We present an autopsy case of a 29-week-old male fetus with a very unusual set of congenital
granular cell tumors, including gingival
epulis and granular cell lesions of the kidneys, lung, heart, esophagus, small and large intestine, thyroid, adrenals, spleen, urinary bladder, testis, pituitary, and leptomeninges. The granular cells were distributed mainly through the stroma of the organs, but they also involved the epithelial lining of the seminiferous and renal tubules. Ultrastructurally, the gingival, pulmonary, and renal
tumors were basically the same in appearance as membrane-bound heterogeneous bodies. Immunohistochemical studies were negative for
S100 protein,
lysozyme,
alpha 1-antitrypsin,
cytokeratin, and
vimentin in the gingival mass as well as in other systemic lesions. The immunohistochemical reaction pattern of the granular cells in our case was more like the cells of the congenital granular cell
epulis rather than adult
granular cell tumor because of its negative reaction to
S100 protein. However, the involvement pattern was that of the adult form of
granular cell tumor. Several developmentally different cells, such as renal tubular epithelial cells, seminiferous tubular cells, gingival stromal cells, and parenchymal cells of many organs, were involved in this granular cell process. The myofibroblastlike cells seen in the region of segmental dysplasia of the kidney showed the same cytoplasmic material as in typical granular cells. Based on these findings, it is suggested that a histogenesis of multiple cell origin of the
granular cell tumor could be strongly supported.