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  Table of Contents    
ORIGINAL ARTICLE  
Year : 2012  |  Volume : 55  |  Issue : 4  |  Page : 439-442
Mucinous tubular and spindle cell carcinoma of kidney: A clinicopathologic study of six cases


1 Department of Pathology, Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, Pakistan
2 Department of Pathology and Microbiology, Aga Khan University Hospital, Karachi, Pakistan

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Date of Web Publication4-Mar-2013
 

   Abstract 

Background: Mucinous tubular and spindle carcinoma (MTSCC) of kidney is a rare, low-grade polymorphic tumor. Recent studies have described a wide morphology spectrum of this tumor. Aim: To report the clinico-pathologic features of six cases of MTSCC of kidney. Materials and Methods: Six cases of MTSCC of kidney were studied and literature was reviewed. Immunohistochemistry was done by Envision method. Results: The age of the patients ranged from 44 to 84 years (mean 58.5 years). Four patients were males and two were females. The tumor was located in the left kidney in four cases and in the right kidney in two cases. The tumor size ranged from 4.5 to 15 cm (mean 6.4 cm). All tumors exhibited an admixture of tubules, spindle cells, and mucinous stroma in variable proportions. Tubules were predominant in five cases and spindle cells in one case. Psammomatous calcifications, papillations, and necrosis were seen in two cases. Collections of foamy histiocytes were noted in four cases. Cytoplasmic vacuoles and osseous metaplasia were seen in one case each. All cases were Fuhrman's nuclear grade II. Five cases were of stage pT1, and one was pT3. All cases stained positive for alcian blue at pH 2.5. Immunohistochemical stain CK7 was positive in all cases and CD10 was positive in 1/1 case. All patients were alive and well at follow-up of 12-59 months (mean 33.5 months). No metastases were detected. Conclusions: We report six cases of MTSCC of kidney, a rare distinct variant of RCC, with a favorable prognosis. A male predominance was seen in our cases. MTSCC shares histologic and immunohistochemical overlap with papillary renal cell carcinoma (PRCC) and cytogenetic analysis should be performed in difficult cases to avoid a misdiagnosis.

Keywords: CK7, favorable prognosis, kidney, mucinous tubular and spindle cell carcinoma, psammomatous calcification

How to cite this article:
Hussain M, Ud Din N, Azam M, Loya A. Mucinous tubular and spindle cell carcinoma of kidney: A clinicopathologic study of six cases. Indian J Pathol Microbiol 2012;55:439-42

How to cite this URL:
Hussain M, Ud Din N, Azam M, Loya A. Mucinous tubular and spindle cell carcinoma of kidney: A clinicopathologic study of six cases. Indian J Pathol Microbiol [serial online] 2012 [cited 2019 Jul 24];55:439-42. Available from: http://www.ijpmonline.org/text.asp?2012/55/4/439/107776



   Introduction Top


Mucinous tubular and spindle cell carcinoma (MTSCC) of kidney is a rare unique polymorphous tumor. In the 2004 edition of World Health Organization (WHO) classification of renal tumors, it was recognized as a distinct entity. [1] Histologically this tumor is characterized by an admixture of small, elongated tightly packed tubules and spindle cells present in a mucinous background. This tumor carries a favorable prognosis. [1] Our objectives are to describe the clinico-pathologic features of a cohort of MTSCC, along with literature review.


   Materials and Methods Top


Six cases diagnosed as MTSCC of kidney between 2007 and 2011 were reviewed from the surgical pathology files of the Department of Pathology of a tertiary care hospital. The diagnosis of MTSCC was made according to WHO criteria. [1] Patient consent was obtained; however, ethical approval was not sought since this was a retrospective study. All specimens were fixed in 10% buffered formalin and processed routinely for paraffin embedding. Sections of 5 μm thickness were cut and stained with hematoxylin and eosin (H and E). The tumors were analyzed with respect to the percentage of tubules, spindle cells, and myxoid stroma. Fuhrman's nuclear grading system was used for tumor grading. Any additional histological features present were noted. Periodic acid Schiff (PAS) alcian blue stain was performed at pH 2.5.

