J Cancer Res Clin Oncol (2015) 141:345–352 DOI 10.1007/s00432-014-1740-1

ORIGINAL ARTICLE – CLINICAL ONCOLOGY

Clinicopathologic characteristics and prognostic factors of sarcomatoid renal cell carcinoma Yi Yan · Longfei Liu · Jingyu Zhou · Liling Li · Yuan Li · Minfeng Chen · Long Wang · Wei He · Xiao Guan · Xiongbing Zu · Lin Qi 

Received: 11 April 2014 / Accepted: 30 May 2014 / Published online: 2 September 2014 © Springer-Verlag Berlin Heidelberg 2014

Abstract  Objectives  We seek to summarize the clinicopathologic characteristics and prognostic factors of sarcomatoid renal cell carcinoma (SRCC), an uncommon type of renal cell carcinoma. Methods and materials  Between 2004 and 2012, 23 patients with SRCC were treated at a large urology center in south central China. We collect patient’s clinicopathologic features from medical records to assess diagnosis, prognostic factors and efficacy of systemic therapy. Clinical data were absent in 3 cases, and 20 patients were enrolled in the final study. Results Immunohistochemically, almost all SRCC expressed cytokeratin (91 %), epithelial membrane antigen (87 %) and vimentin (100 %). Sarcomatoid differentiation occurs in various kinds of subtypes of RCC with almost the same probability. The median tumor size was 10.5 cm. The CT findings of these tumors revealed low-density (n  = 5; 25 %) or mixed (n = 15; 75 %) masses with necrotic areas and often showed an infiltrative morphology (n = 15; 75 %). All 20 cases demonstrated heterogeneous enhancement, Yi Yan and Longfei Liu have contributed equally to this work. Y. Yan · L. Liu · Y. Li · M. Chen · L. Wang · W. He · X. Guan · X. Zu · L. Qi (*)  Department of Urology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China e-mail: [email protected] J. Zhou  Department of Breast Surgery, Xiangya Hospital, Central South University, Changsha, Hunan, China L. Li  Department of Pathology, Xiangya Hospital, Central South University, Changsha, Hunan, China

and eleven (55 %) cases demonstrated >50 % necrosis. Six cases complicated with calculus and hydronephrosis. Sixteen (80 %) patients demonstrated invasions of tissues localized in Gerota’s fascia, and 8 (40 %) tumors invaded beyond Gerota’s fascia. Fifteen (75 %) patients demonstrated lymph node metastasis, and sixteen (80 %) patients had distant metastasis. Five patients received systemic therapy, and one patient given high-dose interferon-α had a completely response, and one patient received chemotherapy based on gemcitabine had partial response. The median overall survival of all patients was 5.8 months. Patients without distant metastasis had a median overall survival of 35 months compared with 3 months of those with distant metastasis (P 50 % necrosis) within the left kidney which invades the adjacent peritoneum and psoas major. b An

3.3-cm low-density mass within the right adrenal (arrows) that was considered from the left renal tumor. c Multiple low-density masses within the liver (arrows) that were considered form the left renal tumor

examination, 13 cases demonstrated mixed echoic, 6 cases demonstrated hypoechoic and 1 case demonstrated hyperechoic. The CT findings of these tumors revealed low-density (n = 5; 25 %) or mixed (n = 15; 75 %) masses with necrotic areas and often showed an infiltrative morphology (n  = 15; 75 %) (Fig. 1). During the nephrographic phase of enhancement, all 20 cases demonstrated heterogeneous enhancement. Eleven (55 %) cases demonstrated >50 % necrosis, five (25 %) cases demonstrated 50 % necrosis. Different from other studies, calculus and hydronephrosis was presented in 6 (30 %) cases (Figs. 2, 3). The average size of these 6 tumors was 7.4 cm. All six cases have the symptoms of hematuria, four of which were painless, whole course and grass hematuria. Among them, 2 patients underwent percutaneous nephrolithotomy stone lithotripsy

before the detection of tumor, and 1 patient, whose tumor was first discovered in pathological examination, underwent nephrectomy because of calculus and hydronephrosis and had still survived (62 months). Morphologic features of 20 cases on CT and ultrasound were summarized in Table 2. Sixteen (80 %) patients demonstrated invasions of tissues localized in Gerota’s fascia, and 8 (40 %) tumors invaded beyond Gerota’s fascia. Fifteen (75 %) patients demonstrated lymph node metastasis. Sixteen (80 %) patients had distant metastasis at the time of the study, and 12 of these patients had more than 1 metastatic site. Among these metastatic sites, lung (45 %) and liver (30 %) were the most common locations. Local invasion and distant metastasis of 20 cases are summarized in Table 3. Therapy and prognosis All 20 patients underwent radical nephrectomy or cytoreductive nephrectomy with an additional attempt to excise

