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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2011  |  Volume : 22  |  Issue : 2  |  Page : 335-338
Mucinous tubular and spindle cell carcinoma of the kidney associated with tuberculosis

1 Department of Pathology, Habib Bourguiba University Hospital, Sfax, Tunisia
2 Department of Urology, Habib Bourguiba University Hospital, Sfax, Tunisia

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Date of Web Publication18-Mar-2011


Mucinous tubular and spindle cell carcinomas (MTSCC) are low-grade renal epi­thelial neoplasms with approximately 100 documented cases reported in the literature. We report a case of MTSCC in a 79-year-old patient in association with a renal tuberculosis infection that has never been reported. Further investigations are needed to determine the frequency and true prognosis of these tumors.

How to cite this article:
Makni SK, Chaari C, Ellouze S, Ayadi L, Charfi S, Abbes K, Slimen MH, Mhiri MN, Boudaoura TS. Mucinous tubular and spindle cell carcinoma of the kidney associated with tuberculosis. Saudi J Kidney Dis Transpl 2011;22:335-8

How to cite this URL:
Makni SK, Chaari C, Ellouze S, Ayadi L, Charfi S, Abbes K, Slimen MH, Mhiri MN, Boudaoura TS. Mucinous tubular and spindle cell carcinoma of the kidney associated with tuberculosis. Saudi J Kidney Dis Transpl [serial online] 2011 [cited 2022 Oct 5];22:335-8. Available from: https://www.sjkdt.org/text.asp?2011/22/2/335/77624

   Introduction Top

A novel entity among low-grade renal epithe­lial neoplasms has recently been identified un­der the denomination of "mucinous tubular and spindle cell carcinoma" (MTSCC), which was first recognized as a specific entity in the World Health Organization consensus conference on the classification of renal neoplasms in Decem­ber 2002.1 To date, approximately 100 cases of MTSCC have been reported in series, with metastases occurring in rare cases; however, no tumor-related death has been reported.2-4 The histological profile of this tumor is well established. We report the clinical, histological and immunohistochemical study of a case.

   Case Report Top

A 79-year-old male was admitted to the uro­logical department of the University Hospital of Sfax after an episode of asymptomatic gross hematuria two months earlier. Physical exami­nation revealed a painless, hard smooth and mobile tumor in the left flank and hypochon­drium. The magnetic resonance imaging (MRI) showed a large tumor mass in the left kidney measuring 18 × 17 × 10 cm, which well cir­cumscribed, and the T1 and T2 images showed centripetal enhancement delimiting a central area in the form of a starry scar [Figure 1]. A total left nephrectomy was carried out. Grossly, the kidney measured 24 × 21 × 11 cm. In the sagittal section, the tumor was replacing the volume of the kidney, possessing a beige­yellow color; foci of hemorrhage and necrosis were observed. The residual renal parenchyma, the hilar elements and the ureter had a normal appearance. During microscopy, the tumor was predominantly composed of tightly packed and elongated tubular areas, showing a parallel dis­position. Tubules were lined by cuboidal cells with pale to eosinophilic cytoplasm. Tumor cells contained centrally located round nuclei without significant nuclear atypia. Most nuclei contained easily discernible small to medium­sized nucleoli, and an abundant extracellular mucin of acid epithelial type was present. Mu­cin was strongly positive for Alcian blue. Mi­tosis and vascular invasion were not found. There was a focal spindle cell area [Figure 2]. Area of ischemic necrosis was found and psa­mmoma bodies were focally present. There was no intra-tumoral lymphocytic or neuto­philic inflammation. Tumor was confined with­in the kidney with no perirenal adipose tissue or renal vein invasion. The remaining non­tumoral renal tissue contains a tuberculoid granuloma with sometimes a floor of soft ca­seous material in the middle; the Zhiel Neelsen coloration revealed a slightly curved bacilli [Figure 3]. The immunohistochemical study was realised. A panel of antibody cyto-keratin 7 (CK7), epithelial membrane antigen (EMA) [Figure 4]a, vimentine [Figure 4]b, CD10 and CD15 (proximal nephron) were performed. The tumor cell carcinoma showed a diffuse reacti­vity to EMA and CK7 and a focal positivity for the vimentine was observed [Figure 5]. There was no reactivity for CD15 or CD10. The diagnosis was MTSCC of the kidney asso­ciated with tuberculosis. The tumor stage was pT1NxM0. The follow-up one year later was favorable, with no evidence of recurrence.
Figure 1: Magnetic resonance imaging (MRI): Large tumor mass in the left kidney well circumscribed with centripetal enhancement.

