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)

Laminectomy, performed on a 46-year-old woman because of weakness and paresthesias of the lower limbs, showed multiple discrete yellow nodules on the nerve roots of the cauda equina. One of these lesions was excised and proved to be a granuloma consistent with the diagnosis of histiocytosis X.
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PMID:Histiocytosis X of the cauda equina. 108 94

We present 23 cases of Langerhans cell histiocytosis with central nervous system (CNS) involvement. The major complaints were a mass on the cranial vault in fifteen (65%), visual disturbance in four (16%), polyurea-polydipsia in three (13%), and progressive weakness in all extremities in one patient (4%). Neurological examination revealed no abnormality in sixteen patients (70%), cranial nerve palsy, visual field defect and optic atrophy in six (26%) and paraparesis in one (4%). Tumoral mass was found to be located on the cranial vault (65%), in the suprasellar region (21%) and in the spinal column (8%). The cranium and spinal column were both involved in one patient. All patients underwent surgery; craniectomy with grossly total tumor excision plus cranioplasty (65%), craniotomy with subtotal tumor excision (26%), and vertebrectomy with grafting (13%) were performed. The clinical, radiological and histopathological features, as well as therapeutical considerations are discussed and the pertinent literature is reviewed.
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PMID:CNS involvement of Langerhans cell histiocytosis. Report of 23 surgically treated cases. 900 88

This report concerns the clinicopathologic features of 4 patients with CD56/neural cell adhesion molecule (NCAM)-positive Langerhans cell sarcoma (LCS). Three of the patients were elderly, between 59 and 62 years of age at presentation, and the other was 35 years old. The presenting symptoms included fever, bone pain, and weakness. The patients shared some clinical findings, such as multiorgan involvement of lymph nodes, skin, lung, bone marrow, and spleen. LCS carries a poor prognosis, and 3 of the patients died of the disease within several years of presentation despite multiagent chemotherapy and radiotherapy. Of special interest is that all of the cases showed CD56 expression on the tumor cells in addition to expression of CD1a, S100beta, and langerin, the presence of which suggests derivation from Langerhans cells. For control, CD56 was also examined in 8 cases of Langerhans cell histiocytosis (LCH), a single-system unifocal or multifocal disease, and the results of staining of the tumor cells were negative. Our findings indicated that CD56 may be a clinically relevant biologic marker for predicting an intractable course of Langerhans cell neoplasms, although it is often difficult to draw a definite morphologically-based distinction between LCS and LCH.
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PMID:CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases. 1591 64

We report an unusual case of spinal Langerhans' cell histiocytosis of the cervicothoracic junction in a young adult man. A 17-year-old male was referred to our institution with a 3-week history of cervicothoracic pain associated to a weakness of his right upper limb. Computed tomography and magnetic resonance imaging showed a collapsed T1 vertebral body with epidural soft tissue showing mass effect on spinal cord. The patient underwent a classic anterior cervicotomy. Complete removal of the lesion could be achieved, but the soft consistency of C7 and T2 body precluded a solid anterior fixation and an extended resection of C7 and T2 body had to be performed. Then a C6 - T3 stabilisation using an anterior plate fixation and cyanomethylacrylate graft was performed. Postoperative course was uneventful. At 2 years follow-up, the patient was asymptomatic and radiological workup showed a perfect stability of anterior fixation system. Aggressive surgical management of eosinophilic granuloma should be considered in some selected cases particularly when spinal instability or neurological deficit occurs. In this young patient a modified anterior cervicotomy allowed a comfortable approach to the anterior aspect of T3 vertebra for spinal fixation.
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PMID:Spinal Langerhans' cell histiocytosis in a young adult: case report and therapeutic considerations. 1745 94

The authors present a rare case of C-6 vertebral involvement in a 12-year-old boy with histiocytosis X. The patient presented with limitation in movements of neck and upper extremities. Computed tomography (CT) and magnetic resonance (MRI) imaging were used in the preoperative workup. Surgery was performed via an anterior cervical approach along with stabilization using a fibula strut graft and plate fixation. Pathological assessment showed infiltration of Langerhans cells accompanied by a mixture of many eosinophils, giant cells, neutrophils and foamy cells. The patient went on to make a full recovery with complete resolution of his motor weakness. In an extensive review of the literature there are very few cases of cervical histiocytosis X reported. In addition, surgical management of this type of lesion has rarely been discussed.
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PMID:Rare C-6 vertebral involvement in a child with histiocytosis X: case report. 1759

