1/9. Pagetoid self-healing Langerhans cell histiocytosis in an infant.We report Langerhans cell (LC) histiocytosis in a male infant who developed numerous papular lesions on the trunk and posterior scalp soon after birth and spontaneously recovered from the disease within 7 months. Histologically S-100-positive cells were detected in the epidermis and papillary dermis, in some lesions mostly in the epidermis. Tumor cells in the epidermis were either clustered, forming nests, or scattered singly in pagetoid fashion. Electron microscopy confirmed the presence of Birbeck granules in these cells. They exhibited many interesting features usually not found in normal LCs, including mitosis, frequent apoptosis, Birbeck granules invaginated in the nucleus, autophagocytosis of Birbeck granules, and active ingestion of extracellular material through Birbeck granules attached to cell membranes. It is suggested that either a strong epidermotropism of tumor cells or a proliferation of the resident LCs of the epidermis is responsible for this intraepidermal growth pattern. Cellular necrosis through very active apoptosis and the superficial nature of the growth might have contributed to the self-healing course in this patient.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
2/9. Choroidal Langerhans' cell histiocytosis.PURPOSE: To report a patient with choroidal Langerhans' cell histiocytosis. methods: A solitary tumor was found in the left eye of a 49-year-old male who had no definite history of systemic disorders, but had observed visual disturbances for a period of 1 month. ultrasonography, fluorescein angiography, and indocyanine green angiography were performed and the eyeball was enucleated. We prepared the specimen for microscopic examinations. RESULTS: Fluorescein angiographic findings of the lesion were mottled hyperfluorescence in the arteriovenous phase and strong hyperfluorescence in the late phase. Hypofluorescence in both early and late phases showed on indocyanine green angiogram. The lesion of choroid was widely infiltrated by histiocytes, though no extraocular invasion was found. Immunohistochemical studies including S-100 and CD 68 staining revealed characteristic features of Langerhans' cell histiocytosis. Electron microscopic examination of the histiocytes showed histiocytosis X body (Birbeck granule) in the cytoplasm and indented nucleus. CONCLUSION: We consider that this is a case of choroidal Langerhans' cell histiocytosis with no evidence of systemic lesions.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
3/9. Langerhans cell histiocytosis of an adult with tumors in liver and spleen.We describe a 58-year-old male with multiple histiocytic tumors in the liver and spleen. Multiple tumors in the liver and spleen were seen by image analysis, and splenectomy showed a large splenic tumor with a small nodule and a swelling lymph node in the hilus. Histological features of the tumors in the liver and spleen revealed proliferation of histiocytic cells with large and clear cytoplasm and a horseshoe-shaped nucleus. Immunohistochemical studies revealed the presence of S-100 protein and CD1a antigen in the tumor cells, and neither lymphocytic marker nor lysozyme was detected. No definite Birbeck granules were seen ultrastructurally, thus the tumor cells could be classified into Langerhans cell type without Birbeck granules. Administration of adriamycin, vincristine, cyclophosphamide and prednisolone reduced size and number of the liver tumors, and the histiocytic cells could not be detected in repeatedly biopsied tissue from liver tumor. We present the clinical, immunohistological and cytological features in a visceral type of adult Langerhans cell histiocytosis, which responded well to chemotherapy.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
4/9. An ultrastructural and histochemical study on bone lesions of hand-Schuller-Christian disease.A correlated light microscopical, histochemical and ultrastructural study was made on bone lesions repeatedly biopsied from three patients with hand-Schuller-Christian disease. The histiocytic cells proliferating in the early lesions had a markedly indented nucleus and contained in their cytoplasm varying numbers of Langerhans cell granules and round or irregular-shaped lipid droplets. Histochemically, the lipid droplets were verified to the lipo-protein rich in fatty acids. Such histiocytic cells differ from highly phagocytic histiocytes or macrophages because of indistinct phagocytotic activity, and are regarded as a metabolic type of histiocytes. The late lesions were mostly dominated by a xanthogranulomatous tissue chiefly comprised of xanthoma cells and collagen fibers. The xanthoma cells were usually filled with numerous lipid vacuoles which were histochemically proved to mainly contain cholesterol. Almost all the xanthoma cells were closely attached to collagen fibers and often had well-developed rough endoplasmic reticula. Ultrastructurally, it is suggested that the xanthoma cells are originated from fibroblasts or fibrocytes. As an important factor to induce proliferation and xanthoma cell transformation of fibroblasts in the late phase, the presence of such a lipoprotein produced in the histiocytic cells of the early phase is emphasized.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
