Article
Oncology
Johannes Zeller, Jurij Kiefer, David Braig, Oscar Winninger, Daniel Kraus, Salome Hagelstein, Steffen U. Eisenhardt
Summary: Atypical fibroxanthoma and pleomorphic dermal sarcoma are rare tumors with similar clinical and histologic features but distinct prognoses. This study analyzed the treatment outcomes of 32 patients and found that PDS patients exhibit a more aggressive growth pattern and require extensive resections for local control.
Article
Dermatology
Mathias Orholt, Kiya Abebe, Louise E. Rasmussen, Frederik L. Aaberg, Laerke J. Lindskov, Grethe Schmidt, Anne Lene Wagenblast, Michael M. Petersen, Anand C. Loya, Soren Daugaard, Mikkel Herly, David Hebbelstrup Jensen, Peter Vester-Glowinski
Summary: A standardized follow-up program for patients with atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) based on nationwide long-term estimates of local recurrence and metastasis in Denmark was recommended. Clinical visits for 4 years can be limited for AFX patients, while PDS patients should have clinical visits and PET/CT twice a year for the first 3 years and once a year for a minimum of 1 year.
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
(2023)
Article
Dermatology
Thomas Kuntz, Julian Siebdrath, Silke C. Hofmann, Mehmet Baltaci, Joerg Schaller, Martin Hellmich, Luise Stach von Goltzheim, Chalid Assaf, Frank Oellig, Alena-Lioba Michalowitz, Doris Helbig, Alexander Kreuter
Summary: In recent years, there has been a significant increase in the diagnosis of atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS), possibly due to changes in diagnostic and terminology standards. The use of immunohistochemical markers has also been linked to the rise of AFX/PDS. AFX/PDS may be more common than previously thought and represents the most frequent subtype of cutaneous sarcoma.
JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT
(2022)
Article
Dermatology
Anna Maria Cesinaro, Graziana Gallo, Sabrina Tramontozzi, Mario Migaldi
Summary: The study reviewed 71 tumors meeting criteria for AFX and PDS, finding them mainly located on the scalps of elderly men with some lesions invading surrounding tissues. Spindle cell morphology appears to be associated with a more invasive growth pattern and aggressiveness.
JOURNAL OF CUTANEOUS PATHOLOGY
(2021)
Article
Dermatology
Iris Marie Anders, Carolin Schimmelpfennig, Karolin Wiedemann, Dennis Loeffler, Christoph Kaempf, Conny Blumert, Kristin Reiche, Manfred Kunz, Ulf Anderegg, Jan-Christoph Simon, Mirjana Ziemer
Summary: The histogenetic origin of atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) is still unclear. This study found strong clinical, histomorphological, and molecular genetic similarities between AFX/PDS and undifferentiated cutaneous squamous cell carcinoma (cSCC), suggesting a common origin. The RNA transcriptome analysis revealed distinct clustering of AFX/PDS and differentiated cSCC, but undifferentiated cSCCs fell between the two groups without forming their own cluster.
JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT
(2023)
Article
Dermatology
Iris Marie Anders, Carolin Schimmelpfennig, Karolin Wiedemann, Dennis Loeffler, Christoph Kaempf, Conny Blumert, Kristin Reiche, Manfred Kunz, Ulf Anderegg, Jan-Christoph Simon, Mirjana Ziemer
Summary: This study investigates the histogenetic origin of atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS). Through clinical, histomorphological, and immunohistochemical evaluations, similarities between AFX/PDS and undifferentiated cutaneous squamous cell carcinoma (cSCC) were discovered. RNA transcriptome analysis showed that AFX/PDS and differentiated cSCC formed their own clusters, while undifferentiated cSCCs fell in between. Differential gene expression analysis identified undifferentiated cSCC cases that were more likely to be AFX/PDS based on their expression profile. These findings provide evidence for molecular similarities and a common histogenetic origin between AFX/PDS and undifferentiated cSCC.
JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT
(2023)
Article
Genetics & Heredity
Melike Ak, Abdullah Kahraman, Fabian M. Arnold, Patrick Turko, Mitchell P. Levesque, Martin Zoche, Egle Ramelyte, Reinhard Dummer
Summary: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are rare skin tumors with similar clinicopathological features but different prognoses. Diagnosis by exclusion using immunohistochemistry is common in clinical practice, emphasizing the importance of stringent diagnostic criteria and correct differentiation for optimal outcomes in management and treatment. Genomic analysis using next-generation sequencing (NGS) reveals potential gene alterations that could differentiate AFX and PDS, highlighting the need for deep tumor profiling for further insight.
