Factors associated with the number and size of renal angiomyolipomas in sporadic angiomyolipoma (sAML): a study of adult patients with sAML managed in a Dutch tertiary referral center.
PURPOSE
To describe the patient characteristics, treatments, disease monitoring, and kidney function of patients with sporadic angiomyolipoma (sAML), stratified by the number and size of renal angiomyolipomas (AMLs).


METHODS
Single-center retrospective analysis of patients with sAML treated from 1990 to 2015 in a dedicated clinic for inheritable tumor syndromes in a tertiary referral center from the Netherlands. Patients' first AML assessment at the clinic was defined as the index date. Patient characteristics were measured at the index date. Treatments, disease monitoring, and kidney function were measured post-index date.


RESULTS
The study sample included 53 patients followed for a total of 184.6 patient-years. At the index date, the largest AML was ≥ 3.5 cm for 26 patients and < 3.5 cm for 27 patients (including six patients with five or more AMLs of < 3.5 cm). As compared to patients with AMLs < 3.5 cm, patients with largest AML ≥ 3.5 cm had higher frequency of pre-index bleeding episodes (31 vs. 4%), pre-index hypertension (35 vs. 15%), post-index nephrectomy (19 vs. 4%), post-index embolization (8 vs. 0%), and post-index renal scans (1.14 vs. 0.74 scans/year). Kidney impairment was especially pronounced in young adults with AML ≥ 3.5 cm. On average, patients with sAML developed chronic kidney disease stage two earlier than the general Dutch population (age 42 vs. 55 years), but later than the patients with tuberous sclerosis complex (35 years).


CONCLUSIONS
Patients with sAML, especially those with larger AMLs, have high disease burden.
['Ruud Bosch|J L H|JLH|', 'Vekeman|Francis|F|', 'Duh|Mei Sheng|MS|', 'Neary|Maureen|M|', 'Magestro|Matthew|M|', 'Fortier|Jonathan|J|', 'Karner|Paul|P|', 'Ionescu-Ittu|Raluca|R|', 'Zonnenberg|Bernard A|BA|http://orcid.org/0000-0003-0103-1979']
D000328:Adult D000367:Age Factors D000368:Aged D018207:Angiomyolipoma D004621:Embolization, Therapeutic D005260:Female D005919:Glomerular Filtration Rate D006470:Hemorrhage D006801:Humans D006973:Hypertension D007680:Kidney Neoplasms D008297:Male D008875:Middle Aged D009378:Neoplasms, Multiple Primary D009392:Nephrectomy D009426:Netherlands D051436:Renal Insufficiency, Chronic D012189:Retrospective Studies D062606:Tertiary Care Centers D014057:Tomography, X-Ray Computed D047368:Tumor Burden D014463:Ultrasonography
Angiomyolipoma Treatment Disease monitoring Kidney function