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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2013  |  Volume : 24  |  Issue : 3  |  Page : 557-560
Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid transformation

Department of Histopathology, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia

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Date of Web Publication24-Apr-2013


Mucinous tubular and spindle cell carcinoma (MTSCC) of the kidney is a recently described entity in the World Health Organization (WHO) 2004 classification and has a rela­tively indolent behavior. Sarcomatoid differentiation has been well documented in most histologic variants of renal cell carcinoma and its presence is known to have a worse prognosis. Its occurrence in an otherwise benign MTSCC is extremely rare. Here, we report a unique case of MTSCC in a 64-year-old patient with multiple areas of high-grade spindle cells and large areas of necrosis in it. The patient had a rapidly fatal clinical outcome.

How to cite this article:
Arafah M, Zaidi SN. Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid transformation. Saudi J Kidney Dis Transpl 2013;24:557-60

How to cite this URL:
Arafah M, Zaidi SN. Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid transformation. Saudi J Kidney Dis Transpl [serial online] 2013 [cited 2022 Oct 5];24:557-60. Available from: https://www.sjkdt.org/text.asp?2013/24/3/557/111066

   Introduction Top

Mucinous tubular and spindle cell carcinoma (MTSCC) of the kidney is an uncommon, re­cently described variant of renal cell carci­noma, first reported in 1997 by Mac Lennan and his colleagues under the name of "low-grade collecting duct carcinoma." According to the World Health Organization (WHO), it is a low-grade polymorphic renal epithelial neo­plasm with mucinous tubular and spindle cell features. [1] Because of the inherent spindle epi­thelial cell component, many of these tumors were previously diagnosed as sarcomatoid papillary renal cell carcinoma (RCC). Till now, there are less than 80 cases of MTSCC reported in the literature. [2] The tumor occurs predominantly in females, with male to female ratio of 1:4. Patients range in age from 13 to 82 years with a mean age of 58 years. The pa­thological spectrum of this tumor and immunohistochemical profile is variable. Histologically, these tumors are characterized by admix­ture of low-grade tubular cuboidal cells with an array of spindle cells in a mucinous back­ground. Variations such as mucin-poor histo­logy and presence of focal papillations can occur. [3] Very few cases have demonstrated re­currence or metastasis to regional lymph nodes, and distant metastases at the time of presentation. [4] Sarcomatoid change in RCC has an overall incidence of 8% and is well docu­mented to occur in the more common subtypes of conventional RCC. [5] Herein, we present an unusual case of MTSCC with sarcomatoid change, who developed multiple distant metas­tases with a rapidly fatal clinical outcome.

   Case Report Top

A 64-year-old male presented with a month's history of abdominal pain, constipation, and progressive swelling on the left side of the abdomen. Computed tomography scan showed large retroperitoneal, well-demarcated mass, measuring 20 × 19 cm, mainly cystic with peripheral solid-enhancing component and multiple tiny calcifications. Metastasis was not detected in the preoperative workup. The pa­tient underwent left open radical nephrectomy. The patient died eight months post-nephrectomy after developing multiple liver, spleen, and left-side pleural metastatic nodules.

Gross examination showed the kidney dis­torted by a large mass located at the upper pole. The cut section revealed a circumscribed golden yellow, variegated mass, 20 × 16 × 12 cm, with areas of hemorrhage and necrosis. The tumor compressed the renal capsule and pelvis but did not invade it. Histologically, the tumor was composed of tubular structures lined by uniform cuboidal cells with scant amphophilic cytoplasm and round nuclei with inconspicuous nucleoli corresponding to Fuhrman grade 2 [Figure 1]a. The tubular com­ponent was associated with a variable amount of Alcian blue-positive mucinous background [Figure 1]b. There were abrupt transitions to the cells with spindle cell morphology. Large areas of necrosis were seen with cholesterol cleft formations. In addition, there were mul­tiple foci composed of high-grade spindle cells, merging with typical benign MTSCC histo­logy. The atypical spindle cells were arranged in poorly formed fascicles and showed mode­rate to severe pleomorphism with occasional mitotic figures [Figure 1]c. Mucinous stroma was absent in the sarcomatoid component. These undifferentiated sarcomatoid components comprised approximately 20% of the tumor. There was lympho-vascular space invasion by these spindle-shaped sarcomatoid cells.
Figure 1:

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The tubular and spindle cell components of classic MTSCC showed strong expression of alpha-methylacyl-CoA racemase (AMACR) [Figure 1]d and cytokeratin-7, and focal ex­pression of epithelial membrane antigen. The RCC antibody, CD10, E-cadherin, and CK-19 were negative. The sarcomatoid component showed patchy positivity for CK-7, was nega­tive for AMACR, and the M1B1 index was raised.

