Atypisches Fibroxanthom und pleomorphes dermales Sarkom

Ziemer, Mirjana; Jäger, Iris; Dippel, Edgar

doi:10.1007/s00105-019-04471-8

Cited by 9 publications

(13 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Interestingly, AFX accounted for almost half (49.3 %) of all cutaneous sarcomas, and if AFX/PDS are viewed as one spectrum of tumor entity they accounted for 61.1 % of all cutaneous sarcomas within the observation period. Nevertheless, the prevalence of AFX is still referred to as 0.24 % [13,35]. This study was performed by Anderson et al in 2007, long before further (immuno-)histological advances and improvements in terminology were made [13,32,41].…”

Section: Discussionmentioning

confidence: 99%

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

Kuntz

Siebdrath

Hofmann

et al. 2022

J Deutsche Derma Gesell

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 99%

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

Kuntz

Siebdrath

Hofmann

et al. 2022

J Deutsche Derma Gesell

View full text Add to dashboard Cite

show abstract

“…AFX and PDS are diagnoses of exclusion and differentiation from other primary cutaneous tumors arising on sun-exposed skin, such as squamous cell carcinoma, particularly the spindle cell subtype, melanoma, leiomyosarcoma and angiosarcoma, deserves exhaustive immunohistochemistry. AFX and PDS represent a spectrum of tumors with similar histopathological, immunophenotypical, and molecular features 9,15 . Ulceration is frequently observed in both entities and does not affect tumor behavior.…”

Section: Discussionmentioning

confidence: 99%

“…Recent studies found similar genetic alterations in the two neoplasms [6][7][8] . Both AFX and PDS are diagnoses of exclusion, to be made after careful immunohistochemical study, in order to not miss other neoplasms such as squamous cell carcinoma, melanoma, leiomyosarcoma or angiosarcoma 9 .…”

Section: Introductionmentioning

confidence: 99%

“…[6][7][8] Both AFX and PDS are diagnoses of exclusion, to be made after careful immunohistochemical study, in order to not miss other neoplasms such as squamous cell carcinoma, melanoma, leiomyosarcoma or angiosarcoma. 9 Apart from a few histopathologic criteria, 4 it is still unclear what really differentiates these two entities. Cases of metastatic AFX have been reported, [10][11][12][13] while, on the other hand, PDS indicates a category of tumors arising in the same clinical setting of AFX but showing more propensity to recur or metastasize.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Atypical fibroxanthoma and pleomorphic dermal sarcoma: A reappraisal

et al. 2020

View full text Add to dashboard Cite

This article is protected by copyright. All rights reserved. Background: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) share clinical, pathological, immunohistochemical and molecular features, though PDS is associated with a more aggressive behavior. Methods: We revised 71 tumors fulfilling criteria for AFX and PDS to further stratify their biological potential. Results: Lesions predominate on the scalp of elderly men, were frequently ulcerated, one case presented necrosis, one vascular, and another perineural invasion. Fifty-one tumors were limited to reticular dermis (71.8%), 20 invaded subcutaneous tissue, focally in 13 cases (18.3%), and diffusely in seven (9.9%). A significant more frequent subcutaneous invasion was observed in tumors showing predominantly spindle compared to pleomorphic/mixed cell morphology (p= 0.02). At a follow-up of 17-125 months, 4 cases recurred locally, 4, 6, 10 and 13 months after surgery; no metastases were observed. Three tumors were composed of spindle cells, and one of clear cells. Three cases had margins focally involved, the fourth case had clear margins. Conclusion: Depth of invasion and state of margins are criteria predicting prognosis in AFX/PDS; in addition, spindle cell morphology seems to be related to a more infiltrative pattern of growth and consequently aggressiveness. Grouping these tumors on a morphologic base could contribute to clarify their different biological behavior.

show abstract

“…Mittels neuer immunhistochemischer Marker erfolgte eine Neubewertung des malignen fibrösen Histiozytoms. Für die Tumore, die trotz Immunhistologie keiner spezifischen Entität zugeordnet werden können, wurde der Begriff "undifferenziertes pleomorphes Sarkom" (UPS) geprägt [2]. Die in der Haut lokalisierten UPS werden auch "pleomorphes dermales Sarkom" (PDS) genannt.…”

Section: Introductionunclassified

Atypisches Fibroxanthom oder pleomorphes dermales Sarkom – eine Frage der Infiltrationstiefe

Hasler

Beissert

Laske

2021

Aktuelle Dermatologie

View full text Add to dashboard Cite

ZusammenfassungDas atypische Fibroxanthom (AFX) ist eine seltene, niedrig-maligne mesenchymale Neoplasie der Haut. Die Abgrenzung zum malignen fibrösen Histiozytom bzw. pleomorphen dermalen Sarkom (PDS) wurde seit der Erstbeschreibung Anfang der 1960er-Jahre immer wieder kontrovers diskutiert. Mittlerweile wird der Begriff des pleomorphen dermalen Sarkoms für Tumore verwendet, die eine Infiltration der Subkutis, nekrotische Anteile oder eine perineurale oder lymphovaskuläre Infiltration aufweisen. Die Unterscheidung der beiden Entitäten erfolgt folglich vorwiegend histologisch, eine immunhistochemische Abgrenzung ist bisher nicht möglich.Wir berichten über 2 Patienten mit atypischem Fibroxanthom und pleomorphem dermalem Sarkom. Beide Tumorentitäten sollten mittels weiter Exzision oder mikrografisch kontrollierter Chirurgie vollständig exzidiert werden. Insbesondere beim pleomorphen dermalen Sarkom kommt es häufiger zu Rezidiven und Metastasierung, deshalb sollte der Sicherheitsabstand größer als beim AFX gewählt werden und v. a. in den ersten 3 Jahren nach der Behandlung eine engmaschige Nachsorge erfolgen.Eine einheitliche Klassifikation der beiden Tumorentitäten, die das aggressivere Verhalten des tiefer infiltrierenden pleomorphen dermalen Sarkoms berücksichtigt, wäre erstrebenswert.Zudem sollten beide Tumore der Meldepflicht unterliegen, um weitere epidemiologische Daten sammeln zu können.

show abstract

Atypisches Fibroxanthom und pleomorphes dermales Sarkom

Cited by 9 publications

References 21 publications

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

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

Atypical fibroxanthoma and pleomorphic dermal sarcoma: A reappraisal

Atypisches Fibroxanthom oder pleomorphes dermales Sarkom – eine Frage der Infiltrationstiefe

Contact Info

Product

Resources

About