5/9. Primary benign histiocytosis X of the stomach. A report of a case showing spontaneous remission after 5 1/2 years.A 49-year-old Japanese woman was found to have innumerable, small sessile elevations throughout the stomach as revealed by radiographic and endoscopic examination. Endoscopic biopsy showed that the elevations were formed by a diffuse proliferation of histiocytes in the lamina propria mucosae. The histiocytes were characterized by abundant interdigitating cytoplasmic projections, Birbeck granules, an oval or indented nucleus, and an absence of phagocytosis. They stained for S-100 protein but not for lysozyme, alpha-1-antitrypsin, or nonspecific antigen cross-reacting with carcinoembryonic antigen. Five and a half years after the first diagnosis, most of the elevated lesions disappeared, but a few histiocytoid cells were noted microscopically. Systemic examination showed no abnormalities outside the stomach. The patient has continued to be well. This case was diagnosed as primary benign histiocytosis X of the stomach. Four similar cases have been reported. This is the first case that was diagnosed using biopsy techniques and in which the natural history--i.e., from multiple polypoid extension throughout the stomach to spontaneous remission--was observed.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
6/9. histiocytosis X in adult with skin and uncommon central nervous system involvement.Report of a case of histiocytosis X with adult onset, widespread skin lesions and rare central nervous system involvement resulting in dizziness and ataxia. On autopsy histiocytosis X infiltrates in the central nervous system were demonstrated with granuloma formation in the temporal lobe and nucleus dentatus of the cerebellum.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
7/9. Isolated abducens nucleus damage due to histiocytosis X. Electro-oculographic analysis and physiological deductions.A case of complete rightward conjugate gaze paralysis, with electro-oculographic recordings, is reported. The only additional feature on clinical examination was a right infranuclear facial paresis. Radiographs of the chest showed multiple infiltrates in both lung fields; lung biopsy with ultrastructural study led to the diagnosis of histiocytosis X. A CT scan showed a single small tumour-like lesion, hyperdense and clearly defined, lying centrally in the fourth ventricle with a small ventral extension located just to the right of the midline. With appropriate treatment all the clinical signs rapidly subsided, together with the disappearance of the right ventral extension of the lesion visible on the CT scan. The clinical and CT scan data suggested that the initial parenchymatous damage almost exclusively involved the right abducens nucleus, a structure which is very rarely affected in an isolated manner. Both main clinical features of the abducens nucleus syndrome which distinguish it from the recently described paramedian pontine reticular formation syndrome were present, namely absence of all ipsilateral conjugate movements and persistence of ipsilaterally directed saccades in the contralateral hemifield of movement. Furthermore, differences between peak velocities of both eyes in oculocephalic movements suggested that a pathway analogous, at least functionally, to the ascending tract of Deiters described in the cat and in the monkey may exist in man. Lastly, some recent clinical data concerning this unusual cause of CNS damage are briefly reviewed.- - - - - - - - - - ranking = 6keywords = nucleus (Clic here for more details about this article) |
8/9. Benign histiocytosis X of stomach. Previously undescribed lesion.histiocytosis X of the stomach of a 47-year-old Japanese woman, who underwent subtotal gastrectomy following a clinical diagnosis of scirrhous carcinoma, was studied by light and electron microscopy as well as by immunohistochemistry. The histiocytoid cells proliferated monotonously in the lamina propria mucosae of the atrophied mucosa covering the body and fornix. They were arranged in a sheet- or pavement stone-pattern and included some giant cells. The histiocytoid cells had a reniform to irregularly indented nucleus and conspicuous cytoplasm. Ultrastructurally, they were characterized by interdigitating cytoplasmic extensions and abundant tubulovesicular structures including Langerhans granules. S-100 protein, alpha 1-antitrypsin, and alpha 1-antichymotrypsin were immunohistochemically identified in the cytoplasm. Endoscopic biopsies of the extragastric digestive tract, a biopsy of the lymph node, and bone marrow aspiration excluded a systemic disorder. The case is regarded as benign localized histiocytosis X of the stomach, a previously undescribed gastric lesion.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
9/9. Langerhans cell histiocytosis in the elderly: a report of three cases.Langerhans cell histiocytosis is most common in children and is unusual in the elderly. We describe 3 cases of langerhans cell histiocytosis limited to the skin in elderly patients. biopsy specimens showed a dermal infiltrate abutting the epidermis composed of atypical langerhans cells with abundant eosinophilic cytoplasm and a "kidney-shaped" nucleus. Immunoperoxidase stain CD1a was positive in all 3 cases and S-100 stain was positive in 2. Electron microscopy revealed Birbeck granules in the cytoplasm of the atypical langerhans cells in 2 cases. Langerhans cell histiocytosis with skin involvement has a chronic course with an overall good prognosis. However, cutaneous manifestations may precede systemic involvement by many years.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |