S1‐guideline atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS)

Abstract

Summary Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are rare cutaneous neoplasms representing histomorphological, genetic as well as epigenetic variants of a disease spectrum. Both tumors typically manifest as nonspecific, often ulcerated, skin‐ to flesh‐colored nodules in chronically sun‐damaged skin of elderly male patients. AFX is a rather well demarcated, often rapidly growing tumor. PDS tumors are poorly circumscribed and are characterized by aggressive infiltrative growth. Fast as well as slow growth behavior has been reported for both tumors. Histologically, both are composed of spindle‐shaped and epithelioid tumor cells with pleomorphic nuclei as well as atypical multinucleated giant cells. Atypical mitoses are common. In contrast to AFX, PDS involves relevant parts of the subcutis and shows areas of tumor necrosis and/or perineural infiltration. Due to the poorly differentiated nature of AFX/PDS (Grade 3), histopathologically similar cutaneous sarcomas, undifferentiated carcinomas, melanomas and other diseases have to be excluded by immunohistochemical analysis. The treatment of choice is micrographically controlled surgery. In cases of AFX, a cure can be assumed after complete excision. Local recurrence rates are low as long as PDS tumors are surgically removed with a safety margin of 2 cm. Metastasis is rare and mostly associated with very thick or incompletely excised tumors; it mainly affects the skin and lymph nodes. Distant metastasis is even more rare. No approved and effective systemic therapy has been established.

Kutane Sarkome – nicht alle sind wirklich selten, die meisten „low risk“

“…Die Nachsorge des AFX/PDS muss individuell anhand einer Risikoabschätzung erfolgen, die geltende Leitlinie macht dazu einen Vorschlag mit 3–6-12-monatigen Intervallen über 5 Jahre je nach Ausgangssituation 14 .…”

“…Halbjährliche Inspektionen v. a. im Hinblick auf Lokalrezidive, ggf. am Primärort sonografisch unterstützt, werden für 5 Jahre empfohlen [14].…”

“…Beide Tumoren werden mit einer chirurgischen Exzision behandelt. Die geltende Leitlinie empfiehlt mindestens 0,5 cm mit MKC-Unterstützung für das AFX, möglichst 2 cm auch unter MKC für das PDS, freilich mit Respektierung von Patientenwünschen und anatomischen Limitationen [14]. Bei ausgedehntem Befund können strahlentherapeutische Maßnahmen einfließen [15,16].…”

Hemato-Oncological Diseases as Risk Factor for Recurrence or Metastasis of Pleomorphic Dermal Sarcoma

“…Although accurate incidence data do not exist, the incidence is increasing due to demographic changes. Therefore, up to now, they represent the most common sarcomas of the skin ( 1 , 2 ).…”

“…They typically occur in chronically light-exposed sites with a non-specific clinical presentation in the form of ulcerated or polypoid tumors ( 7 , 8 ). Histopathologically, both tumors are very poorly differentiated (“dedifferentiated”) atypical malignant skin neoplasms requiring the exclusion of other poorly differentiated skin tumors such as “dedifferentiated” cutaneous squamous cell carcinoma (cSCC), malignant melanomas, vascular tumors, and other sarcomas, as well as reticulohistiocytomas and atypical fibrous histiocytomas ( 1 , 2 , 8 – 11 ). Tumor cell morphology includes a variable spectrum of atypical spindle-shaped and epithelioid cells with pleomorphic, vesicular, or hyperchromatic nuclei, as well as atypical multinucleated giant cells and often atypical mitoses, which in the case of AFX remain confined to the dermis and in the case of PDS encompass significant portions of subcutaneous adipose tissue or other deeper structures.…”