Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The monoclonal antibody Ki-M1P recognizes a formalin/paraffin-resistant differentiation epitope of monocytes and their macrophage derivatives [Radzun et al., Lab Invest 65:306, 1991]. To evaluate its usefulness for neuropathology, we examined a variety of routinely processed tissues using immunohistochemistry. In normal brains, positivity was restricted to ramified microglial cells. Intense labeling of macrophages, ramified and ameboid microglial cells, and rod cells was seen in brains with various degenerative and inflammatory disorders. Astrocytes were negative as determined by double-immunofluorescence labeling using Ki-M1P and anti-glial fibrillary acidic protein (GFAP). Histiocytic lesions (histiocytosis X, xanthogranulomas, granulomatous inflammation) were immunopositive. Among 107 tumors, reactivity of Ki-M1P was observed with some schwannoma and meningioma tumor cells. In addition to macrophages, most gliomas contained small, elongated Ki-M1P-positive cells, which were negative for GFAP. Positivity was also found in two glioblastoma cell lines. Immunoblotting performed on spleen, meningioma and glioblastoma specimens revealed one to three bands in the range of 110 to 130 kDa. We conclude that Ki-M1P can serve as a reliable marker for brain macrophages and microglial cells in routinely processed normal and non-neoplastic tissues, whereas due to the unexpected immunoreactivities results obtained with neoplastic tissues should be carefully interpreted.
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PMID:Ki-M1P as a marker for microglia and brain macrophages in routinely processed human tissues. 146 66

Inflammatory pseudotumors (IPT) are rare lesions composed of inflammatory cells admixed with collagen tissue. Although IPT are ubiquitous, intracranial locations are rare. In this study, four intracranial IPT of the plasma-cell-granuloma (PCG) type are reported. Four patients presented with lesions located, respectively, in the right cavernous sinus, the left cavernous sinus with extension to the tentorium cerebelli, the vermis cerebelli, and the pituitary stalk. All patients were operated on, but complete resection could not be achieved in cases 1 and 2. Follow-up was favorable in all cases, although case 1 still complained of headaches 2 years after operation. All cases were studied on histologic and immunohistochemical bases, and ultrastructural analysis was performed on two cases. In cases 1, 2, and 4, IPT were made up of plasma cells admixed with lymphocytes and rare histiocytes in a fibrous tissue-the density of which varied from case to case. In case 3, the mass was composed of plasma cells associated with numerous foamy histiocytes and polymorphonuclear cells. No light chain restriction could be demonstrated when immunohistochemistry was performed, and ultrastructural study did not disclose features reminiscent of meningioma or histiocytosis X. Intracranial IPT should not be confused with other diseases such as meningioma, lymphoproliferative disorders, or histiocytosis X. Although intracranial locations are much rarer than pulmonary ones, histology is identical in both sites and shows different patterns in its evolution. This is in agreement with the inflammatory origin of this lesion.
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PMID:Intracranial plasma cell granuloma: a report of four cases. 782 2

A rare case of concurrent plasma cell granulomas (PCG) of the lung and the central nervous system (CNS) is reported. A 30-year-old man was presented with recurrent left headaches lasting for two years. Computerized tomographic (CT) scan and magnetic resonance imaging (MRI) of the head disclosed a process extending from the lateral aspect of the left cavernous sinus to the tentorium cerebelli and the infratemporal fossa through the foramen ovale. At the same time, chest-X ray and CT scan showed three symptomless masses of the pulmonary right lower lobe. Histological examination of cerebral samples and of one of the pulmonary nodules revealed the presence of a fibrous tissue containing numerous lymphocytes and plasma cells as well as remnants of vascular and respiratory structures. Immunohistochemical study proved these cells to be polyclonal. Ultrastructural analysis confirmed the presence of lymphoid cells and failed to disclose any argument for meningioma or histiocytosis X. The differential diagnostic problems of PCG are discussed as well as considerations about clinicopathological features, histogenesis and pathogenesis of inflammatory pseudotumours (IPT).
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PMID:Coexistence of plasma cell granulomas of lung and central nervous system. 883 77

The most common indication for the use of radiation therapy in the treatment of benign central nervous system disease is for the treatment of benign brain tumors, such as meningioma, pituitary adenoma, acoustic neuroma, arteriovenous malformation, and craniopharyngioma. Other less common benign intracranial tumors treated with radiation include chordoma, pilocytic astrocytoma, pineocytoma, choroid-plexus papilloma, hemangioblastoma, and temporal bone chemodectomas. Benign conditions, such as histiocytosis X, trigeminal neuralgia, and epilepsy, are also amenable to radiation treatment. There have also been reports of radiosurgery being used for the treatment of movement disorders and psychiatric disturbances, such as obsessive-compulsive and anxiety disorders. For benign brain tumors, radiation therapy as either primary or adjuvant therapy plays an integral role in improving local control. In the treatment of trigeminal neuralgia, epilepsy, tremor, and some psychiatric disturbances, radiosurgery may help ameliorate or eliminate some symptoms. Patients with benign central nervous system disease are expected to live a long time. As such, treatment should be highly conformal and based on three-dimensional planning using magnetic resonance imaging, computed tomography, or both. It is critical that damage to normal brain be minimized.
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PMID:Radiation therapy for benign central nervous system disease. 1009 4

Central diabetes insipidus (DI) can be the outcome of a number of diseases that affect the hypothalamic-neurohypophyseal axis. The causes of the condition can be classified as traumatic, inflammatory, or neoplastic. Traumatic causes include postoperative sella or transection of the pituitary stalk, while infectious or inflammatory causes include meningitis, lymphocytic hypophysitis, and granulomatous inflammations such as sarcoidosis and Wegener's granulomatosis. Various neoplastic conditions such as germinoma, Langerhans cell histiocytosis, metastasis, leukemic infiltration, lymphoma, teratoma, pituitary adenoma, craniopharyngioma, Rathke cleft cyst, hypothalamic glioma, and meningioma are also causes of central DI. In affected patients, careful analysis of these MR imaging features and correlation with the clinical manifestations can allow a more specific diagnosis, which is essential for treatment.
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PMID:MR imaging of central diabetes insipidus: a pictorial essay. 1175 30

Salivary gland tumors are uncommon and most of them are of epithelial origin. Mesenchymal tumors affecting the parotid are extremely rare, and we present a series of 19 cases. All parotid tumors (600 cases) treated at the Department of Head and Neck Surgery from A.C. Camargo Hospital, Brazil from 1953 to 2003 were reviewed and 19 cases of nonlymphoid mesenchymal origin were selected. The histological characteristics were reviewed and clinical features were obtained from the medical charts. 15 out of 19 were benign tumors, including 5 lymphangiomas, 5 neurofibromas, and one case each of schwannoma, lipoma, solitary fibrous tumor, meningioma and giant cell tumor. Four malignant tumors were classified as rhabdomyosarcoma, fibrosarcoma, Langerhans cell histiocytosis and endodermal sinus tumor. From the malignant cases, only the patient with fibrosarcoma died due the tumor, the other three are alive with no signs of recurrence. In our series of 600 cases of parotid gland tumors, nonlymphoid mesenchymal tumors corresponded to 3.16% (19 cases; 15 benign and 4 malignant). All cases were treated by surgery with no recurrences, except one case of fibrosarcoma whose patient died of distant metastasis.
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PMID:Nonlymphoid mesenchymal tumors of the parotid gland. 1828 91

Malignant extraconal orbital tumors are very rare during childhood and must be referred as soon as possible to a highly specialized center to be managed by a multidisciplinary team. They are often referred on an emergency basis. Both diagnosis and treatment must be undertaken as soon as possible. The course of these malignant tumors can be acute and can jeopardize the function of the eye or be life-threatening, especially in the event of metastatic locations. Extra-axial proptosis is by far the most frequent revealing symptom. Local and general examinations are of utmost importance. Sometimes diagnosis should be clear with the association of an orbital tumor and deterioration of the general health status favoring metastatic disease. Most metastatic neuroblastomas present such clinical symptoms in young children. Today both CT and MRI are highly valuable in assessing the diagnosis and starting the management of these tumors. Biopsy is mandatory to confirm the diagnosis. It can be reinforced by molecular biology. Among the primitive tumors, soft tissue sarcomas, especially rhabdomyosarcomas, are the most frequent. The diagnosis is suggested when the onset of the disease is acute and the course is rapid. Most respond to neoadjuvant chemotherapy. In the event of a residual tumor, local treatment is indicated so that surgery and/or radiotherapy are used as second-line treatment. Prognosis is closely related to histology. It can be satisfactory (Langerhans' cell histiocytosis, lymphoma, meningioma, infantile fibrosarcoma) or poor (metastatic tumor, rhabdoid tumor).
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PMID:[Malignant extraconal tumors of the orbit in childhood]. 2030 50

Infantile myofibromatosis is a mesenchymal disorder of early childhood characterized by the formation of tumors in the skin, muscle, viscera, bone, and subcutaneous tissue. Although relatively rare overall, it represents the most common fibrous tumor of infancy. The etiology of this disorder is unknown. Infantile myofibromatosis can present as a solitary or multicentric form. With the multicentric form, bone is often involved, but solitary bone lesions account for only 10% of the cases. Imaging findings are not pathognomonic, and the differential diagnosis usually includes eosinophilic granuloma (Langerhans cell histiocytosis), osteomyelitis, metastasis, osteoblastoma, epidermoid cyst, hemangioma, fibrous dysplasia, fibrosarcoma, and meningioma. A histological pattern is typical, but there are no histopathological differences between the solitary and multicentric forms. Solitary lesions generally have a favorable prognosis if totally removed, with a 10% recurrence rate; incompletely resected lesions recur. We report the case of a 9-year-old boy who came to our attention with a solitary infantile myofibroma of the calvarium, appearing as a tight-elastic, lightly tender mass in the left frontal area, eroding both the inner and the outer tables. Histopathologically, the specimens showed a spindle-cell tumor with dense reticulin fiber network and expression of smooth muscle actin. Fifty-eight months MR follow-up after total removal showed no residual or relapse.
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PMID:Solitary infantile myofibromatosis of the cranial vault: case report. 2127 63

Rosai-Dorfman disease (RDD)-sinus histiocytosis with massive lymphadenopathy-represents a peculiar proliferation of histiocyte-like cells in patients. The condition was described by Rosai and Dorfman in 1969, after examining 4 cases, as an idiopathic histiocytic disorder. In 1972, they studied an additional 30 cases of patients with RDD. A histioproliferative disorder, RDD is characterized by bilateral, painless, cervical lymphadenopathy in 81% of patients. Fever, leukocytosis, elevated sedimentation rate, and polyclonal hypergammaglobulinemia may also be found. In 30% of patients, extranodal involvement is present and may include the skin, eye orbit, upper respiratory tract, or testes. Cases involving the central nervous system are rare and account for < 5% of patients with RDD. We report on a 78-year-old woman presenting with new-onset headache, dizziness, and imbalance, which had been present for a few weeks prior to admission. Magnetic resonance imaging of the brain showed 2 enhancing lesions within the right and left cerebellar hemispheres. Biopsy of the mass demonstrated a lymphohistiocytic infiltrate involving the cerebellum with foci of emperipolesis (phagocytosed lymphocytes). The adjacent cerebellum showed myelinated nerve fibers with reactive gliosis. A thorough work-up and histopathologic exam of the biopsied mass demonstrated lymphohistiocytic infiltrate with foci of emperipolesis (phagocytosed lymphocytes) consistent with RDD. Other differential considerations, such as primary or secondary neoplasms, infections, lymphoproliferative disorders, granulomatoses, Langerhans cell histiocytosis, and lymphocyte-rich meningioma were ruled out by additional histopathologic exam.
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PMID:Case report of intracranial Rosai-Dorfman disease. 2414 94

We report a 41-year old male who presented to the Emergency Department after falling while water-skiing. He had a previous medical history included chronic headaches, which had persisted for the last 2-3 months prior to presentation. Computed tomography of the head showed a small hypersensitivity with a small extra axial collection with a maximum thickness of 1mm. Differential diagnoses included an arachnoid cyst, haemangioma, meningioma or a secondary lesion. A diagnosis of Langerhans Cell Histiocytosis was made based on the histopathology examination and the immunoperoxidase staining.
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PMID:A rare case of langerhans cell histiocytosis of the skull in an adult: a systematic review. 2417 50


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