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Primary leiomyosarcoma kidney – A rare entity with a diagnostic challenge

1 Department of Pathology, Maulana Azad Medical College, Lok Nayak Hospital, New Delhi, India
2 Department of Surgery, Maulana Azad Medical College, Lok Nayak Hospital, New Delhi, India

Date of Submission12-Feb-2019
Date of Decision23-Aug-2019
Date of Acceptance29-Oct-2019
Date of Web Publication26-Nov-2020

Correspondence Address:
Shramana Mandal,
Department of Pathology, Maulana Azad Medical College, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.JCRT_107_19

 > Abstract 

Primary leiomyosarcoma kidney is a rare tumor with an aggressive nature. Leiomyosarcoma is one of the common histologic types of the sarcomas, comprising 60% of all sarcomas. Sarcomatoid renal cell carcinoma is a close differential of renal leiomyosarcoma as both tumors have spindle-shaped cells. The former has a more pleomorphic character with nuclear grade four, which can be differentiated on the basis of immunohistochemistry. Hence, the diagnosis of primary renal leiomyosarcoma poses a diagnostic challenge. One such case of renal leiomyosarcoma in a 45-year-old male is being discussed here.

Keywords: Leiomyosarcoma, pleomorphic, sarcomatoid carcinoma

How to cite this URL:
Chaudhary D, Rath A, Mandal S, Khurana N, Agarwal P N. Primary leiomyosarcoma kidney – A rare entity with a diagnostic challenge. J Can Res Ther [Epub ahead of print] [cited 2022 Jul 1]. Available from: https://www.cancerjournal.net/preprintarticle.asp?id=300995

 > Introduction Top

Renal sarcomas constitute 1%–2% malignant renal tumors.[1] Leiomyosarcomas constitue 60% of all sarcomas.[2] To the best of our knowledge, this is one of the few reported cases from the Indian subcontinent. We report a case of leiomyosarcoma kidney in a 45-year-old male, while most of the reported cases are in females.

 > Case Report Top

A 45-year-old male presented with pain in the left flank region for 2 years. No other abnormality was found on both general and systemic examination. There was no history of hematuria/palpable mass per abdomen. Renal ultrasonography was suggestive of nodular hyperechoic lesion 2.5 cm × 2.5 cm near the hilum of the left kidney. Contrast-enhanced computed tomography of the abdomen revealed enhancing lesion adjacent to the left hilum measuring 3 cm × 2 cm in the perirenal area with retroperitoneal lymphadenopathy. Frozen sections sent showed fascicles and bundles of spindled out cells with mild–moderate pleomorphism and focal mitosis. No necrosis/epithelial components were seen. A left radical nephrectomy was done. On gross, the kidney measured 10 cm × 7 cm × 2 cm with globular gray–white growth near the hilum measuring 3 cm × 2 cm × 2 cm [Figure 1]a.
Figure 1: (a) Gross: Gray–white growth near the hilum of the left kidney, (b) H and E sections showing a tumor with adjacent normal renal parenchyma (×100)

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Microscopically, a tumor was composed of spindle-shaped cells arranged in fascicles [Figure 1]b and [Figure 2]a. The cells had moderate eosinophilic cytoplasm, oval vesicular nucleus, and moderate pleomorphism. Few mitotic figures were noted [Figure 3]b. Few giant cells were also noted. The tumor was seen infiltrating into the surrounding perinephric fat [Figure 2]b, and there was evidence of perineural invasion [Figure 3]a. However, no foci of hemorrhage or necrosis was identified. No epithelial component was recognized after the extensive sampling.
Figure 2: (a) H and E sections with tumor composed of spindle cells in fascicles (×200), (b) H and E sections with tumor infiltrating perinephric fat (×200)

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Figure 3: (a) H and E sections showing tumor with perineural invasion (×200), (b) H and E sections showing tumor with frequent mitosis (×400)

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Immunohistochemically, the tumor cells showed diffuse and strong positivity for smooth muscle actin, vimentin, and desmin [Figure 4]a, [Figure 4]b and [Figure 5]a. Tumor cells were negative for epithelial membrane antigen, CK, HMB45, S100, and Ki-67 index [Figure 5]b and [Figure 5]c. On the basis of morphology and immunohistochemical findings, the diagnosis of leiomyosarcoma kidney was rendered.
Figure 4: (a) Immunohistochemistry – positive for vimentin, (×400), (b) immunohistochemistry – positive for smooth muscle actin (×400)

