Abstract
Siblings were examined for multiple large tumors on various parts of the body accompanied by whitish nodules, hypertrophic gingiva, and flexural contracture of the large joints of the extremities. The tumor cells were embedded in an amorphous ground substance which was definitely PAS-positive and resistant to amylase digestion. Electron microscopic and tissue culture studies showed that the ground substance was produced by the tumor cells. The cells had intracytoplasmic granules which seemed to be secreted. We tried to establish a clinical entity together with nine similar cases described in the literature.
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