Myxoma is not a single entity: a review of the concept of myxoma

Abstract

Myxoid lesions can be subdivided into (1) mainstream myxomas of soft tissues, (2) mainstream myxomas located outside the soft tissue, (3) inadequately substantiated myxomas, (4) myxoid soft tissue tumors or lesions not regarded as myxomas, (5) myxoid fatty conditions, (6) other soft tissue lesions and tumors that are sometimes markedly myxoid, (7) other soft tissue tumors in which myxoid foci may be seen, and (8) nonneoplastic myxoid conditions of soft tissue. More than 60 such conditions are listed and the five entities regarded as mainstream soft tissue myxomas (namely, intramuscular myxoma, juxta-articular myxoma, superficial angiomyxoma, aggressive angiomyxoma, and myxoid neurothekeoma [myxoma of nerve sheath]) are reviewed in detail. Intramuscular myxoma is exclusively intramuscular, usually affects middle-aged women, is most commonly located in the thigh, and does not recur after simple excision. Multiple intramuscular myxomas are rare and are usually associated with monostotic or polyostotic fibrous dysplasia and Albright's syndrome. Juxta-articular myxoma histologically resembles an intramuscular myxoma, but involves periarticular tendons, ligaments, joint capsules, muscles, and even the subcutis of adults. It may be associated with osteoarthritis of the adjacent joint. Some 30% recur locally. Superficial angiomyxoma also has been called cutaneous myxoma. It affects all ages, with a peak incidence in the third and fourth decades; arises in the trunk, lower limb, head, and neck regions; and usually measures less than 5 cm in diameter. Epithelial components are present in approximately 25% of tumors. Approximately one third recur locally, but there have been no metastases. Patients with multiple lesions may have the Carney complex. Aggressive angiomyxoma usually arises in the pelvic and perineal regions and affects females seven times as often as males. Tumors usually measure 10 cm or more in diameter, invade surrounding tissues, and recur in approximately 50% of cases. None have metastasized. Myxoma of nerve sheath (the myxoid variant of neurothekeoma) preferentially affects the dermis and subcutis of the cervicofacial areas and shoulders of young women. Most patients are younger than 40 years; one third of them are in the second decade of life. The majority of tumors measure between 0.5 and 1.5 cm. Only three of 102 cases compiled from the two largest published series recurred; none metastasized. The different clinicopathologic features and behavior of these five mainstream myxomas indicate that myxoma is not a single entity.