Increase of atypical fibroxanthoma and pleomorphic dermal sarcoma: a retrospective analysis of four German skin cancer centers

Abstract

Summary Background and objectives In recent years, considerable insight has been gained into the pathogenesis, diagnosis and treatment of cutaneous sarcomas, including atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS). Both entities have shown increasing incidence rates in the last decade. This study was initiated to evaluate how these new insights impact the number of diagnoses of AFX/PDS compared to other cutaneous sarcoma entities. Patients and methods In a retrospective study of four German skin cancer centers, all histopathological reports of cutaneous sarcomas (AFX, PDS, dermatofibrosarcoma protuberans, cutaneous leiomyosarcoma, angiosarcoma, and Kaposi sarcoma) confirmed by board‐certified dermatopathologists were analyzed during a time‐period of seven years (2013–2019). Additionally, utilization of immunohistochemical markers (including pan‐cytokeratin, S100, desmin, CD34, CD10, procollagen‐1, CD99, CD14, and CD68) as an adjunct to diagnose AFX/PDS was recorded. Results Overall, 255 cutaneous sarcomas were included in the present study. The diagnosis of a cutaneous sarcoma has consequently risen from 2013 to 2019 (from 16 to 52 annual cases). The results of AFX/PDS revealed 4.6 times more diagnoses in 2019 than in 2013. Atypical fibroxanthoma represented the most common subtype, displaying 49.3 % of all diagnosed cutaneous sarcomas. Additionally, the increase of AFX/PDS was linked to the use of immunohistochemistry, with specific immunohistochemical markers used in 57.1 % of cases in 2013 compared to 100 % in 2019. Conclusions This retrospective study of four German skin cancer centers demonstrates a substantial rise of AFX/PDS, possibly due to recently established diagnostic and terminology standards. This rise is probably linked to increased utilization of specific immunohistochemical markers. Atypical fibroxanthoma/PDS may be more common than previously thought and seems to represent the most frequent cutaneous sarcoma subtype.

Hydrochlorothiazide and increased risk of atypical fibroxanthoma and pleomorphic dermal sarcoma

“…1 A continuous rise of AFX/PDS diagnoses has been observed in recent years, which may be due to various factors such as an aging society. 2 Evidence indicates that PDS is a more aggressive variant of AFX, and thus both tumors are currently seen as two variants of the same entity. [3][4][5] AFX/PDS can be challenging both clinically and histologically, and immunohistochemistry is essential for the correct diagnosis of AFX/PDS.…”

“…[3][4][5] AFX/PDS can be challenging both clinically and histologically, and immunohistochemistry is essential for the correct diagnosis of AFX/PDS. 2,[6][7][8][9] AFX/PDS usually affect elderly patients, most commonly with a mean age between 70-80 years. 10,11 Up to 90% of affected patients are male.…”

Hydrochlorothiazid: Erhöhtes Risiko für atypisches Fibroxanthom und pleomorphes dermales Sarkom

“…1 In den letzten Jahren wurde ein kontinuierlicher Anstieg von AFX und PDS beobachtet, was auf verschiedene Faktoren wie eine alternde Gesellschaft zurückzuführen sein könnte. 2 Vieles weist darauf hin, dass das PDS eine aggressivere Variante des AFX darstellt. Beide Tumoren werden aktuell als zwei Varianten derselben Entität angesehen.…”

Ist die Nomenklatur atypisches Fibroxanthom und pleomorphes dermales Sarkom noch zeitgemäß?

“…Die Arbeit von Kuntz et al [1]. spiegelt unsere alltägliche Beobachtung wider: Atypische Fibroxanthome (AFX) und pleomorphe dermale Sarkome (PDS) werden heute häufiger diagnostiziert.…”

“…Dies müsste jedoch noch an einem größeren und überregionalen Kollektiv bestätigt werden. Mögliche Ursachen hierfür werden von den Autoren diskutiert, wobei die Verbesserung in der Diagnosestellung als auch die älter werdende Bevölkerung zwei wichtige Faktoren sind [1]. Als weitere wichtige Einflussfaktoren auf die steigende Inzidenzrate des AFX und des PDS werden die frühere inkonsistente Nomenklatur und die nicht einheitlichen diagnostischen Kriterien angeführt [3, 4].…”