Alveolar soft-part sarcoma: a review of the pathology and histogenesis

The descriptive designation, alveolar soft-part sarcoma, continues to be used for this uncommon soft-tissue malignancy because an acceptable hypothesis for its histogenesis has not been advanced, despite studies with electron microscopy and immunohistochemistry. These techniques have, nevertheless, provided significant information that is useful in the differential diagnosis of the tumor and pertinent in speculation concerning its nature. The most intriguing ultrastructural feature is the secretory process that culminates in the formation of the distinctive cytoplasmic crystals. Myogenic differentiation has been favored in a number of recent reports on the basis of immunohistochemical findings and the presence of the crystals does not rule out the possibility, but accounts of immunoreactivity for the myogenic regulatory protein MyoD1 have not been confirmed in subsequent studies or in the authors' own staining of six cases.