Juxta occipital epithelioid sarcoma

: A 24 years old female patient with a juxta-occipital epitheloid sarcoma is reported. The lesion, a slow growing assymptomatic nodule, started 5 years ago reaching 4 cm in diamter. It was deep seated in the cervical muscles but easy to surgically remove. No evidence of metastases or local recurrence was detected for the next six months in spite of the limited excision performed. Clinical and histological features of the tumor were reviewed and its differential diagnosis discussed, particularly with synovial sarcoma and clear cell sarcoma of tendons and aponeuroses. Histogenetic hypothesis were briefly considered and the relevant literature was reviewed.