Angiomatoid Fibrous Histiocytoma: Epidemiological, Anatomopathological, Clinical Profile, Treatment, and Outcomes in a Series of 15 Cases

Abstract

Introduction: Angiomatoid fibrous histiocytoma (AFH) is a rare soft-tissue neoplasm with intermediate behavior, originally described as a lesion occurring in the subcutaneous tissue of the extremities in young individuals. Objective: To strengthen the availability of pathological data and improve the clinical approach to this disease by healthcare providers. Method: A retrospective study analyzing 15 cases treated between 2000 and 2023, involving a review of medical records and an anatomopathological re-examination of stored slides. Results: The mean follow-up period was 7.16 years, and patient ages ranged from 5 to 44 years, with a predominance of females under 20 years old. The lesions were predominantly located in the limbs (60%). Surgical resection was the treatment of choice and the compromise of surgical margins was associated with risk of relapse, which was observed in five cases (33.3%), two of which resulted in death (mortality rate of 13.3%). Six patients (40%) received adjuvant therapy, two underwent margin widening with no subsequent recurrence. Metastases occurred in 27% of the patients, primarily affecting the lungs and lymph nodes. Oncological discharge was achieved in 66.6% of the cases. Among the patients who died, the mean survival time was 5.8 years. Histologically, the presence of characteristic cells in a syncytial arrangement, a rim of inflammatory cells, and pseudoangiomatoid spaces, along with immunohistochemical positivity for desmin, proved to be essential diagnostic features. Conclusion: Given the predominance of the disease in the limbs of young patients, the study highlights the importance of wide resection with clear margins to achieve best clinical outcome.