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A l-week-old baby boy presented with hepatosplenomegaly, coarse facial features, and cloudy corneas. A metabolic storage disease was considered and he underwent cutaneous and liver biopsy. By light microscopy the skin was normal. Kupffer cells were enlarged and had foamy cytoplasm. Ultrastructural examination of skin and liver demonstrated features compatible with Farber's disease: curvilinear and "banana" bodies, zebra-like structures, and concentric lamellar bodies. A deficiency of lysosomal acid ceramidase was subsequently demonstrated in cultured fibroblasts and in liver tissue corroborating the ultrastructural findings.

Inflammatory pseudotumor is a tumoral non-neoplastic lesion of unknown etiology that has been described in several locations. We report the histopathological and immunohistochemical findings of this lesion in the cervix of a 58-year-old woman whose main complaint was pelvic pain.

We present the histological, ultrastructural, and immunohistochemical findings of two granular cell tumors of different histogenesis: a mediastinal granular cell schwannoma, and an uterine granular cell leiomyoma. Ultrastructurally the mediastinal tumor showed granular cell changes of the Schwann cells which were reactive for S-100 protein and Leu 7 antigen, but not for actin, desmin, CEA, EMA, or cytokeratin. Ultrastructural study of the uterine lesion demonstrated smooth muscle cells with only a few "autophagic" facuoles to cells nearly replaced by lysosomes. Immunohistochemically this tumor showed reactivity for actin, desmin, and Leu 7 antigen, but was S-100 protein, CEA, EMA, and cytokeratin negative.

A case is presented of a primitive neuroectodermal tumor located in the left frontal cerebrum, which demonstrated electron microscopic and immunocytochemical evidence of rhabdomyoblastic differentiation. Within the group of supratentorial primitive neuroectodermal tumors, only medulloepithelioma has previously been shown to manifest "divergent" differentiation. In several respects our case is analogous to infratentorial "medullomyoblastoma."

Seven cases of primary cerebral neuroectodermal tumors with predominant neuroblastic features were studied ultrastructurally and five were evaluated immunohistochemically. The fine structural features were indicative of neuroblastic differentiation by the presence of elongated cytoplasmic processes, electron-dense neurosecretory granules, and neurotubules. Five of the seven cases had the morphologic findings of classic cerebral neuroblastoma, and the sixth case, originally diagnosed as an oligodendroglioma, had the features of a differentiated neuroblastoma. Desmoplastic and/or stromal foci were intermingled with neuronal-ganglionic cells and neuroblasts in the seventh case. In addition to strong immunoreactivity for S-100 protein and glial fibrillary acidic protein in the desmoplastic areas, the spindle cells had fibroblastic and Schwannian features by electron microscopy in the latter case. The neuroblastic cells and fibrillary network were immunoreactive for neuron-specific eno-lase and neurofilament in the five study cases. It is concluded that cerebral neuroectodermal tumors may express an range of phenotypic features from the exclusive neuroblastic stage to a neuronal and stromogenic phase analogous to the classic neuroblastoma of the sympathetic nervous system.

The clinicopathologic features of two cases of dedifferentiated chondrosarcoma (DCS) are presented, in which anaplastic components showed the electron microscopic features of malignant fibrous histiocytoma, as well as immunoreactivity for alpha-1-antitrypsin and alpha-1-antichymotrypsin. Rare cells also displayed S100 protein in high-grade areas of the primary tumors, but a pulmonary metastasis lacked this determinant. These findings could be interpreted as reflecting a retained potential for primitive chondrogensis in primary DCS, which may be lost in its metastases. In all other respects, this tumor appears to assume the morphological and immunocytochemical attributes of a fibrohistiocytic neoplasm.