|Year : 2022 | Volume
| Issue : 1 | Page : 25-27
Giant clear cell renal cell carcinoma: An extremely rare and surgically challenging tumor
Prem Kumar1, Deepak Kumar Gupta2, Pankaj Kumar1, Somesh Janoria1, Pranjal Prem3
1 Department of Urology, Ranchi Urology Centre, Wardha, Nagpur, Maharashtra, India
2 Department of Cardiology and Interventional Cardiology, Pulse Super Speciality Hospital, Ranchi, Jharkhand, India
3 J.L.N Medical College and Hospital, Wardha, Nagpur, Maharashtra, India
|Date of Submission||15-Jan-2021|
|Date of Decision||06-Aug-2021|
|Date of Acceptance||13-Sep-2021|
|Date of Web Publication||20-Apr-2022|
Ranchi Urology Centre, Buxi Compound, Behind Shyam Petrol Pump, Bariatu, Ranchi - 834 009, Jharkhand India.
Source of Support: None, Conflict of Interest: None
Renal cell carcinoma is the most common renal cancer occurring in the sixth and seventh decades of life. Most of the renal cell carcinomas are detected incidentally at an early stage. It is rare to find a giant renal cell carcinoma. Most of the giant renal cell carcinomas reported were of chromophobe or sarcomatoid variety. Giant renal clear cell carcinoma is extremely rare. In this case report, we are presenting the largest giant renal clear cell carcinoma ever reported from the Indian subcontinent.
Keywords: Angioembolization, clear cell variant, giant renal cell carcinoma, radical nephrectomy
|How to cite this article:|
Kumar P, Gupta DK, Kumar P, Janoria S, Prem P. Giant clear cell renal cell carcinoma: An extremely rare and surgically challenging tumor. Oncol J India 2022;6:25-7
|How to cite this URL:|
Kumar P, Gupta DK, Kumar P, Janoria S, Prem P. Giant clear cell renal cell carcinoma: An extremely rare and surgically challenging tumor. Oncol J India [serial online] 2022 [cited 2022 May 27];6:25-7. Available from: https://www.ojionline.org/text.asp?2022/6/1/25/343570
| Introduction|| |
In the current era of imaging, renal cell carcinomas are usually detected as small renal masses. Giant renal cell carcinoma is rarely reported due to its slow growth rate and increased use of imaging techniques. It is very rare to encounter a giant renal clear cell carcinoma exceeding 20 cm in size.
Here, we report a surgically challenging giant renal clear cell carcinoma for its extreme rarity.
| Case Report|| |
A 60-year-old male was referred to our center with a history of bilateral lower-limb swelling, anorexia, and breathlessness on exertion for 2 months. He had a history of left flank fullness for 6 months. He had few episodes of hematuria 10 years ago which he neglected. He had no other relevant history. He had no underlying comorbidities. Physical examination revealed pallor, bilateral pedal edema, and palpable left flank mass.
His biochemical investigation revealed hemoglobin – 7 g/dl, alkaline phosphatase – 324 IU/L, serum creatinine – 2.0 mg/dl, serum protein – 5.6 g/dl, serum albumin – 2.8 g/dl, and serum globulin – 2.8 g/dl. Other biochemical parameters were within normal limits. Noncontrast computed tomography of the abdomen and chest [Figure 1]a and [Figure 1]b revealed a large ill-defined heterogeneous renal mass on the left side measuring 23.1 cm × 11.1 cm, involving all the poles and the renal pelvis with extension to the left renal vein. There were multiple foci of calcifications. Anteriorly, the mass was abutting the pancreas and posteromedially the left psoas muscle with loss of fat plane. Multiple prominent lymph nodes (largest size 10 mm) were seen at the left hilar, left para-aortic, preaortic, precaval, and retrocaval regions. The right kidney was normal in size, shape, and position. There was no evidence of liver or lung metastasis.
|Figure 1: Noncontrast computed tomography abdomen showing large left heterogeneous renal mass on axial cut (a) and coronal section (b)|
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After preoperative workup and optimization with blood transfusions, chest physiotherapy, and parenteral nutrition, the patient underwent preoperative left renal artery angioembolization for devascularization of large renal mass. Left radical nephrectomy was done 48 h later under general anesthesia. Transperitoneal approach was taken with midline incision from xiphisternum to below umbilicus. There was a dense desmoplastic reaction around Gerota's fascia. The left renal mass was measuring approximately 25 cm × 12 cm [Figure 2] with extension up to the diaphragm superiorly and inferiorly into the pelvis. There were engorged peritumoral venous plexuses. Medially, the tumor was extending up to the aorta. The left renal vein had type I tumor thrombus which was delivered with the specimen. There was profuse intraoperative bleeding. The approximate blood loss was 1500 ml. The patient received 4 units of whole blood and 4 units of fresh frozen plasma perioperatively.
