Auris Nasus Larynx 42 (2015) 469–471
Contents lists available at ScienceDirect
Auris Nasus Larynx journal homepage: www.elsevier.com/locate/anl
Atypical Fibroxanthoma: A case series and review of literature Sridhayan Mahalingam a,*, Aadarsh Shah b, Andrew Stewart a a b
Epsom and St Helier University Hospitals NHS Trust, Wrythe Lane, Carshalton SM5 1AA, United Kingdom North Middlesex University Hospital, Sterling Way, London N18 1QX, United Kingdom
A R T I C L E I N F O
A B S T R A C T
Article history: Received 18 November 2014 Accepted 5 April 2015 Available online 22 April 2015
Intro/objective: Atypical Fibroxanthoma (AFX) is a rare cutaneous neoplasm arising from myofibroblast or fibroblast-like cells that predominantly affects the head and neck region. It commonly mimics more invasive neoplasms and is a diagnostic challenge to clinicians. The aim of this study was to develop a better understanding of AFX, focusing on recent developments in diagnosis and management. Methods: A retrospective case series and review of recent literature were carried out. Results: Over a 17-year period, seven cases were identified (six male, mean age at presentation was 75.9 years). Two patients underwent complete excision and five patients had curettage and cauterisation. Two patients developed local recurrence but none demonstrated signs suggestive of metastatic spread. Histologically all seven lesions displayed a spindle cell pattern. Where performed, immunohistochemical staining was positive for Vimentin, CD10, CD68 and actin, and negative for CAM 5.2, CD34, Melan-A, S100 protein, HMB45, Cytokeratin A1/A3. Conclusion: Our patient demographics, histopathology and immunohistochemistry are comparable to previous studies. Although advances have been made in immunohistochemical analysis, we are yet to discover a specific diagnostic immunostain for AFX. Clinical findings should therefore be correlated with histology and a panel of immunohistochemical stains should be used. Given the potential for recurrence or metastases, Moh’s Micrographic Surgery with regular follow-up may be the preferred management. ß 2015 Elsevier Ireland Ltd. All rights reserved.
Keywords: Atypical Fibroxanthoma AFX
1. Introduction Atypical Fibroxanthoma (AFX) is a rare cutaneous neoplasm that predominantly affects the head and neck region. It was originally defined in 1961 as a dermal tumour composed of atypical spindle cells with a benign clinical course [1]. An accumulation of evidence in conjunction with recent advances in histopathology and immunohistochemistry has allowed us to develop a greater understanding of this condition over time. Contrary to initial beliefs, it is currently defined as a tumour of intermediate malignant potential arising from myofibroblast or fibroblast-like cells [2,3]. Previous studies have suggested that AFX has a greater male to female preponderance [4], with ultraviolet (UV) radiation thought to be the major risk factor [2]. Given its rarity and lack of differentiating factors from other potentially more aggressive cutaneous neoplasms, the clinical diagnosis is challenging and
* Corresponding author at: Epsom and St Helier University Hospitals NHS Trust, Wrythe Lane, Carshalton SM5 1AA, United Kingdom. Tel.: +44 02082962435; fax: +44 02082963233. E-mail address: [email protected] (S. Mahalingam). http://dx.doi.org/10.1016/j.anl.2015.04.001 0385-8146/ß 2015 Elsevier Ireland Ltd. All rights reserved.
