Multiple carcinosarcomas of the kidney: a case report and review of the literature

  • Authors:
    • Hakan Ozturk
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  • Published online on: September 5, 2014
  • Pages: 212-216
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Carcinosarcomas are biphasic tumors comprising epithelial and mesenchymal components. Primary carcinosarcoma of the kidney is extremely uncommon and accounts for <1% of all malignant renal tumors. Primary carcinosarcoma of the kidney generally occurs after the age of 60 and the majority of the patients are men. This is the case report of a 56‑year‑old male patient who presented with left flank pain. Ultrasonography and computed tomography (CT) revealed ureterohydronephrosis and a left distal ureteral stone (25 mm). renal scan with diethylenetriamine pentaacetate and dimercaptosuccinic acid revealed a non‑functional kidney and a left nephroureterectomy was performed. The pathological examination of the surgical specimen revealed high‑grade multiple carcinosarcomas according to the Union for International Cancer Control and cancer staging according to the tumor‑node‑metastasis classification determined the disease as stage T3aN0M0. At 6 months, the patient was administered systemic adjuvant chemotherapy (CTx) due to widespread lung and liver metastases on 18F‑fluorodeoxyglucose positron‑emission tomography/CT. However, no response was achieved with systemic CTx. the precise histogenesis of this type of cancer has not been determined. Carcinosarcoma of the kidney is a biphasic tumor and its biphasic nature must be confirmed using immunohistochemical methods during pathological diagnosis. The mesenchymal components of sarcomatoid carcinomas must be verified by pathological examination. Metaplastic changes may have malignant potential but should not be considered as malignant lesions. The most significant histopathological parameter that supports the diagnosis of sarcomatoid carcinoma is the identification of transitional zones between the epithelial and mesenchymal cells. Carcinosarcoma is characterized by aggressive malignant potential and a poor prognosis. An effective curative method has not yet been established, with the exception of radical surgery. It is therefore recommended to perform efficient surgical excision with adequate surgical margins.


The case reports on carcinosarcoma of the kidney are extremely rare in the literature. Sarcomatoid kidney carcinoma was first described by Farrow et al (1) in 1968. However, carcinosarcoma of the urinary tract was first described by Robson (2) in 1935. Although this type of tumor accounts for <1% of all malignant renal tumors, it requires strict follow-up upon establishing the diagnosis due to its aggressive nature and high metastatic potential. The presence of the sarcomatoid component is an indication of an aggressive tumor nature (3). Carcinosarcoma of the kidney is a biphasic tumor and the biphasic nature of the tumor must be confirmed using immunohistochemical methods while establishing the pathological diagnosis (4). Tumor location in the renal pelvis and calyceal epithelial components together with mesenchymal malignant components have been considered to promote early metastasis (3).

Case report

Clinical characteristics

A 56-year-old male patient presented with left flank pain persisting over the previous 6 months. The patient's history included diabetes mellitus and heavy smoking. The liver function tests were normal. The blood biochemistry results were as follows: Glucose, 153 mg/dl; creatinine, 1.5 mg/dl; urea, 56 mg/dl; white blood cell count, 7.11×103/µl; hemoglobin, 12.3 g/dl; platelet count, 308,000 mm3; sodium, 133 mmol/l; potassium, 4.9 mmol/l; chloride, 103 mEq/l; calcium, 8.6 mg/dl; and erythrocyte sedimentation rate, 42 mm/h. On physical examination, there was tenderness on palpation in the left lumbar region. Ultrasonography revealed left-sided grade IV hydronephrosis and the computed tomography (CT) revealed left ureterohydronephrosis and a urinary stone in the left distal ureter measuring 25 mm in diameter. Renal scan with dimercaptosuccinic acid and diethylenetriamine pentaacetate revealed a non-functional left kidney and the patient underwent a nephroureterectomy.

