Status:
Ready to upload
Record number:
2106
Adverse Occurrence type:
MPHO Type:
Estimated frequency:
Malignant rhabdoid tumor of the kidney is a pediatric mesenchymal neoplasm, i.e., a sarcoma. Therefore the guidelines for typical renal cell carcinoma do not apply.
Most recent risk assessment for sarcoma (Council of Europe, 2018): Donors with active sarcoma are considered to represent an unacceptable risk for organ donation regardless of disease stage. Donors with a history of sarcoma are also generally considered to represent unacceptable risks for transmission. After curative therapy and recurrence free survival of more than five years, they are still assumed to be associated with a high risk for transmission. Gastrointestinal stromal tumors are not grouped with other sarcomas and are considered separately.
Time to detection:
4 months posttransplant
Alerting signals, symptoms, evidence of occurrence:
Hematuria and graft dysfunction led to ultrasound and CT scan showing 7.3 cm mass in kidney allograft
Demonstration of imputability or root cause:
Identification of donor origin by DNA analysis (fluorescent in situ hybridization of XY chromosomes and short tandem repeat analysis) of tumor cells.
Imputability grade:
3 Definite/Certain/Proven
Groups audience:
Keywords:
Suggest new keywords:
Case report
Malignancy
deceased donor
kidney transplant
FISH
Sex chromosomes
DNA typing
Renal cancer, other
CNS tumor, other or type not specified
Astrocytomas and glioblastomas not further specified (WHO grade 1-4)
Sarcoma, other or type not specified
Therapy
Suggest references:
Xiong J, Su T, Zhu P, Ao Q, Ruan Q, Wang G. Malignant rhabdoid tumor in the renal allograft of an adult transplant recipient: a unique case of a rare tumor. Diagn Pathol. 2017;12(1):86.
Note:
Please add category of harm to recipient->malignancy->kidney and urinary tract->renal cancer, other (non renal cell carcinoma)--> OK (EP)
Done MN 5/6/21
Very interesting case. KL concurs 5/5/22
Expert comments for publication:
Malignant rhabdoid tumor is an aggressive pediatric neoplasm with poor prognosis. This 47 year old patient received 2 kidneys from a 56 day old infant who died with a CNS tumor suspected to be astrocytoma (never biopsied). Pretransplant imaging showed no renal mass. The authors suspect but admittedly cannot prove that the CNS tumor was actually an atypical teratoid/rhabdoid tumor that metastasized to the donor kidney and grew posttransplant. Their conclusion is that a CNS tumor in a young infant should not just be assumed to be an astrocytoma, and vigilance for other possibilities should be maintained. Despite the aggressiveness of the tumor, the recipient was alive and without evidence of tumor 10 months following removal of the affected donor kidney (the contralateral donor kidney was not removed).