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Figure 5: (a) Immunohistochemistry - positive for desmin (×400), (b) immunohistochemistry negative for CK, (c) immunohistochemistry for Ki-67 index negative

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 > Discussion Top

Leiomyosarcoma is a malignant tumor of smooth muscle component. Leiomyosarcomas can involve the uterus, retroperitoneum, lower extremity, and head and neck. The kidney is a rare site for leiomyosarcoma, representing 1%–2% of all malignant renal tumors. An extensive literature search has shown that only 11 cases of leiomyosarcoma kidney have been reported in the Indian subcontinent. We hereby report the 12th case [Table 1]. They can arise from the renal capsule, smooth muscle tissue of the vessels, or renal pelvic wall.[3] In the present case, the tumor had arisen from the hilum.
Table 1: Recent case reports of primary leiomyosarcoma kidney in the Indian subcontinent

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Signs and symptoms are similar to renal cell carcinoma (RCC) with a history of a palpable mass, hematuria, and pain and hence indistinguishable from it. They are more common in females with a mean age of presentation 50–60 years. Our case is unique as our patient was a male in the fourth decade. The tumor has more preponderance for the right side.[4] Grossly, it has a whorled appearance with the areas of hemorrhage, necrosis, and cystic degeneration.[5] Microscopically, alternating fascicles of spindle-shaped cells are seen with individual cell having a blunt end and eosinophilic cytoplasm.[4] There are areas of necrosis and nuclear pleomorphism with variable mitotic figures.

Renal sarcomas originate from the capsule or perisinus region, possess a pseudocapsule, and can expand to large size. There are chances of frequent metastases with a high recurrence rate.

Differential diagnosis of leiomyosarcomas includes sarcomatoid RCC, monophasic synovial sarcoma, epithelioid angiomyolipoma, fibrosarcoma, and malignant peripheral nerve sheath tumor. Distinguishing features between these are described in [Table 2].
Table 2: Differential diagnosis of primary leiomyosarcoma kidney

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Radical nephrectomy is the recommended treatment.[6] The role of adjuvant chemotherapy/radiotherapy remains unclear, The tumor shows a unfavorable prognosis with metastasis to the lungs, liver, and colon.[6] The mean survival rate of renal leiomoyosarcoma is quite low, varying from 6 months to 2 years.[1]

Small size (<5 cm), low histologic grade, and renal-limited disease are some favorable prognostic factors.[7] Histologic grade of the tumor depends on the mitotic count, necrosis, and nuclear pleomorphism.[7] To conclude, leiomyosarcoma kidney is a rare tumor with a poor prognosis. Histopathological examination, extensive sampling of the tumor along with immunohistochemistry, is necessary for its diagnosis to exclude sarcomatoid type RCC.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

Demir A, Yazici CM, Eren F, Türkeri L. Case report: Good prognosis in leiomyosarcoma of the kidney. Int Urol Nephrol 2007;39:7-10.  Back to cited text no. 1
Sharma D, Pradhan S, Aryya NC, Shukla VK. Leiomyosarcoma of kidney: A case report with long term result after radiotherapy and chemotherapy. Int Urol Nephrol 2007;39:397-400.  Back to cited text no. 2
Choudhury M, Singh SK, Pujani M, Pathania OP. A case of leiomyosarcoma of kidney clinically and radiologically misdiagnosed as renal cell carcinoma. Indian J Cancer 2009;46:241-3.  Back to cited text no. 3
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Farrow GM, Harrison EG Jr., Utz DC, ReMine WH. Sarcomas and sarcomatoid and mixed malignant tumors of the kidney in adults. I. Cancer 1968;22:545-50.  Back to cited text no. 4
Deyrup AT, Montgomery E, Fisher C. Leiomyosarcoma of the kidney: A clinicopathologic study. Am J Surg Pathol 2004;28:178-82.  Back to cited text no. 5
Venkatesh K, Lamba Saini M, Niveditha SR, Krishnagiri C, Babu S. Primary leiomyosarcoma of the kidney. Patholog Res Int 2010;2010:652398.  Back to cited text no. 6
Dhamne SA, Gadgil NM, Padmanabhan A. Leiomyosarcoma of the renal pelvis. Indian J Pathol Microbiol 2009;52:549-51.  Back to cited text no. 7
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1], [Table 2]


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