For immunohistochemistry, sections were dewaxed, rehydrated, and moistened with running tap water. Pretreatment was done in a microwave oven at 800 W for 20 min with target retrieval solution at high pH; they were then incubated with antibodies on an automated immunostaining system (DAKO autostainer plus, Glostrup Denmark) for 25 min at room temperature. Immunohistochemical studies were performed with the ready-to-use Envision system using the following antibodies: CK7 (monoclonal; dilution 1:200 DakoCytomation, Glostrup, Denmark) and CD10 (monoclonal; prediluted RTU DakoCytomation).


   Results Top


Clinical and gross features

Case 1

A 78 year-old man, a known case of prostatic adenocarcinoma and with Gleason's score 6, presented with a history of hematuria on follow-up. A left renal mass was found on computed tomography (CT) scan. Left radical nephrectomy showed a 4.5 × 3 cm circumscribed gray-white solid tumor in the upper pole confined to the kidney [Figure 1].
Figure 1: Gross appearance of mucinous tubular and spindle cell carcinoma. Well-circumscribed, solid homogenous gray-white tumor in the upper pole

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Case 2

An 84 year-old female presented with a history of left flank pain. A 5.3 × 5 cm circumscribed solid tumor was found in the upper pole of the left kidney. Multiple cortical cysts were seen ranging in size from 0.3 to 3.3 cm and a stone was present in the renal pelvis.

Case 3

A 44 year-old male presented with a history of left flank pain. Left nephrectomy showed a 6 × 5 cm circumscribed solid gray-yellow tumor in the lower pole. No capsular or perinephric fat invasion was grossly seen.

Case 4

A 50 year-old male presented with left flank pain associated with a mass. Left radical nephrectomy specimen revealed a 15 × 10 cm tumor involving all the poles. The tumor grossly extended into the perinephric fat with foci of necrosis. Renal sinus was grossly free of tumor.

Case 5

A 50 year-old female presented with 5 months history of right flank pain. The nephrectomy specimen showed a 4.8 cm circumscribed tumor in the upper pole of right kidney grossly confined to the organ.

Case 6

A 45-year-old male presented with left flank pain and body aches. Ultrasonogram showed a tumor in the left lower pole of kidney. The tumor was removed in multiple fragments measuring 3 × 2 cm in aggregate.

Histological Findings

The histological examination of five cases revealed a circumscribed tumor and one case (case 4) showed infiltration into the perinephric fat. All cases were composed of a variable mixture of tubules and spindle cells present in a mucinous background. Tubules were predominant in five cases and spindle cells in one case. The tubules were short [Figure 2]a to elongated, curvilinear [Figure 2]b, lined by cuboidal cells with inconspicuous to small nucleoli set in a mucinous stroma which was rich in four cases and poor in two cases. Focal collapsed parallel tubular arrays were seen lined by spindle cells [Figure 2]c. The tubules showed abrupt transition to spindle cells which were bland [Figure 2]d. Psammomatous calcifications were seen in two cases [Figure 3]a. These were mostly present in the tubular lining and focally in the lumen of the tubules. A focus of osseous metaplasia was noted in one case [Figure 3]b. Well-formed papillae formations were noted in two cases [Figure 3]c. One case showed cytoplasmic clearing [Figure 3]d. Occasional mitoses were seen in all cases. Collections of foamy histiocytes were noted in four cases. Necrosis was seen in two cases (case 4 and 5), but it was not associated with increased mitotic activity. A sparse inflammatory infiltrate comprising of lymphocytes, eosinophils, and focal plasma cells was noted of in five cases. One case exhibited abundant hemorrhage with cholesterol clefts and hemosiderin macrophages. All cases were Fuhrman's nuclear grade 2/4. Five cases were stage pT1, and one case (case 4) was pT3.
Figure 2: (a) Short tubules in a myxoid stroma and (b) elongated serpentine tubules with abundant myxoid stroma (classic MTSCC, H and E, ×10 magnification). (c and d) Transition between tubular and spindle cells (mucin-poor MTSCC, H and E, 10 and ×20 magnification)

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Figure 3: (a) Psammomatous calcifications in the lumen of the tubules (H and E, ×20 magnification). (b) Mucinous tubular and spindle cell carcinoma with true papillae formation (H and E, ×20 magnification). (c) Focus of osseous metaplasia (H and E, ×10 magnification; inset, ×40 magnification). (d) Cytoplasmic vacuole formation (H and E, ×20 magnification)

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The mucinous background of all the cases was positive for alcian blue at pH 2.5. Immunohistochemical stain CK7 was positive in both tubules and spindle cells [Figure 4]a in all cases and CD10 was positive in 1/1 case [Figure 4]b.
Figure 4: (a) Diffuse CK7 and (b) CD10 positivity in both tubules and spindle cells of mucinous tubular and spindle cell carcinoma (immunoperoxidase, ×20 magnification)

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Follow-up

All patients were alive and well with a follow-up of 12-59 months (mean 33.5 months). No metastases were detected.


