Kaposi sarcoma is commonly seen in -
• DFSP is a low-grade sarcoma because it may recur locally but rarely metastasizes.
• Monomorphous, mononuclear, spindle cell lesion involving both dermis and subcutis.
• MC site: Trunk (50%) >Extremities (30%) >Head and neck (20%)
Pathology
• Large lesions often are associated with satellite nodules; Positive for CD34
• Have unpredictable radial extensions of tumor permeating through the subcutaneous tissue large distances from the primary nodule.
• More than 75% of DFSP have a ring chromosome, composed of translocated portions of chromosomes 17 and 22
Clinical Features
• Typically presents in early or mid-adult life, beginning as a nodular cutaneous mass.
• Pattern of growth: Slow and persistent
• Lesion enlarges over many years, it becomes protuberant
Treatment
• Aggressive resection with special attention to radial margins (local recurrence rate <5%)
• Up to 50% recur after simple excision.
• Imatinib: First line of treatment for advanced disease.
Upper limbs
Lower limbs
Head and Neck
Trunk