The morphologic appearance and immunohistochemical profile were consistent with the diagnosis of MTSCC of the kidney with sarcomatoid differentiation.

   Discussion Top

MTSCC, a recently described tumor, is unique in renal epithelial neoplasm in that in spite of showing a spindle cell (low-grade sarcomatoid component), it appears to be a tumor with less aggressive biologic behavior. Much is known about this tumor, but its histomorphological features, histogenesis, and relationship to other renal cell tumors remain unidentified. Diffe­rent studies agree that these tumors have a low-grade malignancy, with a low nuclear grade, and low cell proliferation index, and usually have poorly infiltrative borders. [1]

Although generally considered uncommon, the proportions of renal tumors that are MTSCC may be higher because of prior mis-classification as solid/compact variant of pa­pillary RCC or RCC unclassified. Peri-opera­tive diagnosis of most of these tumors is RCC and only histopathologic findings can be diagnostic. [6] Morphologic diversity of MTSCC has been reported and may create diagnostic diffi­culty when the histologic features are not typi­cal. Although the WHO states that sarcomatoid transformation may arise in all types of RCC, sarcomatoid change has been well docu­mented in the more common subtypes of RCC, papillary RCC, chromophobe RCC, and col­lecting duct carcinoma, and has a deleterious effect on the prognosis. [5] It is defined histologically by the presence of spindle cells with nuclear pleomorphism and prominent nucleoli.

Sarcomatoid differentiation in MTSCC is extremely rare, and to the best of our know­ledge, till date only three cases have been reported in the literature with a fatal outcome in two. [7],[8],[9] In our case, the sarcomatoid compo­nent was seen in addition to the typical low-grade spindle cells of MTSCC, the cells of which were pleomorphic with bizarre nuclei and prominent nucleoli with large areas of necrosis. These areas showed high prolifera­tion fraction (M1B1), loss of CK-7 expression, and AMACR negativity, which helped us to differentiate low-grade spindle cells compo­nent in MTSCC and sarcomatoid dedifferen­tiation in the tumor. This transformation re­flects high-grade dedifferentiation in renal epi­thelial tumors and generally has a worse prog­nosis with shorter disease-free survival and earlier, more frequent metastasis [10] as in our case.

To conclude, we presented an extremely rare case of sarcomatoid differentiation in MTSCC. It is important for the pathologist to recognize that sarcomatoid change can occur in an otherwise low-grade MTSCC. It is essential that areas with high-grade spindle cells with an unusual architectural pattern when associated with extensive necrosis, high proliferation fraction, and negative AMACR should be reported and possibility of sarcomatoid diffe­rentiation considered. This is because sarcomatoid differentiation is associated with an aggressive biological behavior and distant me­tastasis with fatal outcome, and thus requires close follow-up of the patient.

   References Top

1.Srigley JR. Mucinous tubular and spindle cell carcinoma. In: Eble JN, Sauter G, eds. World Health Organization Classification of Tumor. Tumors of the Urinary System and Male Genital Organs. Lyon, France: International Agency for Research on Cancer; 2004. p. 40.  Back to cited text no. 1
2.Jung SJ, Yoon HK, Chung JI, Ayala AG, Ro JY. Mucinous tubular and spindle cell carci­noma of the kidney with neuroendocrine dif­ferentiation. Am J Clin Pathol 2006;125:99-104.  Back to cited text no. 2
3.Fine SW, Argani P, DeMarzo AM, 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. 3
4.Srigley JR, Eble JN, Grignon DJ, et al. Unusual renal cell carcinoma (RCC) with prominent spindle cell change possibly related to the loop of Henle. Mod Pathol 1999;12: 107A.  Back to cited text no. 4
5.de Peralta-Venturina M, Moch H, Amin M, et al. Sarcomatoid differentiation in renal cell carcinoma. Am J Surg 2001;25:275-84.  Back to cited text no. 5
6.Ferlicot S, Allory Y, Compérat E, 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. 6
7.Pillay N, Ramdial PK, Cooper K, Batuule D. Mucinous tubular and spindle cell carcinoma with aggressive histomorphology-a sarcomatoid variant. Hum Pathol 2008;39:966-9.  Back to cited text no. 7
8.Dhillon J, Amin MB, Selbs E, Turi GK, Paner GP, Reuter VE. Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid change. Am J Surg Pathol 2009;33:44-9.  Back to cited text no. 8
9.Simon RA, di Sant'agnese PA, Palapattu GS, et al. Mucinous tubular and spindle cell carci­noma of the kidney with sarcomatoid differentiation. Int J Clin Exp Pathol 2008;1:180-4.  Back to cited text no. 9
10.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. 10

Correspondence Address:
Maha Arafah
Pathology Department, College of Medicine, King Khalid University Hospital, King Saud University, P. O. Box 2925, Riyadh 11461
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.111066

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