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CORRESPONDENCE
Year : 2018  |  Volume : 14  |  Issue : 3  |  Page : 694-696

Primary mesenchymal chondrosarcoma of the kidney: A case report and review of literature


1 Department of Urology, Hospital de São João, Porto; Department of Urology, Centro Hospitalar do Tâmega e Sousa, Penafiel, Portugal
2 Patology Laboratory Dr. Macedo Dias, Porto, Portugal
3 Department of Urology, Military Hospital; Department of Urology, Hospital da Lapa, Porto, Portugal
4 Department of Urology, Hospital da Lapa; Medicine Faculty, Porto University, Porto, Portugal
5 Department of Urology, Hospital de São João; Department of Urology, Hospital da Lapa, Porto, Portugal

Date of Web Publication12-Jun-2018

Correspondence Address:
Dr. Pedro Valente
Rua Engenheiro Machado Vaz, N 200, Hab. 1.2, 4250-271, Porto
Portugal
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.172121

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 > Abstract 


Primary mesenchymal chondrosarcoma of the kidney is extremely rare, with only nine cases reported in the English literature. We report a new case of this disease. A 35-year-old man, presented with flank pain, episodic gross hematuria and a painless palpable mass in left abdominal quadrant. Computed tomography scan identified a left renal tumor with 20 cm, with no evidence of regional or metastatic spread disease. The patient underwent radical nephrectomy. The immunohistopathological diagnosis was mesenchymal chondrosarcoma of the kidney. At 18 months of follow-up, there was no evidence of recurrence or distant metastasis. Primary renal chondrosarcoma is so rare that its prognosis is unknown. Disease recurrence is unpredictable and when it is detected, the prognosis is poor. The radical nephrectomy with complete resection of the tumor with wide resection free margins is recommended, and the patients need long-term and close surveillance, with particular attention to local recurrence and uncommon sites of metastization.

Keywords: Chondrosarcoma, kidney, nephrectomy, renal, tumor


How to cite this article:
Valente P, Macedo-Dias JA, Lobato C, Reis M, Pina F. Primary mesenchymal chondrosarcoma of the kidney: A case report and review of literature. J Can Res Ther 2018;14:694-6

How to cite this URL:
Valente P, Macedo-Dias JA, Lobato C, Reis M, Pina F. Primary mesenchymal chondrosarcoma of the kidney: A case report and review of literature. J Can Res Ther [serial online] 2018 [cited 2020 Jul 9];14:694-6. Available from: http://www.cancerjournal.net/text.asp?2018/14/3/694/172121




 > Introduction Top


Mesenchymal chondrosarcoma was first reported by Lightenstein and Bernstein in 1959. This malignant tumor mostly arises in the skeleton, with one-third of cases arising in soft tissues and other organs. Extraskeletal mesenchymal chondrosarcoma usually appears in the head and neck region, followed by the lower extremities, the trunk, and the retroperitoneum.[1]

Primary mesenchymal chondrosarcoma of the kidney is extremely rare, with nine cases of primary renal chondrosarcoma reported in the English literature until now, the first case being published 30 years ago by Pitfield et al.[2],[3],[4],[5],[6],[7],[8],[9],[10] We report the tenth case of mesenchymal chondrosarcoma of the kidney.


 > Case Report Top


A 35-year-old man, with no remarkable medical history, presented with diffuse and unspecific left flank pain and episodic gross hematuria. Physical examination revealed a painless palpable mass in left abdominal quadrant, with 15 cm in length. Blood and urine analysis presented within the normal values.

Computed tomography scan identified a left renal calcified tumor with 20 cm in length, with no evidence of regional or metastatic spread disease [Figure 1].
Figure 1: Computed tomography scan: Transversal view showing left renal tumor with calcifications

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A clinical diagnosis of malignancy of left kidney was made, and the patient underwent left radical nephrectomy via combined midline and left subcostal intra- and extra-peritoneal approach. There were no perioperative complications reported.

Macroscopically, the kidney was 90% occupied by a bulky intra-capsular tumor with 20 cm in length, and in the hilar grass, two nodular masses with 2 cm each was identified.

Microscopically, the tumor destroyed the kidney structures, and proliferation consisted of two components, cartilage with calcification and mesenchymal tissue with negative surgical margins [Figure 2] and [Figure 3].
Figure 2: Histological view: Malignant tissue with atypical fusiform cells (H and E, ×100)

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Figure 3: Histological view: Malignant tissue with mature cartilage (H and E, ×100)

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The nodular hilar masses were similar to malignant tissue. Immunohistochemical staining was positive for Vimentin, CD99, and S-100 protein and negative for Cytokeratin, E-Cadherin, Desmin, and WT1 [Figure 4]. Histopathological diagnosis was mesenchymal chondrosarcoma of the kidney.
Figure 4: Histological view: Imunohistochemical staining positive for S-100, vimentina, and CD99

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After 18 months of follow-up, there was no evidence of recurrence or distant metastasis.


