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Related Concept Videos

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Related Experiment Video

Updated: Jan 20, 2026

The In ovo CAM-assay as a Xenograft Model for Sarcoma
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[Atypical fibroxanthoma and pleomorphic dermal sarcoma].

M Ziemer1, I M Jäger2, E Dippel3

  • 1Klinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Leipzig AöR, Philipp-Rosenthal-Str. 23, 04103, Leipzig, Deutschland. mirjana.ziemer@medizin.uni-leipzig.de.

Der Hautarzt; Zeitschrift Fur Dermatologie, Venerologie, Und Verwandte Gebiete
|August 31, 2019
PubMed
Summary

Atypical fibroxanthoma (AFX) and undifferentiated pleomorphic sarcoma (UPS) are rare skin cancers. Current classification remains challenging, often relying on diagnosis by exclusion due to overlapping features.

Keywords:
Diagnosis by exclusionHistologyMalignant fibrous histiocytomaMolecular pathologyNeoplasm

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Related Experiment Videos

Last Updated: Jan 20, 2026

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Area of Science:

  • Dermatology
  • Oncology
  • Pathology

Background:

  • Atypical fibroxanthoma (AFX), also known as undifferentiated pleomorphic sarcoma (UPS), is a rare malignant skin neoplasm.
  • Initially described as an independent entity and superficial variant of malignant fibrous histiocytoma (MFH), its classification has been debated due to misdiagnoses of dedifferentiated neoplasms.
  • Deep extension, vascular/nerve invasion, and tumor necrosis are risk factors for recurrence in UPS.

Purpose of the Study:

  • To clarify the classification and diagnostic challenges of Atypical Fibroxanthoma (AFX) / Undifferentiated Pleomorphic Sarcoma (UPS).
  • To review the current understanding of AFX/UPS, including clinical, histological, and molecular pathological aspects.

Main Methods:

  • Review of existing literature on AFX/UPS classification and diagnosis.
  • Analysis of clinical and histological features associated with AFX/UPS.
  • Examination of molecular pathological studies aiming for improved classification.

Main Results:

  • AFX and UPS are considered a single disease entity based on clinical and histological features.
  • Despite extensive research, no specific histopathological or molecular pathological characteristic definitively identifies AFX/UPS.
  • The diagnosis of AFX/UPS remains primarily one of exclusion, based on morphology and immunohistochemistry.

Conclusions:

  • AFX and UPS should be viewed as a unified disease entity.
  • Current diagnostic approaches for AFX/UPS rely heavily on excluding other diagnoses.
  • Further research into specific molecular markers is needed for a more definitive classification.