Article
Dermatology
Doris Helbig, Mirjana Ziemer, Edgar Dippel, Michael Erdmann, Uwe Hillen, Ulrike Leiter, Thomas Mentzel, Georg Osterhoff, Selma Ugurel, Jochen Utikal, Dagmar von Bubnoff, Carsten Weishaupt, Stephan Grabbe
Summary: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are rare cutaneous neoplasms with distinct histological and genetic characteristics. They typically present as ulcerated nodules in chronically sun-damaged skin of elderly male patients. Micrographically controlled surgery is the preferred treatment option. Distant metastasis is rare, and there is currently no approved systemic therapy.
JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT
(2022)
Article
Oncology
Doris Helbig
Summary: Immunosuppression appears to be a risk factor for developing atypical fibroxanthoma and pleomorphic dermal sarcoma with worse clinical outcomes, especially in patients with underlying hemato-oncological diseases.
FRONTIERS IN ONCOLOGY
(2022)
Article
Dermatology
Maryam Aghighi, Aleodor A. Andea, Rajiv M. Patel, Douglas R. Fullen, Scott C. Bresler
Summary: This case presents a diagnostic dilemma that was ultimately diagnosed as a benign CD34-positive pleomorphic spindle cell tumor with features overlapping between spindle cell/pleomorphic lipoma and trichodiscoma.
AMERICAN JOURNAL OF DERMATOPATHOLOGY
(2022)
Review
Dermatology
Atieh Jibbe, Brandon Worley, Corinne H. Miller, Murad Alam
Summary: This study aimed to determine appropriate surgical wide local excision margins for atypical fibroxanthoma (AFX) and cutaneous undifferentiated pleomorphic sarcoma (cUPS). Probabilistic modeling based on case-level data showed that a margin of at least 2 cm for AFX and 3 cm for cUPS is required to clear 95% of all tumors. Smaller tumors 1 cm or less can be treated with a more conservative margin.
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
(2022)
Article
Pathology
Dominique Van Midden, Uta E. Flucke, Avital L. Amir, Johannes J. Bonenkamp, Satish F. K. Lubeek, Willeke A. M. Blokx
Summary: This study reveals that the histopathological distinction between AFX and PDS is still challenging, and several cases required reclassification upon review. Recurrence is significantly more likely when the tumor infiltrates the subcutaneous fat or when the tumor diameter exceeds 2 cm.
ANNALS OF DIAGNOSTIC PATHOLOGY
(2022)
Review
Dermatology
Mathias Orholt, Kiya Abebe, Frederik Aaberg, Louise Eggers Rasmussen, Soren Daugaard, Anand Chainsukh Loya, Mikkel Herly, Peter Viktor Vester-Glowinski
Summary: Through studying the suggested immunomarkers in the literature, we found that CD10 is a useful positive immunomarker for differentiating AFX and PDS. Additionally, no significant differences were observed in the immunohistochemical profiles between AFX and PDS.
AMERICAN JOURNAL OF DERMATOPATHOLOGY
(2022)
Article
Dermatology
Thomas Kuntz, Julian Siebdrath, Silke C. Hofmann, Mehmet Baltaci, Joerg Schaller, Martin Hellmich, Luise Stach von Goltzheim, Chalid Assaf, Frank Oellig, Alena-Lioba Michalowitz, Doris Helbig, Alexander Kreuter
Summary: This study retrospectively analyzed data from four German skin cancer centers and found a significant increase in the number of diagnosed cutaneous sarcomas over the past seven years, with atypical fibroxanthoma/PDS being the most common subtype. The use of specific immunohistochemical markers may be associated with the increase in diagnoses.
JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT
(2022)
Review
Dermatology
Jose Coelho-Lima, Jonathan Bruty, James Watkins, Hongxiang Liu, Richard Price, Niki Stefanos
Summary: Clear cell variants of atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are rare cutaneous tumors that primarily occur in sun-damaged skin of elderly individuals. This study presents two cases of clear cell AFX and PDS, highlighting key histopathologic findings and molecular alterations assessed by next-generation sequencing.
JOURNAL OF CUTANEOUS PATHOLOGY
(2022)