Hodgkin's disease presenting with spinal cord compression owing to extradural and bone involvement is extremely unusual. A 48-year-old man presented with progressive lower extremity weakness resulting from spinal cord compression attributable to an epidural mass in the thoracic vertebrae. The patient underwent decompressive surgery, and was then treated with chemotherapy for Langerhans cell histiocytosis. However, the disease progressed, and we performed second decompressive surgery with stabilization. Subsequent histopathological investigations revealed Hodgkin's lymphoma of the bone. Here, we describe an unusual case of spinal Hodgkin's lymphoma and Langerhans cell histiocytosis to draw attention to this combination as a possible diagnosis in patients with mixed inflammatory cell infiltrate lesions in the spine.
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PMID:Simultaneous Occurrence of Hodgkin's Lymphoma and Langerhans Cell Histiocytosis of the Spine : A Rare Combination. 2046 Nov 74

Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corpectemy and fusion. A 5-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying right arm motor weakness. CT scans revealed destruction of C7 body and magnetic resonance imaging showed a tumoral process at C7 with cord compression. Interbody fusion using cervical mesh packed by autologus iliac bone was performed. Pathological examination confirmed the diagnosis of LCH. After the surgery, the boy recovered from radiating pain and motor weakness of right arm. Despite the rarity of the LCH in the cervical spine, it is necessary to maintain our awareness of this condition. When neurologic deficits are present, operative treatment should be considered.
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PMID:Langerhans cell histiocytosis causing cervical myelopathy in a child. 2061 93

Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corporectomy and fusion. An 8-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying upper and lower limbs motor weakness. CT scans revealed destruction of C5 body and magnetic resonance imaging showed a tumoral process at C5 with cord compression. Interbody fusion using anterior cervical plate packed by autologus iliac bone was performed. Pathological examination confirmed the diagnosis of LCH. After the surgery, the boy recovered from radiating pain and motor weakness of limbs. Despite the rarity of the LCH in the cervical spine, it is necessary to maintain our awareness of this condition. When neurologic deficits are present, operative treatment should be considered.
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PMID:[Langerhans cell histiocytosis causing cervical myelopathy]. 2255 59

A 16-year-old girl was referred for the evaluation of headache exacerbation and progressive loss of visual field from one month ago. She also suffered from intermittent diarrhea since 12 months ago and secondary amenorrhea, headache, weight loss (4 - 5 kg) and weakness from six months ago. She had a history of transient polydipsia and excessive urine output during this period. Brain Magnetic Resonance Imaging (MRI) reported a 15 x 15 x 9 millimeters mass lesion in the sellar region. It was extended to the suprasellar cistern with mild compression of the optic chiasm and mild thickening of the pituitary stalk with posterior displacement were reported. In an exisional biopsy of pituitary stalk lesion, the pathology result was indicative of Langerhans cell histiocytosis (LCH). The patient underwent four periods of chemotherapy with prednisolone and vinblastin in 28-day intervals followed by one cycle of radiation therapy. In three months follow up after treatment the tumor size was reduced, Levothyroxin and Prednisolone were tapered, and pituitary hormones were improved.
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PMID:Pituitary stalk thickening in a case of langerhans cell histiocytosis. 2577 95

We report a case of a 45-year-old man with a complaint of both leg weakness and hypoesthesia. Radiological evaluation revealed an osteolytic lesion of the ninth thoracic vertebra. The patient underwent posterior corpectomy with total excision of the tumor, mesh cage insertion with posterior screw fixation and subsequent radiotherapy. Histology confirmed the diagnosis of Langerhans cell histiocytosis (LCH). This case report presents the diagnostic work-up, histopathological evaluation, and the treatment procedures of rare LCH in the thoracic spine.
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PMID:Langerhans Cell Histiocytosis of the Thoracic Spine in an Adult. 2901 9


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