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Ten cases of tuberous sclerosis involving the central nervous system (CNS) in children aged 2 days to 15 years were studied. The abnormal cells found in subependymal, cortical, and white matter lesions were examined by light and electron microscopy. Histochemistry and immunohistochemistry were also employed. The results were similar in all lesions. Approximately one-third of the abnormal cells were positive by glial fibrillary acidic protein (GFAP), one-half by Nissl, and one-quarter by Holzer's stains. The intensity and pattern of GFAP staining varied from cell to cell and could not be predicted before staining. Ultrastructurally the cytoplasm of abnormal cells contained moderate to large numbers of 9- to 12-nm diameter fibrils and frequent dense bodies with crystalline appearance. Stacked rough endoplasmic reticulum was variable. Cell junctions and glycogen were rare. Nuclei were usually vesicular with a prominent nucleolus. Individual cells of tuberous sclerosis have features of both neurons and astrocytes. The disease may affect cells before differentiation. The predominant abnormal features of the cells in tuberous sclerosis are a great increase in fibrils and the presence of dense bodies, which may be a nonspecific reaction or result from a metabolic defect affecting the cells.

A 34-year-old man presented with tumor of his cervical spinal meninges and bone and the dura over the right frontal lobe, which was shown on biopsy to be Hodgkin's lymphoma. Extensive noninvasive evaluation failed to reveal any other sites of disease. Central nervous system involvement with Hodgkin's disease is extremely rare and is almost always a late complication in patients who have widely disseminated disease in the usual nodal sites. The current patient is unique in that his initial symptoms were due to involvement of the central nervous system and he had no evidence of Hodgkin's disease elsewhere.Cancer 581745 Case ReportIn March 1984 a 34-year-old white man presented to Forbes Regional Health Center with a chief complaint of upper extremity weakness and severe radicular pain from the cervical spine over the preceding year. This was accompanied by a greater than 20-pound weight loss and progressive fatigue. Physical examination revealed point tenderness over the C3-C4 region without a palpable mass. There was partial paresis of both upper extremities with the right worse than the left. There were no deficits of Accepted for publication February 14, 1986. the lower extremities. Bowel and bladder function were normal. He had no palpable lymphadenopathy or splenomegaly. A careful pharyngeal examination failed to reveal any evidence of a mass.Cervical spine x-rays, including myelogram and computerized tomography (CT), showed extensive erosion of the C4 vertebral body and an extradural mass that caused almost total block at C5-6. The extent of destruction was such that it was impossible to tell whether the process started in the bone or within the epidural space. There was paravertebral soft tissue swelling without an apparent soft tissue mass. Some narrowing of nerve sleeves at L4-5, without bony changes, was seen. Computerized tomography scan of the brain with contrast enhancement demonstrated a 5 X 3.5 X 1.5 cm mass which appeared to arise from the right frontal bone with displacement of the right frontoparietal lobes (Fig. 1). There were no other bony abnormalities in the skeleton. A chest x-ray and CT scan of the chest, spine, abdomen, and pelvis all were reported as unremarkable.Biopsy of the vertebral mass was undertaken through an anterior approach. The lesion was gray and firm. No bone could be identified and only a small piece of the mass was excised for diagnosis. The tissue obtained consisted of fibrous stroma with many eosinophils and occasional atypical Reed-Stemberg-like cells. Because this piece was not considered to be diagnostic, the intracranial lesion was excised. It was primarily dural and could be separated from the adjacent bone and brain by blunt dissection. This specimen consisted of approximately 10 X 5 X 1 cm of material. There was a large fragment of dura to which a nodular mass of firm yellow tumor was attached (Fig. 2).Portions of both specimens were used to make imprints, blocks embedded in glycol methacrylate, and paraffin-embedded blocks. Zeihl-Neelsen stain was performed o...

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Immunoperoxidase technics were used to examine the immunoglobulin content of sections of pulmonary tissue from two typical cases of lymphomatoid granulomatosis and two cases of pneumonic processes initially diagnosed as lymphomatoid granulomatosis but representing different processes on review. Both "typical" cases and one of the others had a predominantly mixed pattern of all immunoglobulins. One "typical" case showed a focus of exclusively IgG/K staining, which corresponded to histologic malignancy. Less than 1% of cells stained in the fourth case. These results demonstrate that several different processes may fit the morphologic criteria of lymphomatoid granulomatosis; that there is a group of cases that typify lymphomatoid granulomatosis clinically and histologically, and that these cases represent a B-cell proliferation that is initially polyclonal but may evolve into immunoblastic sarcoma. "Typical" cases are similar to other lymphoreticular proliferations with malignant potential, such as angioimmunoblastic lymphadenopathy and Sjögren's syndrome.

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Three cases of tuberous sclerosis in neonates were found to have focal, frequently perivascular, collections of large cells with abundant eosinophilic cytoplasm. These cells resembled those found in brain lesions of tuberous sclerosis but did not stain for acidic protein. Ultrastructurally, they were characterized by many membrane bound cytoplasmic bodies, 90 to 270 nm in diameter, with amorphous contents. Filaments were not demonstrated. Their appearance is considered most consistent with histiocytic origin. Large cells with a histiocytic appearance and a superficial resemblance to those seen in the brain in tuberous sclerosis, but a different ultrastructure and reaction to GFAP staining, may be found in the spleen of neonates with this disease.

This report describes some additional morphologic observations on a mediastinal ganglioneuroma. The neoplastic neurons contained argentaffin cytoplasmic granules presumably representing neurosecretory granules. Cytoplasmic inclusions resembling Pick's bodies were frequently observed in the neuronal components of the tumor. These inclusions consisted of neurofilaments mixed with a variable number of microtubules, dense core vesicles and other organelles. These findings expand the range of conditions in which Pick's bodies are found.