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

A case of disseminated Langerhans cell histiocytosis with resistant central nervous system (CNS) disease in an adolescent is described. The child presented with visible cranial lesions, emesis, headaches, and short-term memory loss. Diagnostic evaluation revealed multiple osseous lesions in the cranium, ribs, vertebral bodies, and pelvis. The clinical course with complications and response to each therapy are sequentially reviewed. Remission, as evidenced clinically and by magnetic resonance imaging, was ultimately accomplished with 2-chlorodeoxyadenosine (2-CDA). The full course of 2-CDA was not tolerated due to bone marrow suppression. CNS histiocytosis is known to be resistant to therapy. Earlier introduction of 2-CDA for CNS disease might offer more successful treatment with less toxicity than seen in patient.
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PMID:Langerhans cell histiocytosis: central nervous system involvement treated successfully with 2-chlorodeoxyadenosine. 1129 88

Benign intracranial hypertension is known to be associated with obesity, endocrine abnormalities, various medications, and cerebral venous sinus thrombosis. We report a patient presenting with headaches and vomiting attributed to benign intracranial hypertension. The diagnostic work-up revealed Langerhans' cell histiocytosis of the occipital bone. There was no evidence for cerebral vein thrombosis by cranial computed tomography scan, Doppler ultrasonography, planar and single photon emission computed tomography technetium 99m-labelled red blood cell scintigraphy, and magnetic resonance angiography. Excision of the occipital bone lesion and a short course of acetazolamide and prednisone were curative. We hypothesize that cytokines secreted by the tumor were responsible for the development of intracranial hypertension.
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PMID:Langerhans' cell histiocytosis presenting as intracranial hypertension. 1141 6

The May 2002 COM. A 38-year-old man presented with new onset seizures and a 69-year-old woman presented with bilateral headaches and episodes of syncope. Both were found to have extra-axial masses that were contrast-enhancing and thought to be meningiomas. Both had complete resection. Microscopic examination revealed an inflammatory lesion composed of plasma cells, scattered lymphocytes and numerous large histocytic cells, which exhibited emperi polesis and were CD1 a negative, but positive for CD68 and S100. The diagnosis of Destombes-Rosai-Dorfman Disease (DRDD) was rendered. Both cases had good long-term outcome. The differential diagnosis of inflammatory masses in the dura (plasmacytoma, lymphomas, plasma cell fibroma, angiofollicular hyperplasia [Castleman's-disease] and Langerhan's cell histiocytosis) are discussed.
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PMID:May 2002: 38-year-old man and 69-year-old woman with dural based masses. 1240 40

Intracerebral Langerhans' cell histiocytosis (LCH) is rare and tends to involve the hypothalamus. The authors report a rare case of LCH in the temporal lobe that subsequently was followed by a brainstem lesion. This appears to be the first case of temporal lobe and brainstem LCH that has been treated successfully and published. A 24-year-old man complained of cacosmia and nausea with a slight headache. He had a left temporal LCH, which was removed completely, but developed a brainstem lesion a year later. The pontine LCH was treated with radiosurgery. The follow-up period was 4 years without any neurological or radiological symptoms or signs. The 12 cases of solitary intracranial non-hypothalamic LCH reported previously are reviewed. Gamma knife radiosurgery effectively controlled the local growth of the pontine LCH without adverse effect.
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PMID:Langerhans' cell histiocytosis of the temporal lobe and pons. 1517 61

We report the occurrence of unilateral cerebral hemisphere edema with subsequent cortical laminar necrosis in the setting of familial hemiplegic migraine (FHM) and permanent neurologic sequelae after resolution of an attack in 1 patient. Contemporaneous with this severe attack of FHM, the patient was found to exhibit multiple systemic and neurological symptoms referable to Erdheim-Chester disease (a rare non-Langerhans cell histiocytosis) that was confirmed by bone biopsy. This case demonstrates the severity possible with a migrainous infarction associated with FHM. The co-occurrence of two such rare entities in 1 patient suggests a possible relationship.
Headache 2004 Oct
PMID:Familial hemiplegic migraine, neuropsychiatric symptoms, and Erdheim-Chester disease. 1544 1

We describe a new case of isolated Langerhans cell histiocytosis (LCH) of the hypothalamus. A 53-year-old female patient presented with polydipsia, headache, anorexia, and fatigue. Neurological imaging revealed a mass projecting from the hypothalamus into the third ventricle. Gross total removal of the tumor was performed. Light microscopy showed LCH, and immunohistochemical studies revealed S-100 and CD1a immunoreactivity in the Langerhans cells. Although the most common CNS site for LCH is the hypothalamus, isolated hypothalamic LCH, confirmed by biopsy, is very rare. The residual mass appeared to remit spontaneously 3.5 years after surgery, with regrowth 3 years later.
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PMID:A case of isolated langerhans cell histiocytosis of the hypothalamus with remission and regrowth after surgery. 1570 Aug 40

