A myogenic or a neural crest cell origin is suspected but is still a matter of debate and standard myogenic tissue markers, apart from desmin, have not been detected in this neoplasm.23-25 Alveolar soft-part sarcoma has the highest incidence of brain metastasis of all soft tissue sarcomas and metastasis may develop prior to primary tumour detection.16,17 Thus, for intracranial ASPS, any lesion detected extracranially would be regarded as the primary lesion. Here, DES is linked to alveolar soft part sarcoma.