Aim To better characterise the Calcifying Aponeurotic Fibroma (CAF) in children and adolescents.
Methods A systematic review of the English literature about CAF in patients under 18 was performed. A total of 97 cases were identified and four new cases from our personal series were added.
Demographic data collected included: age and gender, anatomical location and side of involvement, age at surgery, duration of follow-up, presence of recurrences and the interval between surgery and recurrence.
For study purposes the patients were divided in 4 groups according to age of presentation.
Results The age of presentation was mainly between 1 and 14 years, with a clear male preponderance (72%). The main location was the hand, but 30% were found in “atypical” locations.
The recurrence rate among those having follow-up was 45%, being higher in the younger age group.
No relationship between recurrence and patient gender or tumour location was found.
Conclusion The present review suggests that the age of presentation is earlier than previously reported (11–12 years old), as 79% of the cases were patients younger than 10 years.
The hand is the most common location. The tumour was initially described in the palms and soles of children. However, the foot trails far behind, and is involved in a similar frequency as the knee and the lower back.
As previously suggested, we found an association between the presentation age and the risk of recurrence, with the recurrence being higher in the younger age group, but the age/recurrence correlation was not lineal.
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