[2] AMSF tumors commonly develop in the subcutaneous tissues of the arms or legs of adults with an equal incidence (~1 per million individuals[2]) in males and females.
[6] The variable microscopic appearances of AMSF tumors have made them difficult to correctly diagnose in many cases.
[4] AMSF lesions are treated by surgical resection with the goal to remove all tumor tissue in order to reduce local recurrences.
In extreme cases, a combination of radiation therapy with surgical resection or amputation of an involved appendage has been used to treat these tumors.
[10] Individuals often re-present with a recurrence of their tumor at the site of its previous surgical removal or, in rare cases, present with metastatic disease.
MyxoFS tumors more often occur in a proximal rather than acral location, consist of more prominent capillary vessels, and lack inflammatory and VRS-like cells.
MyxLpS tumors consist of a monotonous cell population arranged in discrete myxoid-cellular clusters with conspicuous thin branching capillaries in a "chicken-wire pattern".
[14][15] Other lesions that frequently present in acral areas of the extremities such as hemosiderotic fibrolipomatous tumor,[16] epithelioid sarcoma, synovial sarcoma, acral fibromyxoma, giant cell tumor of tendon sheath, and clear cell sarcoma are usually distinguished from AMSF based on their clinical presentations, gross pathologies, histopathologies, and/or neoplastic cell expressions of marker proteins, abnormal chromosomes, and/or abnormal genes.
[5] Whenever possible, the first-choice treatment of AMSF tumors is surgical resection with wide margins in order to remove all neoplastic tissue.
[2] This lesion was first described in 1998 independently in three publications which named the disorder "acral myxoinflammatory fibroblastic sarcoma",[19] "inflammatory myxoid tumor of the soft parts with bizarre giant cells",[20] and "inflammatory myxohyaline tumor of distal extremities with virocyte or Reed-Sternberg-like cells".