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)

Solitary focal eosinophilic granuloma (EG) is one element in the spectrum of diseases associated with Langerhans' cell histiocytosis (LCH). This report documents the occurrence of a primary isolated hypothalamic EG in a man who presented with diabetes insipidus and panhypopituitarism. His treatment consisted of complete microsurgical excision of the lesion. After a 13-month follow-up period, no residual tumor was evident on magnetic resonance imaging and no other lesions were present in peripheral tissues. This case is unique in several respects: 1) it is the third documented case of a primary isolated hypothalamic LCH granuloma diagnosed in a living patient; 2) it is the only known example of complete microsurgical excision of such a lesion in the hypothalamic region; and 3) it demonstrates the efficacy of direct surgery in this scenario, as compared with other treatment modalities such as biopsy and irradiation, suggesting that complete surgical excision may represent the treatment of choice for isolated intracerebral LCH granulomas, being curative in most instances. Also, the literature is reviewed for information about the diagnosis and treatment of this particular type of unifocal brain lesion.
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PMID:Microsurgical excision of a primary isolated hypothalamic eosinophilic granuloma. Case report. 968 34

A case of eosinophilic granuloma of the lung in a 24 years old male is presented. The initial manifestations were hemoptysis and cough. During hospitalization the bronchoscopy was performed, which revealed granuloma in the right bronchus. Computer tomography showed tumour in the right bronchus, without pulmonary changes characteristic for histiocytosis X. The granuloma was removed by bronchoscopy, but control examination performed after three months showed the residue. Once again residual tumor was removed by bronchoscopy and oral steroids were ordered. Unfortunately the next local residue was observed and the residual tumour was finally removed by thoracotomy. Now patient is free of symptoms.
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PMID:[A case of eosinophilic granuloma localized in the bronchial wall]. 1035 91

Langerhans' cell histiocytosis (LCH) can involve virtually any site and organ of the body, either as an isolated lesion or as a widespread systemic disease. Bone can be involved as a part of generalised disease or as a separate entity, formerly known as eosinophilic granuloma. The clinical cause of localised LCH (LLCH) to bone is generally benign. Solitary bone lesions may resolve spontaneously after diagnostic biopsy in a period of months to years. If treatment is indicated, as in larger lesions with pain, swelling and risk for spontaneous fracture, the disease can be controlled by chemotherapy or local measures such as surgical curettage, low-dose radiotherapy and intralesional injection of corticosteroids. There are no controlled studies in the literature comparing outcomes of these treatment modalities because of low incidence of the disease and general tendency to regress. Three patients (female, 28 months; male, 9 years; male, 15 years) with LLCH of the mandible were treated in an one stage procedure with intralesional injection of 80, 40 and 80 mg methylpredisolone succinate, respectively, as the primary form of treatment. Patients were seen for clinical and radiological evolution 1, 3, 6, 9 and 12 months after treatment, and yearly thereafter. The overall outcome was excellent. The lesions showed clinically and radiologically complete remission approximately 6 months after treatment. There were no complications nor morbidity of the treatment. After a follow-up period of 12 months, the patients are well with no evidence of residual disease on radiographs. Local injection of corticosteroids should be the initial choice of treatment of LLCH of the mandible, because of the favourable treatment outcome in this disease and possible complications and lasting effects of surgery, radiotherapy and chemotherapy.
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PMID:Intralesional infiltration of corticosteroids in the treatment of localised langerhans' cell histiocytosis of the mandible Report of known cases and three new cases. 1605 80

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

The authors report the case of a large left occipital mass lesion in an 8-month-old boy who presented with seizure. Neuroimaging demonstrated an approximately 5-cm extraaxial tumor, and the patient underwent partial resection. The tumor was strongly attached to the tentorium and falx. In the postoperative course the residual lesion regressed spontaneously, and after 5 years only a slight residual tumor remained along the tentorium. Histopathological examination of the tumor revealed non-Langerhans cell histiocytosis (non-LCH). However, the tumor was not diagnosed as juvenile xanthogranuloma (JXG) because it lacked Touton giant cells. Hence, the authors described this lesion as a fibroxanthogranuloma. Most intracraniospinal non-LCHs have been reported as JXG; however, several cases of xanthomatous tumors with histopathological features resembling those of JXG have been described as fibrous xanthoma, xanthoma, fibroxanthoma, and xanthogranuloma. Among JXG and the xanthomatous tumors, a review of the literature revealed several cases of dural-based tumors; these dural-based tumors have had favorable courses, including the case described in this report. In addition, the patient in the present case experienced spontaneous regression of the residual tumor. The authors report this unique case and review the literature on isolated intracraniospinal non-LCHs, especially in cases of dural-based lesion.
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PMID:Spontaneous regression of infantile dural-based non-Langerhans cell histiocytosis after surgery: case report. 2563 22