Hodgkin's disease presenting with isolated craniospinal involvement.

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.

Effective use of bronchoscopy and sputa in the diagnosis of lung cancer.

The records of 134 patients who underwent bronchoscopy at the Forbes Health System hospitals between January 1, 1982 and December 31, 1983 were reviewed. The number of pre- and postbronchoscopy sputa obtained on each patient, final diagnosis, and follow-up for 6 to 30 months were obtained. Tissue obtained at bronchoscopy yielded a diagnosis of malignancy in 71 of 84 (84.5%) patients who received that final diagnosis. Addition of postbronchoscopy sputa examination increased the yield to 73 of 84 (86.9%). Prebronchoscopy sputa contributed no diagnoses beyond those made by bronchoscopically obtained material. The cost to the patient of examining pre- and postbronchoscopy sputa in addition to bronchoscopically obtained cytologic and biopsy material is 70% higher than that of examining bronchoscopically obtained material alone. Indirect costs of increased hospital stay may also be significant. We propose early bronchoscopy without prebronchoscopy sputa in cases where there is clinical suspicion of lung cancer. Postbronchoscopy sputa should be obtained, but only processed for microscopic examination if the bronchoscopically obtained material does not yield a diagnosis.

Adenocarcinoma of the Fallopian tube. An ultrastructural study.

A case of fallopian tube adenocarcinoma was studied by light and transmission electron microscopy. The neoplastic cells contained abundant mitochondria, moderate to large amounts of rough endoplasmic reticulum (RER) arranged in parallel arrays and often containing amorphous material, annulate lamellae, possible secretory vesicles, and glycogen. The presence of stacked RER and annulate lamellae together is unusual in papillary serous cystadenocarcinoma of the ovary, and has not been described in Fallopian tube adenocarcinoma. Golgi complexes were are. Small acini with projecting microvilli as well as junctional complexes were present, but cilia were not found. The electron microscopic findings suggest secretory activity, and are remarkably similar to those found in papillary serous cystadenocarcinomas of the ovary. The findings also support the hypothesis that ovarian serous tumors and adenocarcinomas of the Fallopian tube are derived from coelomic epithelium.

Immunolocalization of entactin, a sulfated basement membrane component, in rodent tissues, and comparison with GP-2 (laminin).

Entactin is a sulfated glycoprotein in the extracellular basement membrane like matrix produced by M1536-B3 cells, a mouse endodermal line derived from an embryonal carcinoma. It has a molecular weight of 158,000 and is chemically and immunologically distinguishable from GP-2 (laminin) and fibronectin. Antibodies produced against entactin and GP-2 react with subepithelial and vascular basement membranes in rat lung, liver, spleen, and kidney and mouse placenta and kidney when examined by light microscopy. Both antibodies yield staining around the marginal sinus of the white pulp of the spleen. Antientactin reacts with basement membrane and mesangium in rat glomeruli, and anti-GP2 does not. Ultrastructurally, staining in kidneys is strongest at epithelial or endothelial cell membranes bordering basement membranes, with only moderate staining of the basement membrane proper. Intracellular staining is not present. The location of entactin suggests that it has a role in the interaction of cells with extracellular matrix, possibly in adhesion. Lack of intracellular staining suggests that the tissues studied are not actively producing entactin or GP-2 and that these substances may be fairly stable in adult organisms.

Splenic involvement in tuberous sclerosis. Report of three cases.

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.

Disseminated nongranulomatous Mycobacterium avium osteomyelitis.

A 17 month old black girl had multiple, rapidly progressive osteolytic lesions, cervical lymphadenopathy, fever, and weight loss clinically compatible with disseminated malignant disease. Histologically the bone lesions were characteristic of acute nongranulomatous osteomyelitis with a few acid fast organisms. Mycobacterium avium was cultured. Immunologic evaluation showed an intact response to the infection. Disseminated Mycobacterium avium infection may occur in immunologically intact children, clinically simulate malignant disease, and produce an acute nonspecific inflammatory lesion.

Central nervous system pathology of tuberous sclerosis in children.

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.

Immunoglobulin production in lymphomatoid granulomatosis and relation to other "benign" lymphoproliferative disorders.

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.

Intraosseous "chordoid" sarcoma, chondroblastic or lipoblastic origin?

An unusual sarcoma with myxoid features that apparently originated within the scapula and has been locally aggressive for 10 years is reported. Clinically and by light microscopy it was considered to be a cartilaginous tumor, possibly chordoid sarcoma. By histochemical techniques and electron microscopy it most closely resembles a tumor of brown fat.

Diffuse parenchymal amyloidosis of lungs and breast. Its association with diffuse plasmacytosis and kappa-chain gammopathy.

A patient had systemic amyloidosis that extensively involved the lungs and breasts. Diffuse parenchymal pulmonary amyloidosis is rare but well described. Involvement of the breast in systemic amyloidosis is, however, most unusual. This patient's amyloidosis was associated with diffuse plasmacytosis and a kappa-chain gammopathy. The plasmacytosis was most prominent in the renal interstitium. Immunoperoxidase staining of the renal infiltrate and breast demonstrated IgG/kappa-staining plasma cells exclusively, suggesting that these cells were a monoclonal proliferation that contributed to the patient's M-protein and possibly to the patient's amyloid deposits.

Light and electron microscopic observations on a ganglioneuroma.

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.

Fatal myocarditis associated with acute tonsillitis.

A case of myocarditis, hepatic necrosis, and acute anuria associated with acute tonsillitis was described. The previously reported relation between myocarditis and tonsillitis not of diphtheritic or beta-hemolytic streptococcal origin was discussed, as well as the implications for management.