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DEPARTMENT OF MEDICAL ONCOLOGY GCRI, AHMEDABAD |
Renal cell carcinoma (RCC) affects more than 150,000 people annually worldwide, resulting in 78,000 deaths each year. Most RCCs are unilateral, with Bilateral RCC accounting for only 1.4% of all cases. They can either be synchronous or metachronous, familial or non-familial. This is a brief discussion on three different cases of Bilateral RCC highlighting the challenges in its management followed by the review of literature on Bilateral RCC. |
| Case Report: 1 |
| A 51 year old male presented with complaints of weight loss and fever for two months. He was a known case of Hypertension for 2 years and Diabetes for 2 months and was on treatment for both. His CTscan showed –both kidneys enlarged ,Left kidney was malrotated and showed mild hydronephrosis, lobulated 14.9x10.2cms mass lesion was seen arising from upper pole of left kidney another 6x5cms lesion showing heterogenous enhancement was seen in lower pole of right kidney ,few small similar lesions were seen in right upper pole, left lower pole and upper pole. Multiple pulmonary nodules were seen with minimal right pleural effusion. It also revealed thyroid nodules. CT impression was Multicentric RCC involving both kidneys with lung and possible thyroid metastases. USG guided biopsy from renal and thyroid nodules showed renal cell carcinoma with clear cell morphology. This patient was stated on palliative vinblastine 10 mg every 15 days. |
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| Case Report: 2 |
A 56 year old Male patient was diagnosed as having Bilateral clear cell renal cell carcinoma in April 2004. Left Radical Nephrectomy was done May 2004 and Right Partial Nephrectomy on July 2004. Recurrence happened in the Right Kidney after 6 months i.e January, 2005 together with lung secondaries. He was started on cytokine therapy but did not tolerate Interlukine-2 and progressed within 6 months on Interferon and Thalidomide . From July 07 he was started on Sorafenib and after 1 year due to disease progression Sunitinib was started. Sunitinib was stopped within 6 months and once again he was started on Sorafenib due to increase in size of primary mass and lung lesions. He is on Sorafenib for the last 3 months. PET CT does not show any lung mets now however the tumor size has increased from 2cm to almost 8-9 cm in past 4 years.
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| Case Report: 3 |
| A 56, Male, K/C/O Hypothyroidism was diagnosed as having CRF in March 2007. Serum creatinine was 3 mg% .His sonogram at that time showed a mass at the upper pole of left kidney 56x57 mm size with atrophic right kidney. Biopsy from the left kidney lesion revealed Renal cell carcinoma. Stage was T 2 N 0 M 0 . His performance status was ECOG-0. Patient did not take any treatment at that time for RCC ,and was started on medications for CRF. Since last 15 days he has developed C/O abdominal pain for which CT-scan was done which showed well defined lesion in upper pole of left kidney, enlarged mid & lower Para aortic nodes , right kidney is atrophic. His renal functions showed a serum creatinine level of 3.1mg% and hemoglobin was 8.6 g/dl. Stage T 2 N 2 M 0. with a performance status ECOG-0. Patient was counseled regarding his disease status and treatment options including surgery, chemotherapy and targeted therapies. He was started on palliative chemotherapy in the form of Inj Vinblastine 10 mg every 15 days . |
| Discussion: |
Renal cell carcinoma accounts for approximately 3% of adult malignancies and 90-95% of neoplasms arising from the kidney. The incidence of bilateral renal cell carcinoma is about 1.4%. RCC accounts for approximately 1% of all cases presenting to GCRI. |
Bilateral RCC can be either familial or nonfamilial. At least 4 hereditary syndromes associated with renal cell carcinoma are recognized: (1) von Hippel-Lindau (VHL) syndrome, (2) hereditary papillary renal carcinoma (HPRC), (3) familial renal oncocytoma (FRO) associated with Birt-Hogg-Dube syndrome (BHDS), and (4) hereditary renal carcinoma (HRC). |
An analysis of database of 698 consecutive patients undergoing operation for RCC between July 1989 and December 1997 at Memorial Sloan-Kettering Cancer Center identified that Thirty-three of 698 (4.7%) patients operated for RCC had bilateral disease. Four of the 33 (12.1%) patients had either VHL or documented hereditary RCC, and 29 of 33 (87.9%) had non-familial RCC. 1 Our patients did not have any family history of RCC. |
Bilateral RCCs are most commonly of clear cell morphology followed by papillary morphology. In a series of 153 patients with bilateral RCC published in BJUI, 76% patients had clear cell morphology and 19% had papillary morphology. 2 Our patients had clear cell morphology. Most common tumor metastasizing to thyroid gland is RCC followed by lung carcinoma. 3 Usually, thyroid metastases are considered a preterminal event. |
Long-term data support the concept of Nephron Sparing Surgery (NSS), whenever possible it is the treatment of choice for bilateral synchronous sporadic RCC. NSS provides adequate local tumour control and cancer-specific survival. Preservation of renal function is more efficient with bilateral NSS than with unilateral NSS and contralateral Radical Nephrectomy 4 .At 5 and 10 years, the cancer-specific survival rates were 86% and 75%, and the local recurrence-free survival rates were 87% and 80%. The median serum creatinine level at follow-up was 1.18 mg/dL in patients after bilateral NSS and 1.40 mg/dL after unilateral NSS and contralateral RN . |
Metastatic RCC remains one of the most resistant cancers. Conventional therapy has little to offer. RCTs show less than 10% response rate to conventional Chemotherapy (Platinum and Vinblastine). |
There is currently no role for chemotherapy alone in metastatic RCC . Cytokine-based immunotherapy is at present the first-line systemic treatment, although only a small subset of patients is likely to benefit from treatment and toxicity is significant. Targeted therapies using recombinant monoclonal antibodies and RTK inhibitors offer new treatment options with limited toxicity and reasonable response rates. Since RCC tumour proliferation is often driven by stimulation of multiple RTK pathways, it seems that substances that simultaneously inhibit multiple RTKs are more efficient than single pathway blockers. Un for tunately, the clinical response to the substances used so far is not permanent, with a time to progression of 6–12 months. This makes further research which combines inhibitors against various pathways or possibly adds cytokine-based immunotherapy necessary. |
We are thankful to our respected Director Dr. Pankaj Shah Sir for his continuous encouragement and guidance. |
| References: |
1. Bilateral non-familial renal cell carcinoma-G. Grimaldi, V. Reuter and P. Russo Annals of Surgical Oncology, Vol 5, Issue 6, 548-552. |
2. Clinicopathological features and prognosis of synchronous bilateral renal cell carcinoma: an international multicentre experience: Tobias Klatte, Heiko Wunderlich BJU International, Volume 100 Issue 1, Pages 21 – 24 |
| 3. Metastasis to the thyroid gland : A report of 43 cases Nakhjavani M. K. ; Gharib H.; Goellner J. R. ; Van Heerden J. A. Cancer 1997, vol. 79, pp. 574-578 (28 ref.) |
| 4. Department of Urology, Johannes Gutenberg University, Mainz, Allemagne. |
5. Medical treatment options in patients with metastatic renal cell carcinoma A. Haferkamp and M. Hohenfellner, Department of Urology, University of Heidelberg, Germany. |
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