Status:
Ready to upload
Record number:
1891
Adverse Occurrence type:
MPHO Type:
Estimated frequency:
Sarcomatoid renal cell carcinoma is a rare entity, counting for about 1% of all RCC. It is not explicitly described in the following Council of Europe recommendations. The transmission risk of this specific tumor might be increased (compared to clear cell or papillary RCC) due to its naturally aggressive behaviour.
(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:
7 months
Alerting signals, symptoms, evidence of occurrence:
68-year-old male donor without any suspicion for malignancy transmitted tumor to 2 recipients:
Kidney recipient 1 (66 year-old, female): obstructive uropathy one month after transplant (due to lymphocele). Recurrence after 3 months (due to ureterovesical stenosis). 3 months later admission with fever, dyspnea, orthopnea, edema, decreased diuresis, loose stools but negative urine cultures. Ultrasound showed calyceal ectasia with perigraft and perihepatic fluid. Descending pyelography revealed filiform ureter stenosis which was treated by nephrostomy. The fever persisted, kidney function deteriorated and after 18 days MRI showed perigraft fluid. Gallium scan showed the kidney to be the source of Inflammation and graft nephrectomy 24 days after admission revealed sarcomatoid RCC. 2 weeks later, laparotomy showed metastases in bladder, intestine and peritoneum as well as an abscess in the pouch of douglas. The patient died 48 hours later.
Kidney recipient 2: Diagnostics performed after detection of derived/transmitted RCC in contralateral kidney recipient #1 of the same donor. MRI confirmed 3 tumors (3.5cm, 1.7cm, 1cm) in renal graft. During subsequent nephrectomy, a <1cm peritoneal nodule was also removed. Afterwards, PET-CT showed malignant metabolic activity in the right iliac lymph nodes which led to removal of the locoregional lymph nodes. No histology is reported regarding this tumor, no further therapy is described. Patient was alive and tumor-free on peritoneal dialysis 18 months later.
Demonstration of imputability or root cause:
FISH of kidney tumor of female recipient 1 showed Y-chromosome, confirming male donor origin.
No imputability diagnostics is described for male recipient 2.
Imputability grade:
3 Definite/Certain/Proven
Groups audience:
Keywords:
Suggest new keywords:
case report
kidney transplant
FISH (fluorescence in situ hybridization)
kidney and urinary tract
renal cell carcinoma
deceased donor
renal cell carcinoma, sarcomatoid
Reference attachment:
Suggest references:
Llamas F, Gallego E, Salinas A, Virseda J, Pérez J, Ortega A, et al. Sarcomatoid renal cell carcinoma in a renal transplant recipient. Transplantation Proceedings. 2009;41(10):4422-4.
Note:
First review done on 2018-09-09 (Kerstin)
Since we have more papers dealing with sarcomatoid RCC, we might want to add a keyword that describes this specific diagnosis, e.g. "renal cell carcinoma, sarcomatoid"?
9/11/18: Agree- added keyword; Mike
Expert comments for publication:
Slight inconsistencies in described dates and time intervals make the paper a little confusing. Additionally, no histological result is described for kidney recipient 2 and there is no saying that he had a sarcomatoid RCC as well. The authors describe him to have "another" renal tumor without specification. This limits the value of the paper which nevertheless describes at least the confirmed donor-derived/transmitted sarcomatoid RCC in kidney recipient 1. But it has to be read with some caution not to misinterpret the findings.
Concluding, there is a certain suspicion for tumor transmission from donor to recipient due to the short time of detection in both kidneys after transplant. But the tumors in the 2 kidney recipients might well be different tumors and might have developed de novo after transplantation in the grafts (donor-derived). This appears unlikely given the short time interval and dissemination in recipient #1. Unfortunately there is no description about the condition of possible other organ recipients of the same donor.