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)

Histiocytosis X is a disease of unknown etiology, characterized by a mass of proliferating histiocytes, plasma cells and inflammatory cells foaming a granuloma within the reticuloendothelial elements of any organ in the body. In the central nervous system (CNS), hypothalamic disorder of histiocytosis X is often found, but histiocytosis X in other regions is quite rare. We report a case of a 5-year-old girl with histiocytosis X of the zygoma presenting as a mass lesion in the tentorium cerebelli. A computed tomographic (CT) scan demonstrated a tumor at the left tentorial region, extending along the dura mater of the tentorium cerebelli. Magnetic resonance imaging (MRI) revealed a low signal intensity region on both T1 and T2-weighted images. MRI with Gd-DTPA showed a homogeneous enhanced mass extending to right and inferior sites with a thickened tentorium. As the thickened dura matter continued from the left middle fossa to the mass lesion, the tumor was considered to arise from the left zygoma and extend to the tentorium cerebelli. CNS extension of histiocytosis X is manifested either as (1) the cerebral type or (2) the dural type. Many cases of cerebral type histiocytosis X including hypothalamic disorder have been reported. Only 6 cases of the dural type of histiocytosis X have been described. Although the lesions of the cerebral type of histiocytosis X show prolonged T1 and T2 values on MRI, the MRI findings of the dural type have not been reported. The present case is the first report of the appearance of the lesion on MRI.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of dural type of histiocytosis X presenting as a mass lesion in the tentorium cerebelli]. 819 35

Primary Langerhans cell histiocytosis (LCH) of the female genital tract is very uncommon. Since the natural history of this tumor is unpredictable, with spontaneous remissions and exacerbations, establishment of the diagnosis is often difficult. In the few reported cases of LCH involving the vulva there were systemic manifestations of the disease. We report the clinical and histopathological findings in the case of a 36-year-old woman who presented with a 9-year history of vulvar lesions. The diagnosis of LCH was established by immunohistochemical techniques, which demonstrated sheets of S-100 protein-positive histiocytes in the dermal tumor. The patient received radiotherapy to the vulva and responded with complete remission.
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PMID:Primary Langerhans cell histiocytosis of the vulva. 824 96

A solitary massive tumor developed in the left eye of a 61-year-old female who had a history of insulin-independent diabetes mellitus for 20 years and whose left eye had become blind due to repeated anterior uveitis in the recent several years. The eyeball was enucleated and used for further examinations. Intraocular tissues were widely infiltrated by histiocytes, though no extraocular invasion or further metastasis was found. Histological examination including alpha 1-anti-chymotrypsin and S100 staining revealed the features characteristic of histiocytosis X. This is probably a case of intraocular histiocytosis X with no evidence of systemic symptoms.
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PMID:[A case of intraocular histiocytosis X]. 833 72

Many pediatric centers are beginning to accumulate a large experience in the use of thallium-201 (201Tl) imaging with 201Tl requires a state-of-the-art high-resolution gamma camera computer system with single photon emission computed tomography (SPECT) capability and a physician-directed tailored examination. Tumor imaging with 201Tl, with its multifactorial localization mechanisms that are different from those for gallium-67, offers a distinct advantage over gallium tumor imaging with a short total imaging time. Tumors are variable in avidity and intensity of thallium uptake. Primary and metastatic disease can be detected with 201Tl scintigraphy. Baseline pretreatment determination of thallium avidity is crucial to its efficacy in therapeutic response assessment. Adjunctive SPECT imaging provides greater sensitivity for lesion detection and direct comparison of physiology (thallium uptake) with anatomy (computed tomography and magnetic resonance imaging). The sensitivity and specificity for detection of pediatric brain tumors has been reported as 77% and 93%, respectively. Thallium-201 brain SPECT also provides a less expensive and more readily available alternative to positron emission tomography for assessing the functional state of pediatric brain tumors. Extremity osteogenic sarcoma and Ewing's sarcoma have 100% sensitivity for 201Tl uptake pretreatment. Early results confirm an association between 201Tl uptake and histological tumor response. The determination of residual/recurrent disease versus thymic rebound and other nonneoplastic change in thallium-avid lymphoma, rhabdomyosarcoma, and germ cell tumors that involve the thorax can be confirmed with a 201Tl SPECT examination. Soft-tissue tumors elsewhere in the body may be detected with 201Tl scintigraphy. Thallium-201 does not exhibit 100% specificity for tumors. False-positive 201Tl uptake has been seen in histiocytosis X, benign bone tumors, stress fractures, and inflammation.
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PMID:Thallium-201 for oncological imaging in children. 837 97

Indeterminate and interdigitating cell tumors are rare proliferations of immunoregulatory cells that demonstrate morphologic, immunologic, and ultrastructural features similar to their cells of origin. Although an association of lymphoproliferative disease with Langerhans' cell histiocytosis is well described, only sporadic cases of non-Langerhans' dendritic cell proliferations have been published. The authors describe three patients with low grade B-cell lymphoproliferative disease who developed subsequent indeterminate cell or interdigitating cell tumors. When the two cases of indeterminate cell tumor are added to those previously described in the literature, it appears that 4 of 13 cases (31%) are associated with a history of low grade B-cell malignancy. Possible explanations for the relationship between these two disorders are discussed.
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PMID:Dendritic cell tumors associated with low-grade B-cell malignancies. Report of three cases. 852 15