often it is a diagnosis of exclusion. It commonly presents to dermatologists and plastic surgeons, however due to its preponderance for the head and neck it is often frequently encountered by Head and Neck Surgeons. Thus its clinical significance must be recognized within the specialty of Otolaryngology and Head and Neck Surgery. Here we present a case series of patients diagnosed with AFX and a review of recent literature in an attempt to develop a better understanding of this condition (Fig. 1). 2. Materials and methods A retrospective case series of all the patients diagnosed with AFX was carried out in one hospital site over a 17-year period. For each individual case, we reviewed patient demographics, key clinical features, histopathology, immunohistochemistry, treatment modalities and follow-up. Results were correlated with recent literature. 3. Results Seven cases of AFX were identified with a 6:1 male to female ratio (Table 1). All the patients were of Caucasian origin and mean
S. Mahalingam et al. / Auris Nasus Larynx 42 (2015) 469–471
470
suggest that curettage and cauterisation are appropriate for small (
Contents lists available at ScienceDirect
Auris Nasus Larynx journal homepage: www.elsevier.com/locate/anl
Atypical Fibroxanthoma: A case series and review of literature Sridhayan Mahalingam a,*, Aadarsh Shah b, Andrew Stewart a a b
Epsom and St Helier University Hospitals NHS Trust, Wrythe Lane, Carshalton SM5 1AA, United Kingdom North Middlesex University Hospital, Sterling Way, London N18 1QX, United Kingdom
A R T I C L E I N F O
A B S T R A C T
Article history: Received 18 November 2014 Accepted 5 April 2015 Available online 22 April 2015
Intro/objective: Atypical Fibroxanthoma (AFX) is a rare cutaneous neoplasm arising from myofibroblast or fibroblast-like cells that predominantly affects the head and neck region. It commonly mimics more invasive neoplasms and is a diagnostic challenge to clinicians. The aim of this study was to develop a better understanding of AFX, focusing on recent developments in diagnosis and management. Methods: A retrospective case series and review of recent literature were carried out. Results: Over a 17-year period, seven cases were identified (six male, mean age at presentation was 75.9 years). Two patients underwent complete excision and five patients had curettage and cauterisation. Two patients developed local recurrence but none demonstrated signs suggestive of metastatic spread. Histologically all seven lesions displayed a spindle cell pattern. Where performed, immunohistochemical staining was positive for Vimentin, CD10, CD68 and actin, and negative for CAM 5.2, CD34, Melan-A, S100 protein, HMB45, Cytokeratin A1/A3. Conclusion: Our patient demographics, histopathology and immunohistochemistry are comparable to previous studies. Although advances have been made in immunohistochemical analysis, we are yet to discover a specific diagnostic immunostain for AFX. Clinical findings should therefore be correlated with histology and a panel of immunohistochemical stains should be used. Given the potential for recurrence or metastases, Moh’s Micrographic Surgery with regular follow-up may be the preferred management. ß 2015 Elsevier Ireland Ltd. All rights reserved.
Keywords: Atypical Fibroxanthoma AFX
1. Introduction Atypical Fibroxanthoma (AFX) is a rare cutaneous neoplasm that predominantly affects the head and neck region. It was originally defined in 1961 as a dermal tumour composed of atypical spindle cells with a benign clinical course [1]. An accumulation of evidence in conjunction with recent advances in histopathology and immunohistochemistry has allowed us to develop a greater understanding of this condition over time. Contrary to initial beliefs, it is currently defined as a tumour of intermediate malignant potential arising from myofibroblast or fibroblast-like cells [2,3]. Previous studies have suggested that AFX has a greater male to female preponderance [4], with ultraviolet (UV) radiation thought to be the major risk factor [2]. Given its rarity and lack of differentiating factors from other potentially more aggressive cutaneous neoplasms, the clinical diagnosis is challenging and
* Corresponding author at: Epsom and St Helier University Hospitals NHS Trust, Wrythe Lane, Carshalton SM5 1AA, United Kingdom. Tel.: +44 02082962435; fax: +44 02082963233. E-mail address: [email protected] (S. Mahalingam). http://dx.doi.org/10.1016/j.anl.2015.04.001 0385-8146/ß 2015 Elsevier Ireland Ltd. All rights reserved.
often it is a diagnosis of exclusion. It commonly presents to dermatologists and plastic surgeons, however due to its preponderance for the head and neck it is often frequently encountered by Head and Neck Surgeons. Thus its clinical significance must be recognized within the specialty of Otolaryngology and Head and Neck Surgery. Here we present a case series of patients diagnosed with AFX and a review of recent literature in an attempt to develop a better understanding of this condition (Fig. 1). 2. Materials and methods A retrospective case series of all the patients diagnosed with AFX was carried out in one hospital site over a 17-year period. For each individual case, we reviewed patient demographics, key clinical features, histopathology, immunohistochemistry, treatment modalities and follow-up. Results were correlated with recent literature. 3. Results Seven cases of AFX were identified with a 6:1 male to female ratio (Table 1). All the patients were of Caucasian origin and mean
S. Mahalingam et al. / Auris Nasus Larynx 42 (2015) 469–471
470
suggest that curettage and cauterisation are appropriate for small (
