- 2025, Volume 83 - Number 4
- Published: October 9, 2025
Atypical fibroxanthoma and pleomorphic dermal sarcoma: a 10-year retrospective study and review of the literature
J. Joana Vieitez-Frade 1, Dora Mancha 1, Pedro de Vasconcelos 2, Luís Soares-de-Almeida 3, Paulo L. Filipe 4
1 Department of Dermatology, Hospital de Santa Maria, Unidade Local de Saúde Santa Maria, Lisbon, Portugal; Dermatology University Clinic, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal; 2 Dermatology and Venereology Department, Hospital de Santa Maria, Unidade Local de Saúde Santa Maria, Lisbon, Portugal; 3 Dermatology and Venereology Department, Hospital de Santa Maria, Unidade Local de Saúde Santa Maria; Dermatology and Venereology University Clinic, Faculty of Medicine, University of Lisbon; Dermatology Research Unit, iMM João Lobo Antunes, University of Lisbon. Lisbon, Portugal; 4 Department of Dermatology and Venereology, Unidade Local de Saúde Santa Maria; Faculdade de Medicina da Universidade de Lisboa, Dermatology Clinic. Lisbon, Portugal
J. Joana Vieitez-Frade, Dora Mancha, Pedro de Vasconcelos, Luís Soares-de-Almeida, Paulo L. Filipe
La información completa de afiliaciones y autor de correspondencia está disponible en la versión original en PDF.
*Correspondence: Dora Mancha, Email not available
Abstract
Objectives: To characterize the demographic, clinical, histopathological, therapeutic, and prognostic features of patients diagnosed with atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) in a tertiary center. Methods: Retrospective observational study conducted from January 2014 to December 2023 at the Dermatology Department of Unidade Local de Saúde Santa Maria. Patients with histological diagnoses of AFX or PDS were included. Results: A total of 22 male patients, all over 65 years old, were included. Most tumors were located on the head (91%), measured < 2 cm (59%), and presented as non-specific plaques, nodules, or tumors, occasionally ulcerated. Histological criteria used to distinguish AFX (73%) from PDS (27%) included adipose tissue invasion (27%), neurotropism (5%), and necrosis (23%). All patients underwent initial surgical excision, five with positive margins. Three PDS patients received adjuvant radiotherapy (RT). One patient had a local recurrence, two patients developed regional metastases, and one patient developed a distant metastasis, all in PDS cases. No disease-related deaths were reported. Conclusions: AFX and PDS are rare cutaneous tumors with similar clinical presentations but distinct prognostic outcomes. Immunohistochemistry plays a critical role in differential diagnosis. Surgery remains the gold-standard treatment, while RT may benefit selected PDS cases. Further studies are needed to standardize therapeutic strategies and establish clear follow-up guidelines for these patients.
