Calcifying aponeurotic fibroma

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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.

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Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Calcifying aponeurotic fibroma.

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No actively recruiting trials found for Calcifying aponeurotic fibroma at this time.

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No specialists are currently listed for Calcifying aponeurotic fibroma.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA 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.