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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 4  |  Page : 514-516
Mucinous tubular and spindle cell carcinoma of kidney: A rare case report and review of the literature


Department of Pathology and Transplant Research Center, and Urology, Shiraz University of Medical Sciences, Shiraz, Iran

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Date of Web Publication1-Oct-2009
 

   Abstract 

Low grade mucinous tubular and spindle cell carcinoma of kidney was newly established as a distinct renal cell carcinoma in the World Health Organization (WHO) classification of 2004. Until now, less than 60 cases have been reported and the largest series represented approximately 15 patients with this type of tumor. Herein, we report a case of mucinous tubular and spindle cell carcinoma in a 63-year-old male presented with right flank pain which was diagnosed after nephrectomy. Pathologists should consider this diagnosis and its spectrum of histopathologic features in mind to ensure an accurate diagnosis.

Keywords: Mucinous tubular and spindle cell carcinoma, kidney

How to cite this article:
Geramizadeh B, Salehipour M, Moradi A. Mucinous tubular and spindle cell carcinoma of kidney: A rare case report and review of the literature. Indian J Pathol Microbiol 2009;52:514-6

How to cite this URL:
Geramizadeh B, Salehipour M, Moradi A. Mucinous tubular and spindle cell carcinoma of kidney: A rare case report and review of the literature. Indian J Pathol Microbiol [serial online] 2009 [cited 2023 Sep 23];52:514-6. Available from: https://www.ijpmonline.org/text.asp?2009/52/4/514/56144



   Introduction Top


Since 1990, a unique group of renal tumors has been described, which is composed of cytological low grade cells in elongated tubules and cords of spindle cells in mucinous stroma. [1]

It has been included by the first time in the World Health Organization (WHO) classification of renal neoplasms in December 2004. [2],[3] Until now, approximately 60 cases of this type of renal tumor has been reported. [4] Herein, we report the clinicopathologic findings of an additional case of this unusual tumor.


   Case Report Top


A 63-year-old man presented with right flank pain with no history of hematuria. His general condition was good, and laboratory indices including blood urea nitrogen/creatinine (BUN/Cr) were unremarkable except for high blood glucose. There was no significant past medical history except previous coronary artery bypass graft before three months of this presentation.

Ultrasound showed a right renal mass confined to the kidney. It was confirmed by computed tomography (CT scan), which showed a 4-cm well circumscribed right renal mass [Figure 1].

Right nephrectomy was performed and the specimen was sent for pathologic evaluation. Gross of the kidney showed a well-circumscribed mass, measured 4 X 4 X 3.5 cm 3 . The cut surface was tan-brown with foci of hemorrhage [Figure 2]. There was no invasion to perinephric and hilar fat.

Microscopic sections of the tumor showed microtubules and long cords. Tumor cells showed cuboidal to spindled-shaped morphology [Figure 3] and [Figure 4], with no evidence of atypia. No invasion to vascular structures or pyelocalyceal system was present. No lymphnode involvement was found.

Immunohistochemistry showed positive reactivity for AE1/AE3, EMA, and E-cadherin, but negative staining for vimentin, RCC, CK7, CK20, and CD 10 [Figure 5] and [Figure 6].

With the diagnosis of mucinous tubular and spindle cell carcinoma, the patient was discharged in a very good condition. After that he did well and now after six months he is in good health condition without any further therapy and all the imaging studies are negative.


   Discussion Top


Low grade mucinous tubular and spindle cell carcinoma is a rare and newly described type of renal cell carcinoma with a relatively indolent behavior. [5],[6]

Since its introduction, only single cases and small series have been reported. Until now, less than 60 cases have been reported. [4]

This tumor is seen in adults, mostly in women and is typically detected as an asymptomatic renal mass. [7] Perioperative diagnoses of most of these tumors are renal cell carcinoma and only histopathologic findings can be diagnostic. [6]

These tumors usually present as a single, firm, homogenous, yellow to tan, brown to pinkish, and well-defined mass. [4] Necrosis and hemorrhage are rare. [6]

Histologically, their typical features include an interconnecting tubular and spindled architecture within myxoid stroma and with low grade nuclei. [6]