Immunohistopathological characteristics

On macroscopic examination, the nephroureterectomy specimen included the left kidney, measuring 18×13×8 mm, a ureteral segment 190 mm in length and a ureteral calculus measuring 25 mm in diameter. The thickness of the renal cortical parenchyma was reduced to 1 mm. Three nodular lesions with irregular margins, brown to dark yellow in color were identified in the kidney, with the largest of the lesions measuring 30 mm in diameter. The total diameter of the nodular lesions was 70 mm. The microscopic examination of the lesions revealed tumor cells with fusiform nuclei and a pink cytoplasm, exhibiting diffuse pleomorphism and areas of necrosis. The mitotic count was 19–20/10 high-power fields. Islands of carcinomatous cells were identified, embedded in a desmoplastic stroma [hematoxylin and eosin (H&E) staining; magnification, x40; Fig. 1]. Sarcomatous areas, composed of pleomorphic fusiform cells with marked atypia were also identified (Fig. 2) (H&E staining, magnification, x40).

On immunohistochemical examination, the tumor cells were pan-cytokeratin+, DKA+, desmin+, vimentin+, CD117, CD34 and S-100 (Figs. 3 and 4). The Ki-67 proliferation index was 70%. Sarcomatous components were identified, together with carcinomatous components and transitional zones between the two. The transitional zones between sarcomatous and carcinomatous areas are demonstrated in Figs. 3 and 4, using pan-cytokeratin and vimentin immunostaining, respectively.

Staging, treatment and outcome

According to Union for International Cancer Control and the tumour-node-metastasis staging system of the European Association of Urology, the case was diagnosed as high-grade, stage T3aN0M0 cystic carcinosarcoma of the kidney (5). On conventional cystoscopy, there was no concomitant bladder tumor. Distant metastasis was not observed at the time of the diagnosis and during nephroureretectomy. However, widespread lung and liver metastases were identified on 18F-fluorodeoxyglucose positron-emission tomography/CT at 6 months follow-up and systemic adjuvant chemotherapy (CTx) was administered. However, there was no response to CTx. The patient was succumbed to the disease in the first year of follow-up.

Ethics statement and informed consent

All the procedures were in accordance with the ethical standards of the responsible Committee on Human Experimentation and with the Helsinki Declaration of 1975, as revised in 2000. Written informed consent was obtained from the patient.


According to the World Health Organization classification of urothelial cancers in 2014, it is recommended to use the term ‘sarcomatoid carcinoma’ for all biphasic malignant tumors in which epithelial and mesenchymal differentiation is confirmed using morphological and/or immunohistochemical criteria (6). However, both definitions were used in this study. Sarcomatoid carcinomas may occur in any location in the urinary tract. Sarcomatoid carcinoma of the bladder is the most common occurrence reported in the literature. Sarcomatoid carcinoma of the kidney is less common, but it is an aggressive tumor with a high metastatic potential. Sarcomatoid carcinoma of the kidney most commonly occurs at ages ≥60 years and the majority of the patients are men. Sarcomatoid carcinomas are solitary tumors and rarely occur in multiple locations (7). Contrary to the information reported in the literature, our case presented with mass lesions in 3 different locations in a cystic hydronephrotic kidney.

There are also other case reports on the multifocal occurrence of these lesions. Ishumura et al (8) reported a case with multiple carcinosarcomatous lesions located concurrently in the kidney and in different locations in the ipsilateral ureter. Sarcomas occur as solitary tumors; however, the identification of tumor lesions with similar histopathological characteristics in different locations of the kidney in the present case suggests that they may be affected by similar carcinogenic factors. The underlying chronic urinary tract obstruction secondary to ureteral lithiasis may have triggered the development of the disease in multiple locations independently from one another. The factors triggering the development of this disease have not yet been clearly determined. Sarcomatoid carcinomas are biphasic tumors with carcinomatous and sarcomatous components that are considered to arise from primitive pluripotent stem cells (9). However, the precise histogenesis of this type of cancer has not been fully elucidated (10). Wang et al (11) identified duplications of chromosomes 3, 7 and 17 and deletion of chromosome 9p21 as the common genetic alterations among sarcomatoid carcinomas of the upper urinary tract.