Examination of the operated specimen revealed a tumor size of 25 cm × 12 cm and the weight of the tumor was 1.1 kg [Figure 3].
Grossly, the cut surface was a gray-white solid cystic tumor almost entirely replacing the entire renal parenchyma. The histopathological diagnosis [Figure 4]a and [Figure 4]b was clear cell type renal cell carcinoma with Grade 2 Fuhrman nuclear grading with renal vein extension. Pathologically, the tumor was pT3a.
|Figure 4: (a) H and E stain ×100 section shows tumor cells arranged in nests separated by thin vasculature, and (b) H and E stain ×400 section shows nests of tumor cells with clear cytoplasm and distinct cell membrane|
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The patient's recovery was delayed due to poor preoperative general condition. The patient was given chest and limb physiotherapy along with parenteral nutritional support. The patient was discharged on postoperative day 11.
The patient was planned for targeted therapy in adjuvant setting after consultation with a medical oncologist, but he died 3 weeks after the surgery due to unrelated cause.
| Discussion|| |
Giant renal cell carcinoma has been defined as renal mass exceeding 20 cm in size and volume of more than 1000 cc., It is extremely rare due to its indolent course with a growth rate of 0.06–0.39 cm annually and widely available imaging facilities. After extensive literature search, we found the world's largest renal malignancy reported recently in 2021 by Takeda et al. of 13.0 kg and the type of pathology is papillary type 1 with a tumor size of 43 cm × 42 cm.
Most of the reported cases were of chromophobe or sarcomatoid type., Till date, only four cases of giant clear cell renal cell carcinoma have been reported.,,, [Table 1] shows the comprehensive analysis of reported cases in the literature of giant clear cell RCC.
|Table 1: Reported cases of giant renal cell carcinoma of clear cell variant|
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Guillaume et al. reported the first and the largest size localized (pT2) clear cell renal cell carcinoma measuring 31 cm × 31 cm × 10 cm. It was very unusual for renal mass of this size to be localized. The patient successfully underwent surgery followed by remains disease free at 2 years of follow-up. Moslemi et al. in their report on huge clear cell RCC found the absence of venous extension or distant metastasis, and the patient was under obligate observation for 9 years after its primary diagnosis. Radical nephrectomy was done which was surgically difficult with severe bleeding. They support that large renal tumor can be managed expectantly for those unfit/refused for surgery but with increased vigilance and delayed intervention may not adversely impact pathological outcome. Although disease progression is higher in patients with larger renal tumors (>4 cm), tumor growth kinetics may identify the aggressive behavior of renal tumors.
Whereas, Chueh et al. reported a large clear cell RCC with renal vein thrombosis, and multiple hepatic and pulmonary metastases. Radical nephrectomy was performed with intraoperatively multiple regional unresectable lymph nodes, and there was profound bleeding with a total blood loss of 8000 ml. Targeted therapy with sunitinib was started after the surgery. There was rapid growth of metastases during follow-up, and the patient survived for 6 months only. Prognosis was very poor even after radical nephrectomy and targeted therapy.
Pramod et al. reported the largest giant clear cell RCC (25 cm × 22 cm × 18 cm) from Asia with a total volume of 9.900 cm3. Cytoreductive nephrectomy was performed but with positive surgical margin on histopathological examination. They reported no patient complaint during 1-year follow-up even without targeted therapy.
In our case, the patient neglected the first symptom of hematuria 10 years ago and presented to us with a huge left renal mass (25 cm × 12 cm) and poor general condition. The renal mass was huge, but there was no distant metastasis. After preoperative workup and optimization, left renal artery angioembolization was done to reduce the vascularity of huge renal mass.
The benefits of renal artery angioembolization preoperatively include a decreased blood loss intraoperatively, the creation of a tissue plane of edema facilitating dissection, and reduction in tumor bulk including the extent of vascular thrombus. Renal artery angioembolization is a safe and effective technique that is well tolerated with few complications, particularly if the time interval from embolization to surgery is reduced to less than 48 h.
The patient was taken up for left radical nephrectomy 48 h later. Transperitoneal approach was taken to get better vascular control. Surgery was very challenging as there were dense desmoplastic reactions around Gerota's fascia. There were dense adhesions with peritoneum and pancreas. Mass was extending from the diaphragm till the pelvis. There were engorged venous plexuses all around. The mass was dissected with great difficulty with profuse bleeding. The total blood loss was around 1500 ml despite preoperative angioembolization.
Our patient expired 3 weeks after the surgery due to unrelated disease which could not be evaluated.
| Conclusion|| |
Giant clear cell RCCs are extremely rare, they are difficult to operate, and there could be significant blood loss. Preoperative angioembolization can help in reducing blood loss. They have poor prognosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]