Kaposiform Hemangioendothelioma C1367420
A rare vascular tumor that typically occurs in infants and young children. It can cause a serious clotting disorder called Kasabach-Merritt phenomenon. Treatment may include medications, surgery, or chemotherapy.
Key facts at a glance
- ICD-10
- C80.1
- ICD-11
- 2A00.0
- Body system
- oncologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Kasabach-Merritt phenomenon (consumptive coagulopathy)
Ulceration of the lesion with bleeding
Generalized symptoms
(oncologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Violaceous infiltrative skin plaque
(integumentary)Swelling or mass in the affected region
(musculoskeletal)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
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
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
mTOR Inhibitor Therapy
Sirolimus (rapamycin) has emerged as an effective first-line medical therapy for kaposiform hemangioendothelioma, targeting the mTOR pathway to reduce tumor proliferation and resolve Kasabach-Merritt phenomenon.
Corticosteroid Therapy
High-dose corticosteroids serve as initial immunosuppressive therapy, particularly when Kasabach-Merritt phenomenon is present, helping to stabilize platelet counts and reduce vascular proliferation.
Additional treatment options
Percutaneous Embolization
Catheter-based embolization of feeding vessels can reduce tumor size and improve consumptive coagulopathy in selected cases with identifiable arterial supply.
procedureSurgical Resection
Complete surgical excision offers potential cure for well-demarcated, resectable tumors when vital structures are not compromised and can be achieved with acceptable morbidity.
surgicalVincristine Chemotherapy
Vincristine provides cytotoxic activity against proliferating endothelial cells and serves as an effective second-line agent when first-line therapies fail or are contraindicated.
medicationWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Kaposiform Hemangioendothelioma C1367420?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Kaposiform Hemangioendothelioma C1367420. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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