A 17-year-old boy presented with a left upper lid swelling, headaches and diplopia. An orbital computerized tomography (CT) scan showed a mass in the left lacrimal fossa eroding bone and extending into the temporalis fossa and intracranially. An urgent biopsy without curettage was carried out and showed Langerhans cell histiocytosis. He was otherwise well and no other lesion was found. He was therefore observed and reviewed regularly. During follow-up it was noted that the mass was reducing in size. Five months after the initial biopsy a further procedure, involving curettage and an intralesional steroid, was carried out at a tertiary referral center. Histology of the material obtained showed no remaining evidence of Langerhans cell histiocytosis. Spontaneous resolution of orbital Langerhans cell histiocytosis has been described clinically and radiologically. This is the first case of spontaneous resolution to be confirmed histologically.
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PMID:Biopsy-confirmed spontaneous resolution of orbital langerhans cell histiocytosis. 1576 15

Cerebral solitary Langerhans cell histiocytosis (LCH) is a very uncommon condition. We describe two new cases: a 30-year-old man with seizures and a tumour in the left frontal lobe, which was composed of a polymorphic infiltrate with a predominance of histiocytes and eosinophils; and a 65-year-old man with headaches and dysarthria, with a left parietal tumour, which showed a diffuse proliferation of histiocytic cells and areas of necrosis. In both cases, the histiocytes were strongly positive for S-100 and CD1a, and Birbeck's granules were demonstrated by electron microscopy in the first case. Both patients underwent a complete excision of their lesions. The second patient received additional postoperative radiotherapy. They were asymptomatic after 26 and 27 months, respectively. It seems that cerebral solitary LCH is a clinicopathological entity with a good outcome. Only 15 cases of this rare process have been previously reported in the English literature.
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PMID:Cerebral solitary Langerhans cell histiocytosis: report of two cases and review of the literature. 1612 May 27

Langerhans cell histiocytosis frequently manifests as lesions of the skull. The disease can present with a wide spectrum of forms, from an isolated eosinophilic granuloma to multiple lesions with diffuse systemic involvement. The authors report the case of a 12-year-old boy with a 1-month history of left temporal and periorbital pain and headaches. Noncontrast computed tomography of the head was done at the time of initial presentation and was interpreted as normal. Over the next month, the patient continued to have headaches and periorbital swelling and began having bloody discharge from his nose. Magnetic resonance imaging of the brain ordered by his pediatrician showed a lesion in the left infratemporal fossa, left orbit, and sphenoid bone. The lesion was biopsied and confirmed to be Langerhans cell histiocytosis. We describe a novel multidisciplinary approach for treatment of this tumor.
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PMID:Novel multidisciplinary approach for treatment of langerhans cell histiocytosis of the skull base. 1859 19

The second most frequent central nervous system involvement pattern in Langerhans cell histiocytosis (LCH) is a rare condition documented in a number of reports called "neurodegenerative LCH" (ND-LCH). Magnetic resonance images confirming the presence of the disease usually demonstrate striking symmetric bilateral hyperintensities predominantly in the cerebellum, basal ganglia, pons, and/or cerebral white matter. The authors here describe for the first time in the literature a patient with ND-LCH and concomitant hydrocephalus initially treated using endoscopic third ventriculostomy (ETV). This 9-year-old boy, who had undergone chemotherapy for skin and lung LCH without central nervous system involvement at the age of 10 months, presented with acute ataxia, headaches, and paraparesis and a 1-year history of gradually increasing clumsiness. Magnetic resonance images showed obstructive hydrocephalus at the level of the aqueduct of Sylvius and signs of ND-LCH. After registering high intracranial pressure (ICP) spikes with an intraparenchymal pressure monitor, an ETV was performed. A second ETV was required months later because of ostomy occlusion, and finally a ventriculoperitoneal shunt was placed because of ostomy reocclusion. Endoscopic third ventriculostomy was initially considered the treatment of choice to divert cerebrospinal fluid without leaving a ventriculoperitoneal shunt and to obtain biopsy specimens from the periinfundibular recess area. The third ventriculostomy occluded twice, and an endoscopic aqueduct fenestration was unsuccessful. The authors hypothesized that an inflammatory process related to late ND disease was responsible for the occlusions. Biopsy specimens from the infundibular recess and fornix column did not show histopathogical abnormalities. Increased ICP symptoms resolved with cerebrospinal fluid diversion. This case is the first instance of ND-LCH with hydrocephalus reported in the literature to date. Shunt placement rather than ETV seems to be the favorable choice in relieving elevated ICP.
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PMID:Failure to treat obstructive hydrocephalus with endoscopic third ventriculostomy in a patient with neurodegenerative Langerhans cell histiocytosis. 1897 98


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