   Discussion Top


MTSCC of kidney is a rare tumor and approximately 100 cases have been reported. [2] MacLennan et al.[3] in 1997 for the first time recognized this tumor as "low-grade mucinous tubulocystic carcinoma of possible collecting duct origin," while Srigley et al.[4] in 1999 reported this lesion as "unusual renal cell carcinoma with cell change possibly related to the loop of Henle." In the December 2002 WHO consensus conference on the classification of renal neoplasms, it was first recognized as a distinct variant of renal cell carcinoma (RCC) and given the name "mucinous tubular and spindle cell carcinoma" because of its three constituent elements, i.e., mucinous background, tubules, and spindle cells. [1]

MTSCC affects patients of a wide age range (21-81 years; mean 53 years) with a female predominance in the majority of the case series. [5],[6],[7] However, a male predominance was seen in our study. MTSCC is often asymptomatic and detected on ultrasound, but it may present with flank pain, mass, and hematuria. [1],[8] All our patients were symptomatic and majority presented with flank pain. Two patients complained of flank pain with mass and hematuria, respectively. An association with nephrolithiasis was noted by Hes et al.[9] and also seen in one of our cases. MTSCC was reported in a patient on follow-up of prostatic adenocarcinoma by Hes et al.[9] and also in one of our patients. Similarly, an association with synchronous conventional RCC has been described by Hes et al.[9] and Ferlicot et al.[5] and with papillary RCC by Paner et al. [6] Associated renal cysts were seen in three cases reported by Ferlicot et al. [5] and Paner et al.,[6] and also in one of our cases.

The tumor size ranges from 1.8 to 17 cm. [7] Grossly, these tumors are usually circumscribed, and tan gray to pale yellow in color. Rarely, abundant necrosis and hemorrhage is seen grossly, possibly attributed to large tumor size (18 × 17 cm and 14.5 × 14 cm, respectively). [2],[8] Case 4 in our series (15 × 10 cm) also exhibited large areas of necrosis and hemorrhage grossly, but microscopically was unaccompanied by atypia or increased mitotic activity. MTSCC was stage 1 in majority of the cases. [6],[7]

Histologically, the three constituent elements are present in variable proportions, with one element usually dominating the others. Mucinous stroma can be inconspicuous necessitating alcian blue stain for detection and thus causing a diagnostic challenge. [10],[11] MTSCC shares some histological features seen in other types of RCC such as collections of foamy histiocytes, calcifications, clear cell change, and papillations. [10] We also observed these features in our series. Psammomatous calcifications have previously been described in only five cases. [2],[10],[12],[13] Osseous metaplasia has been previously described in only one case [10] and was also noted in one of our cases. Necrosis can also be seen, but is not associated with aggressive behavior. [7],[9],[10]

Immunohistochemically, MTSCC expresses markers of both distal (CK7) and proximal tubular (RCC Ma, AMACR, CD15) differentiation. [7] All our cases expressed CK7 and 1/1 was positive for CD10. The main differential diagnosis of MTSCC is sarcomatoid papillary RCC (PRCC). MTSCC shares significant morphological and immunohistochemical overlap with PRCC, [6] but cytogenetically, gains of chromosomes 7 and 17 and loss of Y chromosome typical of PRCC have not been found in MTSCC. [13] Mucinous stroma, sharp transition areas between spindle cells and tubules coupled with low-grade cytology help to distinguish MTSCC from PRCC, but mucin may rarely be found in PRCC, [14] while MTSCC can be mucin poor. [10],[12] Moreover, PRCC with low-grade spindle cell foci has been described, thus requiring cytogenetic analysis in mucin-poor tumors because of different prognosis of these two tumors. [13] [Table 1] summarizes the reported large series of MTSCC.
Table 1: Comparison with other reported series of mucinous tubular and spindle cell carcinoma

