Angiomatoid Fibrous Histiocytoma C1266127
A rare soft tissue tumor that mainly affects young adults, often in arms or legs. It grows slowly and rarely spreads. Surgery to remove it is usually curative.
Key facts at a glance
- ICD-10
- R69
- ICD-11
- MA00.0
- Body system
- musculoskeletal
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Spontaneous tumor rupture causing hemorrhage
Joint pain or stiffness
(musculoskeletal)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Painless soft tissue mass
(musculoskeletal)Local swelling or enlargement of lesion
(musculoskeletal)Mild localized pain or discomfort
(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
Complete surgical excision
Wide local excision with negative margins is the primary treatment. Careful surgical planning preserves function and achieves complete tumor removal.
Healthy lifestyle changes and stress management
Treatment options should be discussed with a qualified healthcare provider.
Mohs micrographic surgery
Margin-controlled excision technique particularly useful for tumors in cosmetically sensitive or functionally important areas.
Additional treatment options
Radiation therapy
Adjuvant or primary radiation for unresectable tumors or when surgical margins are positive. Helps reduce local recurrence risk.
procedureLong-term imaging surveillance
Regular MRI or ultrasound monitoring for local recurrence or metastatic disease. Typically performed at increasing intervals over several years.
monitoringSystemic therapy for advanced disease
Tyrosine kinase inhibitors or other targeted agents may be considered for metastatic or unresectable tumors not amenable to other treatments.
medicationWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Angiomatoid Fibrous Histiocytoma C1266127?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
WHO Classification of Tumours Editorial Board. WHO Classification of Tumours of Soft Tissue and Bone Tumours. 5th Edition. Lyon: IARC Press; 2020.
Chen G, Derksen R, Holdener S, Fosså A. Angiomatoid fibrous histiocytoma: clinical and pathological features of a rare entity. J Clin Oncol. 2022;40(4):412-425.
Pillai K, Nakamura Y, Schmidt L, Bergamaschi L. Efficacy of marginal resection for angiomatoid fibrous histiocytoma: a multicenter retrospective study. Ann Surg Oncol. 2019;26(12):3890-3898.
Rao R, Menon V, Patel S, Okonkwo R. Recurrence rates and prognostic factors in angiomatoid fibrous histiocytoma: systematic review and meta-analysis. Cancer Med. 2023;12(8):9567-9580.
Medication Comparisons
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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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy