Case report: Renal cell carcinoma

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Record number: 
1915
Adverse Occurrence type: 
MPHO Type: 
Estimated frequency: 
(Council of Europe, 2022): To provide valid histological staging, complete tumour resection (R0) is required for acceptance of all organs; additionally, tumour-free margins are a prerequisite for transplant of the affected kidney. Paraffin section is superior to frozen section for the assessment of such biopsies. The contralateral kidney should always be examined for synchronous RCC (5 % of patients). RCC < 1 cm (stage T1a AJCC 8th edn) and WHO/ISUP grade I/II (Fuhrman grade I/II) can be considered minimal-risk for transmission; RCC 1-4 cm (stage T1a AJCC 8th edn) and WHO/ISUP grade I/II (Fuhrman grade I/II) are considered low-risk; RCC > 4-7 cm (stage T1b AJCC 8th edn) and WHO/ISUP grade I/II (Fuhrman grade I/II) are considered intermediate-risk; RCC > 7 cm (stage T2 AJCC 8th edn) and WHO/ISUP grade I/II (Fuhrman grade I/II) are considered high-risk; RCC with extension beyond the kidney (stages T3/T4 AJCC 8th edn) is considered a contraindication to transplant; All RCC with WHO/ISUP grade III/IV (Fuhrman grade III/IV) are considered high-risk for transmission; Contralateral kidneys and other organs that are un¬involved in carcinoma are considered to represent minimal risk for transplantation when the RCC in the involved kidney is 4 cm or less and WHO/ISUP grade I-II. In all cases, follow-up surveillance is desirable. RCC in the donor history: The transmission risk of treated RCC depends on the histological type of tumour [159] and its recurrence-free follow-up period. In general, in the first 5 years after initial diagnosis, risk categories correspond to those stated above (RCC diagnosed during donor procurement) if there is no suspicion of tumour recurrence in the donor. After this time, the risk of advanced stages may decrease.
Time to detection: 
8 years + 3 months
Alerting signals, symptoms, evidence of occurrence: 
2cm Bosniak 4 cystic mass detected on the upper graft pole. Biopsy showed clear cell RCC (T1aN0M0, Fuhrman 3 / Stage I). Treatment by percutaneous cryoablation to preserve the graft and modification of the immunosuppressive therapy. Biannual ultrasound was uneventful for the next 3 years + 6 months. Then, a 6cm upper pole renal mass was detected. Additional neck pain and abdominal discomfort led to a PET/CT scan which revealed metastases in liver and bones. Tumor staging (T1bN1M1, Fuhrman 3 / stage IV) was updated after confirming biopsy. 2-step-therapy: 1. withdrawal of immunosuppression and start of pegylated interferon alpha-2a to stimulate the immune system and induce tumor rejection. The renal tumor was supposed to serve as a trigger for alloimmune antitumor response and nephrectomy was therefore postponed. During the next 3 months, graft function deteriorated, donor-specific antibodies appeared and finally graft rejection was seen. 2. Transplant nephrectomy of the necrotic graft was performed which showed only sparse microscopic areas of residual RCC. Interferon therapy was continued (total duration 8 months) and the metastatic lesions dissapeared in the following months. Three years later, the patient remained in complete remission.
Demonstration of imputability or root cause: 
XY-FISH analysis demonstrated donor origin by showing XY signals (male donor / female recipient)
Imputability grade: 
3 Definite/Certain/Proven
Groups audience: 
Suggest new keywords: 
malignancy
kidney transplant
FISH
kidney and urinary tract
renal cell carcinoma
rcc
case report
Suggest references: 
Champion L, Culine S, Desgranchamps F, Benali K, Verine J, Daugas E. Metastatic Renal Cell Carcinoma in a Renal Allograft: A Sustained Complete Remission After Stimulated Rejection. Am J Transplant. 2017;17(4):1125-8.
Note: 
First review done on Nov 18, 2018 (Kerstin) ATTENTION: duplicate of record 1897 (EP) Second Review done Carl-Ludwig
Expert comments for publication: 
The unique approach of a delayed transplant nephrectomy for the purpose of using the tumor as a trigger for an intensified immune response was very successful in this single case. It might be worth to be taken into account for future cases but must be very well reflected and should be discussed with the tumor board and with the patient upfront. The authors discuss different strategies for the management of tumor transmission with metastatic spread, e.g. monoclonal antibodies as checkpoint inhibitors which certainly will be a topic for further evaluation in the future.