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Childhood Kidney Angiomyolipoma C1333000

Published: July 22, 2026Updated: August 20, 2026

A benign kidney tumor made of blood vessels, muscle, and fat. Rare in children, it may be associated with genetic conditions. Most cases are monitored, with surgery considered if tumors are large or symptomatic.

Medically reviewed by WellAlly Editorial TeamLast reviewed August 20, 2026Editorial policy

Key facts at a glance

ICD-10
C80.1
ICD-11
2A00.0
Body system
oncologic
Severity
moderate
Chronic
true

Signs and symptoms

Common signs and symptoms that may indicate this condition.

Generalized symptoms

(oncologic)

Noticeable symptoms that may vary in intensity over time.

Frequency: Common at all stages

Hematuria

(renal)

Flank pain

(renal)

Hypertension

(cardiovascular)

Palpable abdominal mass

(gastrointestinal)

Diagnosis and tests

How this condition is diagnosed and what test results mean.

Initial screening tests

1

Standard diagnostic workup

Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]

Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]

Treatment options

Evidence-based approaches to managing this condition.

First-line treatments

1

Active surveillance

Regular imaging surveillance for small asymptomatic angiomyolipomas (typically <4cm) with periodic ultrasound or MRI to monitor for growth.

monitoring
2

Exercise prescription and weight management

Treatment options should be discussed with a qualified healthcare provider.

lifestyle

Additional treatment options

mTOR inhibitor therapy

Everolimus or sirolimus therapy to shrink large angiomyolipomas (>4cm) or those at risk of hemorrhage, particularly useful in tuberous sclerosis.

medication

Selective embolization

Catheter-based arterial embolization to devascularize large or bleeding angiomyolipomas, preserving kidney function while reducing hemorrhage risk.

procedure

Partial nephrectomy

Kidney-sparing surgical resection of large angiomyolipomas when conservative measures fail or there is significant bleeding risk.

surgical

Dietary modifications

Low-fat diet and adequate hydration may help manage symptoms and support overall kidney health; avoid crash diets that stress renal function.

dietary

When to seek medical help

Recognize when you need immediate medical attention.

Schedule an appointment soon

Symptoms associated with this condition. Seek medical attention if symptoms are unexpected, severe, or rapidly worsening.- Contact your healthcare provider if symptoms persist or worsen. Seek emergency care for sudden severe symptoms.

Frequently asked questions

Common questions about this topic.

Q

What is Childhood Kidney Angiomyolipoma C1333000?

Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]

general

Sources and references

Authoritative medical sources used to create this content.

1

Krueger DA, Northrup H, Roberts P, et al. Tuberous sclerosis complex surveillance and management: recommendations of the 2018 International Tuberous Sclerosis Complex Consensus Group. Pediatr Neurol. 2021;119:50-65.

guideline2021
2

Sahney S, Weiss A, Thiele EA. Renal manifestations of tuberous sclerosis complex in children. Kidney Int Rep. 2020;5(8):1243-1251.

review2020
3

Bissler JJ, Kingswood JC, Radzikowska E, et al. Everolimus for renal angiomyolipoma in patients with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis: extension of the EXIST-2 trial. Lancet. 2019;394(10215):2155-2163.

clinical_trial2019
4

Alam NA, Bayliss KM, Curtis MJ. Pediatric renal tumors: contemporary diagnostic and therapeutic approaches. J Pediatr Urol. 2018;14(5):420-430.

review2018

Medication Comparisons

Compare medications used to treat Childhood Kidney Angiomyolipoma C1333000. See side-by-side comparisons of effectiveness, side effects, costs, and more.

View Medication Comparisons

This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.

If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.

Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy