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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report two cases of Langerhans' cell histiocytosis with unusual central nervous system (CNS) involvement. The first patient had behavioural disturbances, memory loss and diabetes insipidus. His response to a range of treatments was poor. The second patient presented with seizures and headaches suggestive of raised intracranial pressure. Etoposide (VP16) chemotherapy led to a dramatic clinical and radiological improvement. The various CNS manifestations of Langerhans' cell histiocytosis and their management are discussed.
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PMID:Langerhans' cell histiocytosis and the nervous system. 151 12

We reviewed the Tumor Registry for 1981 at the Children's Hospital of Philadelphia to identify all the children with newly diagnosed cancer who were seen initially in the emergency department (ED). Of the 220 new patients listed, 16 (7.3%) sought initial care in the ED (1 per 4,500 ED visits). Seven had leukemia, five had non-CNS solid tumors (2 lymphoreticular, 1 Wilms', 1 neuroblastoma, and 1 ovarian), and four had CNS tumors. Among the children with leukemia, pallor (6) and decreased activity (4) were the most common complaints. Duration of symptoms ranged from 4 days to 3 weeks. Physical examination showed pallor (5), splenomegaly (4), fever (3), hepatomegaly (3), lymphadenopathy (3), and ecchymoses or petechiae (2). The complete blood count and peripheral smears were all abnormal. The five patients with non-CNS solid tumors had symptoms related to the location of their neoplasms. The patients with Wilms' tumor, neuroblastoma, and ovarian dysgerminoma had abdominal masses; the patient with lymphoma had a large, painful inguinal node; and the patient with histiocytosis X had an infiltrative rash, gingivitis, and pneumonitis. Of the four children with CNS tumors, three had headache, and one had an incidentally detected scotoma following head trauma. All four eventually had abnormal neurologic exams and computer tomographic scans, but two were discharged initially with psychiatric diagnoses. We conclude that cancer, although rare in children, occurs with greater relative frequency in the referral hospital ED than that predicted by published cancer rates from the referring hospital's ED.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of cancer in the pediatric emergency department. 384 22

A man aged 56 years, previously healthy, developed asthenia, hypersonnia, apathy, later polydipsia and bulimia, headache and episodes of unconsciousness. There was temporary improvement with steriod therapy, but ever-deepening stupor appeared till death due to bronchopneumonia. All blood chemistry examinations were normal. The CSF IgG was elevated but no neoplastic cells were seen. At autopsy areas of grayish color in the basal gagnlia and granulomatous tissue in the floor of the third ventricle and in the mamillary bodies were seen, and the gonads were attrophied. Microscopically, in the floor of third ventricle, mamillary bodies and adjacent leptomeninges there was granulomatous tissue made up of more-or-less typical cells of the reticulum, polymorphonuclear cells, plasma cells and some phagocytes along with proliferation of small blood vessels and reticulin fibers. In addition, the white matter of the frontal lobes, pons, middle cerebellar peduncle and cerebellar white matter contained diffuse proliferation of pleomorphic histiocytic elements with questionable atypical mitoses. Notwithstanding, the morphology of our case suggests that it is a peculiar form of malignant reticulosis (or malignant histocytosis) related to histiocytosis X. The duplicity of the features of our case suggests it to be neoplastic, where the proliferative phase is followed by a granulomatous and sclerotic one.
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PMID:A peculiar case of malignant cerebral reticulosis: clinico-pathological study. 615 91

The authors report a case of histiocytosis X which presented with neurological manifestations in a 20 year old man: episodes of headache with vomiting, followed, three months later, by the development of paralysis of the right 6th and 7th cranial nerves associated with nystagmus. The CT scan revealed a tumour-like lesion on the floor of the IVth ventricle with a long axis of 18 mm. The chest x-ray revealed diffuse nodular opacities in the pleural and apical regions with features suggestive of histiocytosis X. The diagnosis was confirmed by surgical biopsy of the typical pulmonary nodules which were rich in histiocytes with X bodies on electron microscopy. The neurological signs disappeared after one month of treatment with Prednisone (1 mg/kg/day) and Vincaleukoblastine (10 mg/week). By the 3rd month, the pulmonary lesions were reduced and the intra-ventricular formation had regressed by 40%. In the authors' series of 29 cases of confirmed histiocytosis X in adults, the present case is the only one with a clinical neurological presentation, apart from 3 cases of diabetes insipidus. A review of the literature confirms the rarity of this type of presentation. The suggestion of the diagnosis by the chest x-ray appearance enabled a dangerous neuro-surgical operation to be avoided.
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PMID:[Cerebral and pulmonary histiocytosis X. Neurologic manifestations disclosing a pseudotumoral formation on the floor of the 4th ventricle]. 633 82

A retrospective analysis of 40 cases with histiocytosis X was undertaken to find out the course of primarily localized disease, and the prognosis of children with initially disseminated disease. Bone lesions recurred in nine of 23 children with localized histiocytosis X. In eleven cases other organ manifestations occurred as well; in four cases without bone relapse. After an observation period of 1-14 3/12 years, nine of 22 children in remission suffer from long-term sequelae like diabetes insipidus, convulsion, extrahypothalamic CNS-disease, orthopedic disability, growth retardation, dystrophia adiposogenitalis , and chronic headache. Four of 17 children with disseminated histiocytosis X died. Our results and others from the literature indicate various risk factors to be prognostically significant. 1) age less than 2 years 2) involvement of spleen and/or lung 3) elevated Lahey-score 4) dysfunction of the hematopoietic system, liver, and/or lung 5) histologic feature resembling malignant type 6) no response to therapy 7) severely affected general health. These factors can be evaluated initially. Considering our own experiences and some risk factors we suggest the definition of four risk groups: 1.) localized histiocytosis X of bone, lymph nodes or skin; 2.) disseminated histiocytosis X with benign histologic type and Lahey-score of one or two; 3.) Lahey-score of 3-8; 4.) disseminated histiocytosis X with dysfunction of certain organ systems and/or malignant histology.
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PMID:[Histiocytosis X--a retrospective analysis of 40 cases with localized or disseminated disease]. 661 Jan 8

An 11-year-old black boy complained of intermittent occipital headaches with nausea and projectile vomiting. Previous skin and lung biopsy specimens were interpreted as histiocytosis X. Cranial computed tomographic scanning disclosed a mass lesion in the region of the choroid plexus of the left lateral ventricle. This was surgically removed but proved nondiagnostic despite extensive histologic examination. An ophthalmologic evaluation showed discrete, elevated, yellow-white choroidal tumors in both maculas. The ophthalmoscopic appearance, as well as ultrasonography and computed tomography, led to the diagnosis of choroidal osteomas.
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PMID:Bilateral choroidal osteomas associated with fatal systemic illness. 697 70

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

A case of Langerhans cell histiocytosis (LCH) in the hypothalamus is presented. A 52 year old man with eosinophilic granuloma of the lung was referred to our hospital. He complained of headache, easy fatigability, high fever, and voiding large quantities of urine, but was otherwise asymptomatic. T1-weighted images demonstrated a small mass in the hypothalamus. After about six months, the mass had grown to 2 cm in diameter. Surgery was performed by a pterional approach and a biopsy specimen was collected. Light microscopic examination of sections of the biopsy specimen showed polymorphous cellular infiltration by histiocytic cells, eosinophils, neutrophils, and lymphocytes. Electron microscopy showed Langerhans cells and Birbeck granules in the cytoplasm. The histological diagnosis was LCH, multifocal type. After low-dose radiation therapy, the mass gradually decreased in size. The clinicopathological features, neuroradiological findings, and treatment are briefly discussed.
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PMID:[Langerhans cell histiocytosis in the hypothalamus: a case report]. 962 69

An 8-year-old boy developed vomiting and severe headache following minor head trauma. A CT scan of the head demonstrated a lytic lesion of the skull and adjacent epidural hematoma. Surgical evacuation and removal of the skull lesion and hematoma were carried out, and pathologic evaluation resulted in a diagnosis of Langerhans' cell histiocytosis (LCH). Epidural involvement of Langerhans' cell histiocytosis is very rare, and we report the first case of LCH presenting as an intracranial epidural hematoma.
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PMID:Langerhans' cell histiocytosis presenting with an intracranial epidural hematoma. 1083 96


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