The biphasic components of the primary carcinosarcoma of the kidney include epithelial and mesenchymal components. The most common epithelial components are urothelial carcinoma, carcinoma in situ, adenocarcinoma, squamous cell carcinoma and small-cell carcinoma. The most common mesenchymal component is leiomyosarcoma and less common components include rhabdomyosarcoma, liposarcoma, osteosarcoma, angiosarcoma, fibrosarcoma, chondrosarcoma, malignant Schwannoma and Ewing's sarcoma (12). The present case was diagnosed with high-grade transitional cell carcinoma together with high-grade leiomyosarcoma. The mesenchymal components of sarcomatoid carcinomas must be confirmed by pathological examination. Osseous and chondroid metaplasia and carcinomas with pseudosarcomatous stroma must be excluded, as metaplastic changes may have malignant potential, but they should not be considered as malignant lesions. One of the most significant histopathological parameters that supports the diagnosis of sarcomatoid carcinoma is the identification of transitional zones between epithelial and mesenchymal cells (10).

The epithelial and mesenchymal components must be distinguished using immunohistochemical methods. The presence of diffuse and marked mitotic activity and atypical mitoses support the diagnosis of sarcomatoid carcinoma (12). Carcinosarcomas are malignant and highly aggressive tumors with a high metastatic potential. Radical nephrectomy is the primary treatment method for renal carcinosarcoma. No beneficial effect of adjuvant radiotherapy and CTx has been demonstrated thus far (9). We did not achieve any response to adjuvant CTx in the present case. The most commonly used chemotherapeutic agents include cisplatin, dacarbazine, docetaxel, gemcitabine, methotrexate, oxaliplatin, doxorubicin, paclitaxel, vincristine and vinorelbine. However, no treatment protocol has been established for adjuvant CTx.

Carcinosarcomas may be asymptomatic, but they may also present as palpable lumbar or abdominal masses, accompanied by lumbar or abdominal pain and hematuria. The patients may also exhibit systemic symptoms, including anorexia, weight loss, malaise, fatigue, fever, night sweats or cough (13).

The etiology of sarcomas remains unknown and there is no sufficient data on their biological behavior. In the literature, there are reports of cases who sustained spontaneous intraperitoneal rupture and life-threatening hemorrhage due to the aggressive nature of carcinosarcomas (14). This type of biphasic tumor is associated with poor prognosis and such tumors are devoid of natural barriers due to the mesenchymal tissue arising from the sarcomatoid component, whereas the tumors typically carry a pseudocapsule. These tumors have high metastatic potential and multifocal occurrence is an indicator of poor prognosis (15), with a 5-year survival rate of <10% in T3 tumors (16). In the present case, multifocal involvement and with advanced stage reduced the life expectancy of the patient.

In conclusion, primary carcinosarcoma of the kidney is a rare tumor with aggressive bilogical behavior and a high metastatic potential. The patients should be closely monitored due to the unknown or unpredictable biological behavior of this tumor. The scarcity of the described cases in the literature does not allow for efficient histopathological classification and staging, further complicating the selection of the appropriate treatment modality with respect to disease stage. Our basic knowledge on carcinosarcomas of the kidney dictates the need for a multidisciplinary approach, including radical surgical excision, radiotherapy and adjuvant CTx.



Farow GM, Harrison EG Jr and Utz DC: Sarcomas and sarcomatoid and mixed malignant tumors of the kidney in adults. 3. Cancer. 22:556–563. 1968. View Article : Google Scholar : PubMed/NCBI


Robson SM: Atypical carcinoma of the urinary bladder simulating myosarcoma. Report of two cases and review of the literature. J Urol. 34:638–669. 1935.


