Neuromas of the appendix. A light-microscopic, immunohistochemical and electron-microscopic study of 20 cases

The neural origin and even the existence of appendiceal neuromas have been questioned. We have studied 20 examples, 7 discovered during a prospective examination of 26 consecutive routine appendectomy specimens (for an incidence of 27%), 2 selected from random cases, and 11 discovered in a retrospective review of 11 randomly selected cases of appendices diagnosed as "fibrous obliteration." By light-microscopy, appendiceal neuromas appear as a loose proliferation of spindle cells usually in a myxoid background, frequently with entrapped fat and connective tissue and infiltrated by eosinophils. Seventeen were located centrally in the appendix without nodule formation. One was central with nodularity and two were confined to the mucosa. The spindle cells were positive for S-100 protein and neuron-specific enolase in all cases. In 12, serotonin positive cells entrapped in the proliferation were present. In 5 of 11 cases with apparent uninvolved appendix present in the specimen, the number of serotonin cells in the crypts was greater than in normal appendix controls. Two appendiceal neuromas contained somatostatin positive cells. Stains for vasoactive intestinal polypeptide, substance P, neurotensin, bombesin and gastrin were negative. Ultrastructural examination of one case confirmed the presence of a mixture of Schwann cells and cells containing neurosecretory granules. We conclude that appendiceal neuroma is a rather common entity, and that most cases of so-called fibrous obliteration actually represent appendiceal neuroma.