A young child was admitted with a cauda equina syndrome believed to be progressive, but unrelated to a congenital anomaly. Magnetic resonance imaging studies revealed the presence of an intradural tumor in the cauda equina. A yellow pasty tumor with nerve root infiltration was identified at surgery. Histologic features were pathognomonic for histiocytosis X. The patient's management and review of the literature are discussed.
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PMID:Cauda equina histiocytosis X. 873 8

Langerhans cell histiocytosis is an infrequent and enigmatic proliferative disorder that commonly presents in the head and neck region. This is an analysis of 77 patients with Langerhans cell histiocytosis treated at Children's Hospital and Dana Farber Cancer Institute from 1974 through 1993. The study focused on clinical findings, anatomic location and extent of disease, therapy, and outcome. The patients were, on average, under 5 years of age at initial presentation. Over 62 percent of the patients had signs and symptoms referred to the craniofacial skeleton. Osteolytic lesions of the cranium were the most common, followed, in frequency, by scalp rash, osteolytic mandibular tumor(s), enlarged nodes, and gingival swelling or ulceration. Single bony lesions usually were treated with curettage or radiotherapy. Chemotherapy was used commonly for advanced disease with multifocal or disseminated presentation. Initial therapy included moderate doses of single agents; other agents were added if no response was achieved. The natural history of Langerhans cell histiocytosis varied from an acute fulminant course, a waxing and waning chronic disease, to spontaneous regression. Young age at presentation and organ dysfunction predicted a poor prognosis. Statistical analysis showed that there was no significant relationship between outcome and extent of skeletal involvement when controlling for age or organ dysfunction.
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PMID:Langerhans cell histiocytosis: an uncommon disease commonly manifesting in the craniofacial skeleton. 876 8

The incidence, risk factors, and outcome of posttransplant lymphoproliferative disease (PTLD) were examined for 298 children undergoing liver transplantation. The overall incidence of PTLD was 8.4% (25 of 298). Intensity of immunosuppression was found to be a major risk factor for the development of PTLD. Cyclosporine and tacrolimus when used as primary immunosuppression were associated with the development of PTLD in 4.3% and 6.6% of cases (P=NS). OKT3 and tacrolimus, when used as rescue therapy for steroid-resistant rejection, were associated with a comparable increase in the risk of developing PTLD (10.9% and 11.1%, P=NS). Patients requiring both OKT3 and tacrolimus to treat refractory rejection were at significantly increased risk for PTLD (28.1% vs. 4.3% or 6.6%, P<0.0001). PTLD was more common in patients who received transplants for Langerhans cell histiocytosis relative to other indications for transplantation (66% vs. 8.4%, P=0.0005). The data also support an association between primary Epstein-Barr virus (EBV) infections following transplantation and the development of PTLD. While only three patients were EBV seropositive before transplantation (14%), 19 patients were EBV seropositive at the time of diagnosis of PTLD (90%), confirming a high incidence of primary EBV infections in patients with PTLD (21 patients had both pre- and posttransplant EBV serologies). In this series, PTLD was associated with a mortality rate of 60%, and 12 of the 15 patients who died had persistent tumor at the time of death. Five of the 13 patients rendered disease-free developed ductopenic rejection. Of the four with severe liver dysfunction, two have undergone successful retransplantation and are alive without evidence of PTLD. In conclusion, intense immunosuppression using OKT3 and tacrolimus as rescue agents was associated with a significant increase in the incidence of PTLD. Primary EBV infection after transplantation further accentuated this risk. Independent of these risk factors, patients with Langerhans cell histiocytosis were at significantly increased risk for PTLD. The identification of high-risk patients should allow the development of protocols to screen patients for primary EBV infections and early indications of PTLD, as well as the institution of preemptive antiviral and antitumor therapies.
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PMID:Posttransplant lymphoproliferative disease in pediatric liver transplantation. Interplay between primary Epstein-Barr virus infection and immunosuppression. 877 85

The cutaneous histiocytoses are best divided in the Langerhans' cell histiocytoses and non-Langerhans' cell histiocytoses. In the former group, the cells react with S100 and CD1a antibodies, while in the latter group they express a variety of macrophage markers. Xanthogranuloma is a frequent childhood tumor and the only common non-Langerhans' cell histiocytosis. Xanthogranulomas contain a mixture of several different types of histiocytes that also appear in more pure forms as both solitary tumors and disseminated processes. The varying histiocyte morphology provides a unifying concept for non-Langerhans' cell histiocytoses.
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PMID:The non-Langerhans' cell histiocytoses in childhood. 888 34

Four brief reports of combined skin lesions are presented: (1) combined basal cell epithelioma and histiocytosis X. (2) combined intraepidermal melanocytic neoplasia (melanoma in situ) and leukaemia cutis, (3) combined melanocytic naevus with pseudoepitheliomatous hyperplasia and desmoplastic trichoepithelioma, and (4) combined melanocytic naevus with lichenoid gold reaction. The importance of the occurrence of such lesions is discussed, as are the clinical and pathological diagnostic difficulties they sometimes pose.
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PMID:Combined skin lesions. 890


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