Overview
Myxoid pleomorphic liposarcoma is a rare cutaneous neoplasm that represents a histomorphological variant within a disease spectrum that also includes atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) 2. These tumors typically present as nonspecific, often ulcerated, nodules in sun-damaged skin of elderly patients 2.Diagnosis
Histologically characterized by spindle-shaped and epithelioid tumor cells with pleomorphic nuclei and atypical multinucleated giant cells, with common atypical mitoses 2.
Pleomorphic dermal sarcoma (PDS) is poorly circumscribed, infiltrative, involves the subcutis, and may show tumor necrosis and/or perineural infiltration, distinguishing it from AFX 2.
Immunohistochemical analysis is necessary to exclude other similar cutaneous sarcomas, undifferentiated carcinomas, and melanomas due to the poorly differentiated nature of AFX/PDS (Grade 3) 2.Management
The treatment of choice is micrographically controlled surgery 2.
Complete excision is assumed to result in a cure for AFX 2.
Local recurrence rates for PDS are low when surgically removed with a 2 cm safety margin 2.
Metastasis is rare and primarily associated with very thick or incompletely excised tumors, affecting the skin and lymph nodes 2.Special Populations
Patients older than 74 years and males have an increased risk of local AFX recurrence 1.
A more intensive follow-up regimen may be considered for patients older than 74 years and males due to higher recurrence risk 1.Key Recommendations
Surgically remove PDS tumors with a safety margin of 2 cm to achieve low local recurrence rates 2. (Evidence: Moderate)
Consider a more intensive follow-up regimen for patients older than 74 years and males due to their higher risk of local AFX recurrence 1. (Evidence: Moderate)
Micrographically controlled surgery is the preferred treatment for AFX and PDS 2. (Evidence: Moderate)References
1 Ørholt M, Aaberg FL, Abebe K, Walsh S, Roenigk RK, Venzo A et al.. Risk factors for local atypical fibroxanthoma recurrence and progression to pleomorphic dermal sarcoma: A meta-analysis of individualized participant data. Journal of surgical oncology 2022. link
2 Helbig D, Ziemer M, Dippel E, Erdmann M, Hillen U, Leiter U et al.. S1-guideline atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS). Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG 2022. link