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Figure 2: A: Proliferation of tightly packed and elongated tubules with a parallel disposition; tumor cells are small with monomorphic, cuboidal shape (HE × 200). Inset: Alcian blue staining of mucinous stroma. (AB × 400). B: Presence of small foci with spindle cell sarcomatoid differentiation (HE × 400).

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Figure 3: Tuberculoid granuloma with caseous materiel in the middle (HE × 400). Inset: Slightly curved bacilli (Zhiel Neelsen × 1000).

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Figure 4: A: Diffuse positivity to EMA (× 400). B: focal positivity for the vimentine (× 400).

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   Discussion Top

MTSCC is a new tumoral entity that has been recently established. It was first recognized and described by Mac in 1997 and then by Srigley et al in 1999. [5] The present case is a sig­nificant example of the complexity expressed by certain forms of renal carcinomas. Our case corresponds, upon its histological and immu­nohistochemical features, to MTSCC, a recent and rare entity. Interestingly, we noted in this case the association with a renal tuberculosis infection. This association had never been re­ported in the literature. MTSCC is preferen­tially observed in adults. Patients' age ranged from 21 to 81 years, with a mean and median of 55.8 and 58.5 years, respectively. The male to female ratio was 1/3. [2],[6] The right kidney was more involved. [7] Their diameter varied bet­ween 1.8 and 17 cm (mean diameter, 6 cm). [2] The tumor is usually present as an asympto­matic mass, often found on ultrasound. Occa­sionally, the patients may present with flank pain or hematuria. [1] The tumors were generally confined (pT1 or pT2) in more than 80% of the cases. [2],[3],[7] Hemorrhage and necrosis may be present. [2],[3],[6] Histologically, the tumor has tubu­lar and solid growth patterns. The predominant architectural pattern was that of vague tubular growth, tightly packed, small elongated tubules with rare, well-formed papillae, separated by pale myxomatous stroma. [4] Cells are small, with a cuboidal or oval shape, eosinophilic cyto­plasm and low-grade nuclear features. The tu­bules are associated with a proliferation of spindle cell components. [2],[3],[6],[8] A spindle cell area formed cords and fascicles mimicking a mesenchymal neoplasm. [6] Fine et al [6] recognized two different variants of MTSCC, "a classic" variant (60% of cases) owing to the presence of characteristic abundant extracellular blue gray mucinous/myxoid matrix (>50%) accom­panying the typical tubular and spindle cell epithelial components. The second variant was designated "mucin-poor." These cases were dis­tinguished by a lack of appreciable extracel­lular mucin on H & E staining. The majority of the cases showed scant (<10%) mucin in cel­lular areas on Alcian blue. Histochemically, the myxomatous stroma exhibits a positive re­action for Alcian blue at pH 2.5. [2],[4] A small cluster of foamy macrophages was frequently associated with mucin collections. [2],[6] MTSCC tumors have a complex immunoprofile. Shen et al [3] demonstrated the expression of differen­tiation markers of proximal tubules, which were renal cell carcinoma marker antigen (RCC Ma) (92%), a-Methylacyl-COA racemase (AMACR) (92%), CD15 (Leu M1) (67%) as well as CK7, which is known to stain the distal tubules (92%). Positive staining was seen with mar­kers that were found in the collecting duct epithelium, including EMA and peanut agglu­tinin (PNA). [2] The remaining markers, which are more specific to the proximal tubules, are CD10, the collecting duct (aquaporin 3) and the kidney-specific cadherin expressed in only a few cases. [3],[9] The vimentine positivity was infrequent. It is observed in 14-40% of the ca­ses. [2],[3],[9] The proliferative rate (MIB-1) was low, suggesting a low proliferation activity, [2],[8] a finding that may in part explain the low malignancy of this tumor type. The immuno­histochemical profile diversity (positivity for markers of the pro-ximal tubules as well as for markers for the distal part of the nephron) suggests tumor cells being pluripotent and dif­ferentiates in different directions based on se­quentially acquired genomic abnormalities. [8] Ultrastructurally, the spindle cells show fea­tures like tight junctions, desmosomes, micro­villous borders, luminal borders and occasio­nal microfilaments. [1] Using comparative geno­mic hybridization (CGH), Rakozy et al [2] first demonstrated that MTSCC showed consistent multiple chromosomal loss (-1, -4, -6, -8, -9, - 13, -14, -15 and -22). Similar results have been reported in subsequent studies. [8],[10] In addition, gains of chromosomes (+7, +16, +17, +20) have also been reported. [8],[10] The association with other renal abnormality as a simple renal cyst, a synchronous renal cell carcinoma, a papillary adenoma [7] or angiomyolipoma was reported. [6] The association with tuberculosis has never been reported. The coexistence of these lesions seems to be a coincidence because of the ende­mic nature of tuberculosis in our country. The differential diagnosis is tubulopapillary carci­noma, particularly the basophilic (type1) tumors with prominent solid growth pattern, metane­phric adenoma, sarcomatoid carcinoma and low­grade collecting-duct carcinoma. [4],[7] The use of immunomarkers has so far been more pivotal in difficult cases, especially when cytogenetic studies are not feasible. [7],[8] The treatment was surgical, and it consists of a widened nephrec­tomy. A partial nephrectomy may be indicated when it is a small tumor. [2],[3] The prognosis is generally favorable: [7],[9] only lymph node metas­tases were reported. There has been no repor­ted case of distant organ metastasis or cancer­specific deaths due to MTSCC. [2],[3],[4] Further in­ vestigations are required to determine the fre­quency and true prognosis of these tumors. [9]