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The prognosis of MTSCC is favorable. [5],[9] All our patients are alive with no evidence of metastasis or recurrence. Rare reports of regional lymph node [7],[9] and distant metastasis and death have been reported. [15]

In summary, we reported six cases of MTSCC of kidney, a rare distinct variant of RCC, with a favorable prognosis. A male predominance was seen in our cases. Grossly evident necrosis is probably of ischemic type and is seen in larger tumors. MTSCC shares histologic and immunohistochemical overlap with PRCC, and cytogenetic analysis should be performed in difficult cases to avoid a misdiagnosis.

 
   References Top

1.Srigley R. Mucinous tubular and spindle cell carcinoma. In: Eble JN, Sauter G, Epstein JI, Sesterhenn IA, editors. World health organization of tumours; pathology and genetics. Tumours of urinary system and male genital organs. Lyon: IARC Press; 2004. p. 40.  Back to cited text no. 1
    
2.Makni SK, Chaari C, Ellouze S, Ayadi L, Charfi S, Abbes K, et al. Mucinous tubular and spindle cell carcinoma of the kidney associated with tuberculosis. Saudi J Kidney Dis Transpl 2011;22:335-8.  Back to cited text no. 2
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3.MacLennan GT, Farrow GM, Bostwick DG. Low-grade collecting duct carcinoma of the kidney: Report of 13 cases of low-grade mucinous tubulocystic renal carcinoma of possible collecting duct origin. Urology 1997;50:679-84.  Back to cited text no. 3
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4.Srigley UR, Eble JN, Grignon DJ, Hartwick RJ. Unusual renal cell carcinoma (RCC) with cell change possibly related to the loop of Henle. Mod Pathol 1999;12:107a.  Back to cited text no. 4
    
5.Ferlicot S, Allory Y, Compérat E, Mege-Lechevalier F, Dimet S, Sibony M, et al. Mucinous tubular and spindle cell carcinoma: A report of 15 cases and a review of the literature. Virchows Arch 2005;447:978-83.  Back to cited text no. 5
    
6.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. 6
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7.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. 7
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8.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. 8
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9.Hes O, Hora M, Perez-Montiel DM, Suster S, Curik R, Sokol L, et al. Spindle and cuboidal renal cell carcinoma, a tumour having frequent association with nephrolithiasis: Report of 11 cases including a case with hybrid conventional renal cell carcinoma/ spindle and cuboidal renal cell carcinoma components. Histopathology 2002;41:549-55.  Back to cited text no. 9
    
10.Fine SW, Argani P, DeMarzo AM, Delahunt B, Sebo TJ, Reuter VE, et al. Expanding the histologic spectrum of mucinous tubular and spindle cell carcinoma of the kidney. Am J Surg Pathol 2006;30:1554-60.  Back to cited text no. 10
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11.Farghaly H. Mucin poor mucinous tubular and spindle cell carcinoma of the kidney, with nonclassic morphologic variant of spindle cell predominance and psammomatous calcification. Ann Diagn Pathol 2012;16:59-62.  Back to cited text no. 11
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12.Argani P, Netto GJ, Parwani AV. Papillary renal cell carcinoma with low-grade spindle cell foci: A mimic of mucinous tubular and spindle cell carcinoma. Am J Surg Pathol 2008;32:1353-9.  Back to cited text no. 12
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13.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 chromosomes 7 and 17 and losses of chromosome Y that are prevalent in papillary renal cell carcinoma. Mod Pathol 2006;19:488-93.  Back to cited text no. 13
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14.Dhillon J, Amin MB, Selbs E, Turi GK, Paner GP, Reuter VE. Mucinous tubular and spindle cell carcinoma of kidney with sarcomatoid change. Am J Surg Pathol 2009;33:44-9.  Back to cited text no. 14
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15.Val-Bernal JF, Gomez-Roman JJ, Vallina T, Villoria F, Mayorga M, García-Arranz P. Papillary (chromophil) renal cell carcinoma with mucinous secretion. Pathol Res Pract 1999;195:11-7.  Back to cited text no. 15
    

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Correspondence Address:
Mudassar Hussain
Department of Pathology, Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore
Pakistan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.107776

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