Overview
Calcifying aponeurotic fibroma (CAF), also known as juvenile aponeurotic fibroma or Keasbey tumor, is a rare benign soft tissue tumor that arises from fibroblastic tissue, typically in association with aponeuroses, tendons, and fascial structures. It most commonly affects the hands and feet, particularly the palms and soles, though it can occasionally occur in other locations. The tumor is characterized histologically by the presence of fibroblastic proliferation with distinctive foci of calcification and cartilage-like areas, which help distinguish it from other fibromatoses. CAF predominantly affects children and adolescents, with most cases presenting before the age of 20, though it can also occur in adults. Patients typically notice a slowly growing, firm, painless or mildly tender nodule in the affected area. The mass is usually well-circumscribed but can infiltrate surrounding soft tissues, which contributes to a notable local recurrence rate after surgical excision. The tumor does not metastasize and is not considered malignant. The primary treatment for calcifying aponeurotic fibroma is surgical excision. Due to the infiltrative nature of the lesion, local recurrence rates have been reported in approximately 50% of cases, particularly in younger patients. Despite the tendency for recurrence, the prognosis is excellent as the tumor remains benign. Wide local excision with clear margins is recommended when feasible, though the location on hands and feet may limit the extent of resection to preserve function. Regular follow-up monitoring is advised to detect any recurrence early. No systemic therapies are typically required.
Also known as:
Sporadic
Usually appears on its own, not inherited from a parent
Childhood
Begins in childhood, roughly ages 1 to 12
Treatments
No FDA-approved treatments are currently listed for Calcifying aponeurotic fibroma.
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Specialists
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
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Common questions about Calcifying aponeurotic fibroma
What is Calcifying aponeurotic fibroma?
Calcifying aponeurotic fibroma (CAF), also known as juvenile aponeurotic fibroma or Keasbey tumor, is a rare benign soft tissue tumor that arises from fibroblastic tissue, typically in association with aponeuroses, tendons, and fascial structures. It most commonly affects the hands and feet, particularly the palms and soles, though it can occasionally occur in other locations. The tumor is characterized histologically by the presence of fibroblastic proliferation with distinctive foci of calcification and cartilage-like areas, which help distinguish it from other fibromatoses. CAF predominant
How is Calcifying aponeurotic fibroma inherited?
Calcifying aponeurotic fibroma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Calcifying aponeurotic fibroma typically begin?
Typical onset of Calcifying aponeurotic fibroma is childhood. Age of onset can vary across affected individuals.