Presence of compact tubules with focal papillary-like structures in this tumor has some similarities with papillary renal cell carcinoma, but extracellular mucin is absent in the latter. [4]

Spindle cells in this tumor can also been confused with papillary renal cell carcinoma with sarcomatoid changes, but in mucinous tubular and spindle cell carcinoma, all the cells show low grade nuclei. [8],[9]

Although, in most cases, Hematoxylin and Eosin (H and E) staining is diagnostic, several studies of different immunohistochemical marker reactivity has been reported in the cases with this diagnosis, such as reactivity for vimentin (55%), EMA (81%), AE1/AE3 (83%), CK7 (91%), CK19 (78%), CK903 (32%), CD10 (16%), RCC (49%), and E-cadherin (93%). [6]

Our case was an old man with right flank pain. Gross of the tumor showed a well-defined mass with foci of hemorrhage and even necrosis. Histology was typical and immunohistochemistry showed reactive cells for AE1/AE3, EMA, and E-cadherin, but tumor cells were nonreactive with vimentin, RCC, CD10, and CK7.

Prognosis of our case was favorable and it was also true in most of the previous cases, but there are still few cases reported and further investigations are required to determine the exact prognosis of this tumor type. [6]

Our case confirms that because of favorable prognosis and huge difference of prognosis between this tumor and other renal cell carcinomas, pathologists should be aware of this rare tumor and consider it in the differential diagnosis of renal tumors, especially those with spindle cell morphology, and papillary renal cell carcinoma. [10]

 
   References Top

1.Fine SW, Argani P, DeMarzo AM, Delahut B, Sebo TJ, Reuter VE, Epstein JI. Expanding the histologic spectrum of mucinous tubular and spindle cell carcinomaof kidney. Am J Surg Pathol 2006;30:1554-60.  Back to cited text no. 1      
2.Lopez-Belron AL, Scrpelli M, Montironi R, Kirkali Z: 2004 WHO classification of renal tumors of the adults. European Urology 2006;49:798-805.  Back to cited text no. 2      
3.Srigley JR. Mucinous tubular and tubular and spindle cell carcinoma. WHO classification of tumors In: Eble JN, Sauter G, Epstein JI, Sesterhenn IA (eds). Tumors of the genitourinary and male genital organs. IARC, Washington DC, USA.  Back to cited text no. 3      
4.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 immunophenotype overlap warrant diagnostic caution. Am J Surg Pathol 2006;30:13-9.  Back to cited text no. 4      
5.Shen SS, Ro JY, Tamboli P, Truong LD, Zhai Q, Jung SJ, et al. Mucinous tubular and spindle cell carcinoma of kidney is probably a variant of papillary renal cell carcinoma with spindle cell features. Ann Diagn Pathol 2007;11:13-21.  Back to cited text no. 5      
6.Ferlicot S, Allory Y, Comperat E, Mege-Lechevalier F, Dimet S, Sibony M, et al. Mucinous tubular and spindle cell carcinoma:report of 15 cases and a reiview of literature. Virchows Arch 2005;447:978-83.  Back to cited text no. 6      
7.Eble JN. Mucinous tubular and spindle cell carcinoma: newly recognized entities in the renal cell carcinomafamily. Pathology 2003;35:499-504.  Back to cited text no. 7      
8.Kuroda N, Nakamura S, Miyazaki E, Hayashi Y, Taguchi T, Hiroi M, et al. Low grade tubular-mucinous renal neoplasm with neuroendocrine differentiation: A histological, immunohistochemical and ultrastructural study. Pathology International 2004;54:201-7.  Back to cited text no. 8      
9.Flening S. Recently recognized epithelial tumors of the kidney. Curr Diagn pathol 2005;11:162-9.  Back to cited text no. 9      
10.Trabelsi A, Stita W, Yacoubi MT, Rammeh S, Hmissa S, Korbi S. Renal mucinous tubular and spindle cell carcinoma. Can Urol Assoc J 2008;2:635-6.  Back to cited text no. 10      

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Correspondence Address:
Bita Geramizadeh
Department of Pathology, Shiraz University of Medical Sciences, Shiraz - 71345-1864
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.56144

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

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    Abstract
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