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Fig. 2  Images of both unenhanced (a) and enhanced (b) CT scanning of sarcomatoid RCC complicated with calculus and hydronephrosis from a 57-year-old male who underwent percutaneous nephrolithot-

omy stone lithotripsy. A 4.5-cm heterogeneous infiltrative low-density mass (25–50 % necrosis) within the right dilatant renal pelvis, residual stones and hydronephrosis was found within the right kidney

Fig. 3  Images of nephrographic phase of enhancement from a 61-year-old male who underwent percutaneous nephrolithotomy stone lithotripsy. a An 8.3-cm heterogeneous infiltrative low-density mass (>50 % necrosis) was originated from the left renal parenchyma of the medium pole, and a double J tube was seen in the renal pelvis.

b A 4.0-cm heterogeneous low-density mass within the left adrenal (arrows) that manifested invasion of the tumor, and multiple low-density masses within the liver that were considered form the left renal tumor. c A coronal multiplanar reformatted image showed the invasion of the medium calyces and the renal pelvis

all tissues and organs that were invaded by the tumor. Two patients were given nephron-sparing surgery as the first therapy and had a tumor recurrence at 14th and 9th month

separately after surgery. Systemic therapy was given to 5 of the 20 patients. Therapeutic regimen included interferon-α, sunitinib and chemotherapy based on gemcitabine. No

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Table 2  Morphologic features of 20 cases on CT and ultrasound Patient

Tumor size (cm)

CT density

Enhancement (Y/N)

Growth pattern

Necrosis (%)

Calculus and hydronephrosis (Y/N)

Ultrasound examination

1 2 3 4 5 6 7 8 9

6.5 5.7 11.4 6.7 18.0 24.1 22.2 6.2 7.4

Mixed Low Mixed Mixed Mixed Mixed Mixed Mixed Mixed

Y Y Y Y Y Y Y Y Y

Well circumscribed Infiltrative Infiltrative Infiltrative Infiltrative Infiltrative Infiltrative Infiltrative Infiltrative

25–50 50 >50 >50 >50 >50 25–50 >50

N N Y Y N N N Y N

Hypoechoic Mixed Hyperechoic Hypoechoic Mixed Mixed Mixed Mixed Mixed

10 11 12 13 14 15 16 17 18 19

4.5 7.5 6.3 19.5 4.0 4.3 8.3 6.8 8.2 23.0

Mixed Low Mixed Mixed Low Low Mixed Low Mixed Mixed

Y Y Y Y Y Y Y Y Y Y

Infiltrative Infiltrative Well-circumscribed Infiltrative Well-circumscribed Well-circumscribed Infiltrative Well-circumscribed Infiltrative Infiltrative

25–50 50 50

Y Y N N N N Y N N N

Mixed Mixed Mixed Mixed Hypoechoic Hypoechoic Hypoechoic Hypoechoic Mixed Mixed

20

9.2

Mixed

Y

Infiltrative

>50

N

Mixed

Table 3  Local invasion and distant metastasis of 20 cases

Survival analysis

Category

Location

No. pts (%)

Invasion of tissues localized in renal fascia

Renal vein Collecting system Perirenal fat

4 (20) 9 (45) 11 (55) 8 (40)

Invasion of tissues out of renal fascia Lymph node metastasis Distant metastasis

Lung Liver Bone Brain Spine Contralateral adrenal

15 (75) 9 (45) 6 (30) 5 (25) 3 (15) 2 (10) 1 (5)

The median overall survival of all patients was 5.8 months (Fig. 4a). Patients without distant metastasis had a median overall survival of 35 months compared with 3 months of those with distant metastasis (P