
5.Paner GP, Srigley JR, Radhakrishnan A, Cohen C, Skinnider BF, Tickoo SK, et al . Immunohistochemical Analysis of Mucinous Tubular and Spindle cell Carcinoma and Papillary Renal Cell Carcinoma of the Kidney. Significant Immunophenotypic Overlap Warrants Diagnostic Caution. Am J Surg Pathol 2006;30:13-9.  Back to cited text no. 5    
6.Jung SJ, Yoon HK, Chung JI, Ayala AG, Ro JY. Mucinous tubular and spindle cell carcinoma of the kidney with neuroendocrine differentiation: Report of two cases. Am J Clin Pathol 2006;125:99-104.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Pins MR, Renshaw AA, Jones EC. Metanephric adenoma and solid tubulopapillary renal cell carcinoma: A comparative immunohistochemical study. Mod Pathol 1999;12:104A.  Back to cited text no. 7    
8.Cossu-Rocca P, Eble JN, Delahunt B, Zhang S, Martignoni G, Brunelli M, et al . Renal Mucinous Tubular and Spindle Cell Carcinoma Lacks the Gains of Chromosomes 7 and 17 and Losses of Chromosomes Y that are Prevalent in Papillary Renal Cell Carcinoma. Mod Pathol 2006;19:488- 93.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]

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Correspondence Address:
Manoj Jain
Department of Pathology, SGPGIMS, Lucknow - 226 014
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.55007

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    Figures

  [Figure 1], [Figure 2]

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    Abstract
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    Case Report
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