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CASE REPORT
Year : 2021  |  Volume : 17  |  Issue : 2  |  Page : 602-605

A rare case of giant chromophobe renal cell carcinoma with sarcomatoid differentiation: Treatment and prognosis


1 Department of Urology, JIPMER, Puducherry, India
2 Department of Pathology, JIPMER, Puducherry, India

Date of Submission27-Jul-2020
Date of Decision10-Sep-2020
Date of Acceptance08-Jan-2021
Date of Web Publication11-Jun-2021

Correspondence Address:
Ramanitharan Manikandan
Department of Urology, JIPMER, Puducherry
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcrt.JCRT_1051_20

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 > Abstract 


Chromophobe renal cell carcinoma (RCC) with sarcomatoid differentiation is an infrequent entity. In the current era of advanced imaging techniques, RCCs are usually detected in early stages, and a localized giant tumor is rarely encountered. Recently, we encountered a patient with a localized giant RCC, which was 36 cm in the largest dimension and weighing 5.1 kg, which was resected entirely but presented relapse and succumbed within 3 months of surgery.

Keywords: Chromophobe renal cell carcinoma, giant renal cell carcinoma, giant renal tumor, renal cell carcinoma, sarcomatoid differentiation


How to cite this article:
Mehra K, Manikandan R, Dorairajan LN, Sreerekha J. A rare case of giant chromophobe renal cell carcinoma with sarcomatoid differentiation: Treatment and prognosis. J Can Res Ther 2021;17:602-5

How to cite this URL:
Mehra K, Manikandan R, Dorairajan LN, Sreerekha J. A rare case of giant chromophobe renal cell carcinoma with sarcomatoid differentiation: Treatment and prognosis. J Can Res Ther [serial online] 2021 [cited 2021 Sep 23];17:602-5. Available from: https://www.cancerjournal.net/text.asp?2021/17/2/602/318106




 > Introduction Top


Chromophobe renal cell carcinoma (CRCC) is a rare type of renal cell carcinoma (RCC) and constitutes only 5% of all RCC.[1] Sarcomatoid differentiation, an indicator of ominous prognosis in RCC, is also a rare transformation and occurs in 5% of all RCC. In CRCC subtype, sarcomatoid transformation occurs in 8% of cases.[2] Because of radiological advances, RCC can now be detected at an early stage. Renal tumors with >20 cm diameter are relatively uncommon.[1] Herein, we present a patient of giant CRCC with sarcomatoid differentiation, who underwent radical nephrectomy with complete resection. However, unfortunately, the patient presented with intra-abdominal relapse and succumbed to the disease within 3 months of the surgery.


 > Case Report Top


A 55-year-old female presented with complaints of left side abdominal pain with progressive weakness in doing regular activities of 20-day duration. She had a single episode of hematuria. She also noticed a mass in the left upper quadrant, which rapidly increased in size over a few days. She also had breathing difficulty on lying supine. On physical examination, there was a lump visible per abdomen [Figure 1]. The mass was palpable, occupying almost the whole of the abdomen. Contrast-enhanced computerized tomography (CT) scan showed a large heterogeneously enhancing tumor of dimensions 40 cm × 32 cm × 15 cm arising from the mid and lower pole of the left kidney. The tumor was encasing the descending colon, crossing the midline, and pushing the rest of the abdominal contents to the right side [Figure 2]. All laboratory parameters were within normal limits. A metastatic workup including CT thorax, serum lactic dehydrogenase, alkaline phosphatase, and serum calcium was within normal limits.
Figure 1: Preoperative image of mass per abdomen showing a huge bulge

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Figure 2: (a-d) Transverse sections with hetrogenous enhancement of the Renal mass (e) Coronal sections showing left giant Ranal mass

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Under general anesthesia, left radical nephrectomy along with left hemicolectomy was performed with transverse to sigmoid colo-colic anastomosis. Pre- and para-aortic lymphadenectomy was performed. The postoperative tumor size was 36 cm × 30 cm × 15 cm and weighed 5.1 kg [Figure 3]. Grossly, the cut surface of the tumor revealed a yellowish solid mass with irregular areas of necrosis and hemorrhage [Figure 4]. The tumor extended to the perirenal fat, which was densely adherent to the colon, making it pT4. Histopathologically it was a CRCC with sarcomatoid differentiation [Figure 5]. On immunohistochemical markers testing, the RCC component was positive for CD117 and negative for CD10, and sarcomatous component was positive for smooth muscle actin [Figure 5]. The patient had an uneventful postoperative recovery. After 3 months of surgery, the patient presented again with abdominal pain and weakness. The CT scan suggested recurrence in the left renal fossa along with liver metastasis [Figure 6]. Ultimately, the patient succumbed to the relapse in hospice.
Figure 3: Postoperative specimen of left radical nephrectomy. The size of the renal tumor was 36 cm

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Figure 4: Cut section of the preserved specimen of the renal tumor. Arrow shows colon which was invaded by tumor. Asterisk shows renal tumor

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Figure 5: (a) Chromophobe renal cell carcinoma (arrow) with sarcomatoid transformation (asterix); (b) chromophobe renal cell carcinoma areas showing nests of tumor cells separated by thin fibrovascular septa. (c) Sarcoma component arranged in long intersecting fascicles (H and E, ×200); (d) chromophobe renal cell carcinoma areas showing cells with distinct cell membrane, wrinkled nuceli, perinuclear halos, and abundant eosinophilic cytoplasm (H and E, ×200); (e) renal cell carcinoma component positive for CD117; (f) renal cell carcinoma component negative for CD10 and (g) sarcomatous component positive for smooth muscle actin (DAB, ×400)

