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Adverse Occurrence type:
Not discussed in European Council Guidelines. Frequency estimated at 1-3% of population (Wallace MJ, Wallace S. Renal angiomyolipoma. In: Adam A, Dondelinger RF, Mueller PR, eds. Interventional Radiology in Cancer. New York: Springer-Verlag; 2004:204).
Time to detection:
Known at transplant. 1 cm mass found in living donor kidney (mother) during evaluation but not resectable due to central location. After a nine months observation period to decrease risk of an underlying malignancy, decision to proceed with transplant was made. Inspection after cold perfusion still revealed no good possibility for ex vivo excision, kidney was implanted without further manipulation. Immediate functioning, recovery without complications. Followup imaging showed no growth with 18 month followup.
Alerting signals, symptoms, evidence of occurrence:
Detected pre-transplant in donor by ultrasound. No complications in recipient.
Demonstration of imputability or root cause:
Identification in donor kidney prior to transplant.
Suggest new keywords:
Known in donor
Fritsche L, Budde K, Rogalla P, Turk I, Neumayer H.-H, Loening SA. Successful living related kidney transplantation despite renal angiomyolipoma in situ. J Urol 162; 480-481, 1999.
I have added some text in the section "Time to detection". And added the last 3 keywords. Mike, please check if this is ok and corresponds to the way you proposed. Feel free to modify but please, let me know to learn for the next review. Thanks for all the pre-done work! Looks good. Mike
Expert comments for publication:
Angiomyolipoma is considered to be a benign tumor curative by resection. Reports of metastatic spread are rare and controversial; the question of metastases versus multicentricity is not conclusively resolved. However, rare highly atypical angiomyolipomas (highly pleomorphic, necrosis, mitotic activity on histologic examination) raise concern for atypical behavior. The authors note in the current report that a large study (Steiner MS et al., J Urol 150:1782, 1993) showed asymptomatic course for angiomyolipomas <4 cm. In followup discussion of this report (in J Urol 163; 924, 2000) the authors state that they were aware of "at least two other cases of rejection of a kidney allograft with angiomyolipoma" but considered that to be coincidental and noted a more likely risk of bleeding from the tumor which should be considered during decision making. It is estimated that 80% of angiomyolipomas are sporadic and 20% are associated with tuberous sclerosis (Steiner MS, et al. The natural history of renal angiomyolipoma. J Urol. 1993;150:1782-1786). Although considered a benign tumor, uncommon variants exist that are capable of aggressive or malignant behavior (reviewed in https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5644357/ )