   References Top

1.Srigley R. Mucinous tubular and spindle cell carcinoma. In: Eblc JN, Sauter G, Epstein JI, Sesterhenn IA (eds). World health organisation classification of tumours: pathology and genetics. Tumours of urinary system and male genital organs. IARC Press: Lyon 2004:40.  Back to cited text no. 1
2.Rakozy C, Schmahi GE, Bogner S, Storkel S. Low grade tubular mucinous renal neoplasms: Morphologic, immunohistochemical, and genetic features. Mod Pathol 2002;15:1162-71.  Back to cited text no. 2
3.Shen SS, RO JY, Tamboli P, 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. 3
4.Kuroda N, Toi M, Hiroi M, Shuin T, Enzan H. Review of mucinous tubular and spindle cell carcinoma of the kidney with a focus on clini­cal and pathobiological aspects. Histopathology 2005;20: 221-4.  Back to cited text no. 4
5.Srigley JR, Eble JN, Grignon DJ, Hartwick RW. 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. 5
6.Fine SW, Argani P, DeMarzo, 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. 6
7.Paner GP, Srigley JR, Radhakrishnan A, 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. 7
8.Brandle P, Lie AK, Bassarova A, et al. Geno­mic aberrations in mucinous tubular and spin­dle cell renal cell carcinomas. Mod Pathol 2006;19:186-94.  Back to cited text no. 8
9.Ferlicot S, Allory Y, Compérat E, et al. Muci­nous tubular and spindle cell carcinoma: A report of 15 cases and review of the literature. Virchows Arch 2005;477:978-83.  Back to cited text no. 9
10.Weber A, Srigley J, Moch H. Mucinous spin­dle cell carcinoma of the kidney. A molecular analysis. Pathology 2003;24:453-9.  Back to cited text no. 10

Correspondence Address:
Saloua Krichen Makni
Department of Pathology, Habib Bourguiba University Hospital, 3029 Sfax
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Source of Support: None, Conflict of Interest: None

PMID: 21422638

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