Cheville JC, Lohse CM, Zincke H, Weaver AL, Leibovich BC, Frank I and Blute ML: Sarcomatoid renal cell carcinoma: an examination of underlying histologic subtype and an analysis of associations with patient outcome. Am J Surg Pathol. 28:435–441. 2004. View Article : Google Scholar


Petersen RO: Urologic Pathology. 2nd. JB Lippincott; Philadelphia, PA: pp. 128–132. 1992


Sobin L, Gospodarowicz M and Wittekind C: TNM Classification of Malignant Tumours. Urological Tumours. Renal Pelvis and Ureter. 7th. Wiley-Blackwell, UICC; pp. 258–261. 2009


Eble JN, Sauter G, Epstein JI and Sesterhenn IA: Infiltrating urothelial carcinoma. World Health Organization Classification of TumoursPathology and Genetics of Tumours of the Urinary System and Male Genital Organs. 1st. IARC Press; Lyon: pp. 1022004


Perimenis P, Athanasopoulos A, Gerathy J and Speakman M: Carcinosarcoma of the ureter: a rare, pleomorphic, aggressive malignancy. Int Urol Nephrol. 35:491–493. 2003. View Article : Google Scholar : PubMed/NCBI


Ishimura H, Momose A, Narita S and Kurotaki H: Synchronous multiple carcinosarcoma of the renal pelvis and ureter: a case report. Acta Urol Jap. 56:381–384. 2010.(In Japanese).


Volker HU, Zettl A, Georg S, et al: Molecular findings in two cases of sarcomatoid carcinoma of the ureter: evidence for evolution from a common pluripotent progenitor cell? Virchows Arch. 452:457–463. 2008. View Article : Google Scholar : PubMed/NCBI


Darko A, Das K, Bhalla RS and Heller D: Carcinosarcoma of the ureter: report of a case with unusual histology and review of the literature. Int J Urol. 13:1528–1531. 2006. View Article : Google Scholar : PubMed/NCBI


Wang X, MacLennan GT, Zhang S, et al: Sarcomatoid carcinoma of the upper urinary tract: clinical outcome and molecular characterization. Hum Pathol. 40:211–217. 2009. View Article : Google Scholar : PubMed/NCBI


Maeda D, Fujii A, Yamaguchi K, et al: Sarcomatoid carcinoma with a predominant basaloid squamous carcinoma component: the first report of an unusual biphasic tumor of the ureter. Jpn J Clin Oncol. 37:878–883. 2007. View Article : Google Scholar


Raman JD, Shariat SF, Karakiewicz PI, et al: Does preoperative symptom classification impact prognosis in patients with clinically localized upper-tract urothelial carcinoma managed by radical nephroureterectomy? Urol Oncol. 29:716–723. 2011. View Article : Google Scholar


Quaresima S, Manzelli A, Ricciardi E, et al: Spontaneous intraperitoneal rupture of pyonephrosis in a patient with unknown kidney carcinosarcoma: a case report. World J Surg Oncol. 9:392011. View Article : Google Scholar : PubMed/NCBI


Chromecki TF, Cha EK, Fajkovic H, et al: The impact of tumor multifocality on outcomes in patients treated with radical nephroureterectomy. Eur Urol. 61:245–253. 2012. View Article : Google Scholar


Jeldres C, Sun M, Isbarn H, et al: A population-based assessment of perioperative mortality after nephroureterectomy for upper-tract urothelial carcinoma. Urology. 75:315–320. 2010. View Article : Google Scholar : PubMed/NCBI

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Ozturk H: Multiple carcinosarcomas of the kidney: a case report and review of the literature. Mol Clin Oncol 3: 212-216, 2015
Ozturk, H. (2015). Multiple carcinosarcomas of the kidney: a case report and review of the literature. Molecular and Clinical Oncology, 3, 212-216.
Ozturk, H."Multiple carcinosarcomas of the kidney: a case report and review of the literature". Molecular and Clinical Oncology 3.1 (2015): 212-216.
Ozturk, H."Multiple carcinosarcomas of the kidney: a case report and review of the literature". Molecular and Clinical Oncology 3, no. 1 (2015): 212-216.