 > Discussion Top


It is not surprising that extraskeletal mesenchymal chondrosarcoma could arise from the kidney because cartilage and kidney have the same mesodermal origin, but only nine reports of primary renal chondrosarcoma in the English literature were found. The distribution of ages of patients was between 16 and 67 years old.[2],[3],[4],[5],[6],[7],[8],[9],[10]

In six cases, the patient presented with flank pain and hematuria; in two cases, the patient was asymptomatic and referred for investigation of an incidental mass found in abdominal ultrasonography; and in one case, the patient presented with shoulder and neck pain. The tumors ranged in size from 3 to 23 cm. All cases were treated with radical nephrectomy or nephroureterectomy, and in one case para-aortic lymphadenectomy was performed.

In one case, surgical margins were positive, and the patient died 2 months after surgery with local recurrence and progression of the disease.[8] In four cases, distant metastasis were identified from 1 to 28 months after surgery and finally succumbed to the disease, even in the case treated with adjuvant radiotherapy and chemotherapy. In two cases, there was no evidence of recurrence or distant metastasis at 1-year and 6 years of follow-up, respectively. There is one report of liver metastasis of primary renal chondrosarcoma 18 years after the nephrectomy. The metastasis was resected, but the patient died 2 years later with the progression of the disease.[9]

Considering all organs, the prognosis for mesenchymal chondrosarcoma is usually poor. Of 23 patients with long-term follow-up in a Mayo Clinic series, the survival rates were 54.6% at 5 years, and 27.3% at 10 years.[11]

In our report, the tumor was completed resected with wide negative surgical margins, and at 18 months of follow-up, there was no evidence of recurrence or distant metastasis.

Primary renal chondrosarcoma is so rare that its prognosis is unknown. Disease recurrence is unpredictable (1 month–18 years), and when it is detected, the prognosis is poor since the tumor responds badly to radiotherapy or chemotherapy.[2],[3],[4],[5],[6],[7],[8],[9],[10]

The radical nephrectomy with complete resection of the tumor with wide resection free margins is recommended, and the patients need long-term and close surveillance with particular attention to local recurrence and noncommon sites of metastization.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
Lightenstein L, Bernstein D. Unusual benign and malignant chondroid tumors of bone. A survey of some mesenchymal cartilage tumors and malignant chondroblastic tumors, including a few multicentric ones, as well as many atypical benign chondroblastomas and chondromyxoid fibromas. Cancer 1959;12:1142-57.  Back to cited text no. 1
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2.
Pitfield J, Preston BJ, Smith PG. A calcified renal mass: Chondrosarcoma of kidney. Br J Radiol 1981;54:262.  Back to cited text no. 2
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3.
Malhotra CM, Doolittle CH, Rodil JV, Vezeridis MP. Mesenchymal chondrosarcoma of the kidney. Cancer 1984;54:2495-9.  Back to cited text no. 3
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4.
Nativ O, Horowitz A, Lindner A, Many M. Primary chondrosarcoma of the kidney. J Urol 1985;134:120-1.  Back to cited text no. 4
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5.
Gomez-Brouchet A, Soulie M, Delisle MB, Escourrou G. Mesenchymal chondrosarcoma of the kidney. J Urol 2001;166:2305.  Back to cited text no. 5
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6.
Kaneko T, Suzuki Y, Takata R, Takata K, Sakuma T, Fujioka T. Extraskeletal mesenchymal chondrosarcoma of the kidney. Int J Urol 2006;13:285-6.  Back to cited text no. 6
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7.
Buse S, Behnisch W, Kulozik A, Autschbach F, Hohenfellner M. Primary chondrosarcoma of the kidney: Case report and review of the literature. Urol Int 2009;83:116-8.  Back to cited text no. 7
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8.
Xu H, Shao M, Sun H, Li S. Primary mesenchymal chondrosarcoma of the kidney with synchronous implant and infiltrating urothelial carcinoma of the ureter. Diagn Pathol 2012;7:125.  Back to cited text no. 8
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9.
Mehanna D, Abu-Zidan FM, Rao S. Liver metastasis in renal chondrosarcoma. Singapore Med J 2004;45:183-5.  Back to cited text no. 9
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10.
Rothberg MB, Bhalodi AA, Reda EF, Zelkovic P, Franco I. Primary renal mesenchymal chondrosarcoma: A case report. Urology 2015;85:676-8.  Back to cited text no. 10
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11.
Nakashima Y, Unni KK, Shives TC, Swee RG, Dahlin DC. Mesenchymal chondrosarcoma of bone and soft tissue. A review of 111 cases. Cancer 1986;57:2444-53.  Back to cited text no. 11
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    Figures

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



 

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