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Figure 6: Computerized tomography scan abdomen after 3 months suggesting relapse and liver metastasis

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 > Discussion Top


CRCC is a rare tumor that constitutes about 5% of adult primary renal tumors.[3] CRCC occurs mostly in the sixth decade and has a better prognosis than the conventional RCC with 5-year survival rate of 80%–90%.[3] CRCC is more common in females (52%) than in males (48%).[1] Most cases of CRCC are diagnosed in Stage 1 or 2. Tumor thrombus is seen in 5% of cases and metastasis is seen in 6%–7% of cases, most common sites being liver and lung.[1] Factors that suggest poor prognosis are large tumor size, sarcomatoid differentiation, and tumor necrosis.[4] The sarcomatoid differentiation makes the prognosis in CRCC worse, decreasing the 10-year cancer-specific survival rate from 90% to 27%.[2] The size of the tumor also suggests the outcome of the tumor. Although there is no standard measurement of size and weight for designating a renal tumor as “Giant,” Caricato et al. first used the word “giant” for the large renal tumor measuring 40 cm in the case they reported.[5] Metastasis is more often encountered when the tumor size exceeds 10 cm.[6]

The pathologists report most of the cases in the literature with emphasis on the pathological description. A few have been reported with the urology point of view. Suzuki et al. reported a giant CRCC, which was 35 cm in the largest dimension and weighing more than 10 kg.[7] They reported a favorable outcome with no recurrence even after 20 months.

Turkan et al. also reported a giant CRCC with 28 cm in the largest dimension and about 2.6 kg in weight.[8] There was no local invasion or distant metastasis in their case. However, the follow-up status of the patient was not mentioned in their report.

The definitive treatment of a localized RCC is complete surgical resection. Based on the S-TRAC trial, in 2017, Food and Drug Administration approved immunotherapy tyrosine kinase inhibitor (TKI) sunitinib in nonmetastatic RCC as an adjuvant treatment that is after surgical resection.[9] The NCCN guideline 2020 suggests a clinical trial or sunitinib in Stage 4 cases that are not amenable for surgical resection.[9] There are still doubts regarding the reproducibility of clinical benefit with TKI, and in February 2018, European Medicines Agencies rejected the use of sunitinib in high-risk localized RCC.[9] A systematic review by Papanikolaou et al. in 2020 regarding systemic therapy for CRCC concluded that CRCC is resistant to immunotherapy.[10]

Our patient had a giant CRCC with dimensions of 36 cm × 30 cm × 15 cm, weighing 5.1 kg with no evidence of metastasis. She underwent en bloc complete resection of renal tumor along with hemicolectomy. However, she developed intra-abdominal relapse after 3 months with synchronous liver metastasis and finally succumbed to the disease. Large tumor size and sarcomatoid differentiation were poor prognostic factors in our patient.


 > Conclusion Top


CRCC has a favorable outcome. However, CRCC associated with large size and sarcomatoid differentiation may have a poor prognosis.

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 initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
Vera-Badillo FE, Conde E, Duran I. Chromophobe renal cell carcinoma: A review of an uncommon entity. Int J Urol 2012;19:894-900.  Back to cited text no. 1
    
2.
Safavi M, Dabiri S, Monsefi N. Sarcomatoid transrormation of chromophobe renal cell carcinoma. Iran J Pathol 2016;11:85-7.  Back to cited text no. 2
    
3.
Leibovich BC, Lohse CM, Crispen PL, Boorjian SA, Thompson RH, Blute ML, et al. Histological subtype is an independent predictor of outcome for patients with renal cell carcinoma. J Urol 2010;183:1309-15.  Back to cited text no. 3
    
4.
Amin MB, Paner GP, Alvarado-Cabrero I, Young AN, Stricker HJ, Lyles RH, et al. Chromophobe renal cell carcinoma: Histomorphologic characteristics and evaluation of conventional pathologic prognostic parameters in 145 cases. Am J Surg Pathol 2008;32:1822-34.  Back to cited text no. 4
    
5.
Caricato M, Valeri S, Ausania F, Caputo D, Rabitti C, Coppola R. Giant abdominal sarcoma. Colorectal Dis 2005;7:422-3.  Back to cited text no. 5
    
6.
Guillaume MP, Baldassarre S, Takeh H, da Costa PM. Localized renal cell carcinoma of an unusually large size: Case report. Acta Chir Belg 2003;103:321-3.  Back to cited text no. 6
    
7.
Suzuki K, Kubo T, Morita T. A giant chromophobe renal cell carcinoma exceeding 10 kg. Int J Urol 2009;16:976.  Back to cited text no. 7
    
8.
Turkan S, Kalkan M, Sener HB, Sahin C. Giant chromophobe renal cell carcinoma. J Case Rep 2015;5:5-7.  Back to cited text no. 8
    
9.
Ghali F, Patel SH, Derweesh IH. Current status of immunotherapy for localized and locally advanced renal cell carcinoma. J Oncol 2019;2019:7309205.  Back to cited text no. 9
    
10.
Papanikolaou D, Ioannidou P, Koukourikis P, Moysidis K, Meditskou S, Koutsoumparis D, et al. Systemic therapy for chromophobe renal cell carcinoma: A systematic review. Urol Oncol 2020;38:137-49.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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