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)

Between 1985 and 1989, 395 patients with primary lung cancer were treated at the Percy military teaching hospital, Paris. Among them were 16 patients (4%) aged between 29 and 40 years (mean: 34 years) and smokers (mean cumulative tobacco consumption 24 P.A.). Six of these (37%) had lung parenchyma dystrophy with bilateral apical bullae of emphysema, 1 had a clean cavity left by a previous lung abscess and 1 had microcytic fibrosis resulting from histiocytosis X of the lungs and bones. In all cases cancer had developed in contact with bullous lesions or sequelae. Cancer was discovered during radiological mass screening in 6 cases (37.5%), on the finding of systemic or thoracic symptoms in 5 cases and because of a distant metastasis in 5 other cases. Pathological examination revealed an adenocarcinoma in 8 out of 16 patients and only one small-cell carcinoma. Nearly two-thirds of the patients had reached an advanced stage: 3 were in stage III A, 1 in stage III B and 6 in stage IV. Eight patients underwent curative surgery (7 lobectomies, 1 pneumonectomy). Survival was known with precision in 14 patients: 8 died after a mean follow-up of 15 months (range: 3 and 31 months); 3 were alive with an active cancer and 4 are still alive in complete remission after curative surgery. Six published studies totalling 387 cases are concordant in demonstrating that primary lung cancer is severe in adults below 40 years of age and that surgery is useful in such cases, even with N2 lymph node involvement.
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PMID:[Primary bronchial cancer in subjects aged 40 or younger]. 166 14

A case of Langerhans' cell granulomatosis associated with gastric adenocarcinoma is reported. A review of the literature demonstrate an association of this entity with Hodgkin or non-Hodgkin lymphomas alone. The discussion is centred on differential diagnosis from the pseudo-sarcoid granulomatous reaction. Further reports may assist in classifying the granulomatous reaction to tumours into two types, epithelioid and Langerhans.
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PMID:Solitary Langerhans' cell granulomatosis of the stomach associated with gastric carcinoma. 393 69

Cytological, immunohistological and electron microscopical observation of 21 percutaneous fine needle punctures of retroperitoneal, pelvic and abdominal lymph nodes after borderline lymphography and computer tomography and 6 punctures of tumours after tomography allowed classification of primary metastases from the small pelvis in 14 patients and characterized tumours in 4 patients, which could not be demarcated by sonography. We distinguished yolk sarcoma metastasis, prostate gland cancer metastasis, three cases of nodular metastases of seminoma cells, and two metastases of melanoma. Malignant cells of Hodgkin's lymphogranuloma and non-Hodgkin's lymphoma were distinguished in seven samples of fine needle puncture. We found malignant cells of adenocarcinoma, T-immunoblastoma, pancreas carcinoma and histiocytosis X in four punctures of tumours. Fine needle puncture processed for electron microscopy with buffered fixation and harvested into Lowicryl K4M resin through centrifugation makes it possible to detect even the minimum of cells present, preserves the structure of cells and enables to correlate cytological findings in semithick sections with correspond ultrastructure in followed series of semithin sections.
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PMID:Malignant cells revealed in fine needle punctures of lymph nodes and tumours by electronmicroscopical methods. 890 20

The health risks associated with cigarette smoking are well known. Cigarette smoking is the most important causative factor in the development of bronchogenic carcinoma. Pulmonary diseases such as chronic bronchitis, centrilobular and panacinar emphysema, respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), and pulmonary Langerhans cell histiocytosis are also related to cigarette smoking. In adenocarcinoma and squamous cell carcinoma, the most common manifestation at computed tomography (CT) is a solitary pulmonary nodule; in small cell carcinoma, hilar and mediastinal adenopathy secondary to metastases; and in large cell carcinoma, a mass with central necrosis or cavitation in the lung periphery. For chronic bronchitis, the most common CT finding is bronchial wall thickening, but this finding is nonspecific. Emphysema, both centrilobular and panacinar associated with alpha-1-antitrypsin deficiency, usually manifests as areas of decreased attenuation and may involve bullous changes. However, centrilobular emphysema more commonly involves the upper lungs, whereas panacinar emphysema more commonly involves the lower lungs. Most patients with RB-ILD have normal high-resolution CT scans; however, abnormalities may be present, the most common of which are areas of ground-glass attenuation. CT appearance of pulmonary Langerhans cell histiocytosis varies depending on the disease stage: In the early stage, typical CT findings include multiple nodules, usually accompanied by cystic spaces with thin, well-defined walls. As the disease progresses, the cystic spaces become more numerous and the number of nodules decreases.
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PMID:Cigarette smoking: CT and pathologic findings of associated pulmonary diseases. 908 79

An unusual case of Langerhans cell histiocytosis in a 7-year-old female is presented. She had ultrastructural evidence of desmosomal biogenesis and formation of gland-like structures by lesional cells; their apical plasma membranes were folded into large numbers of microvilli. Despite the presence of these structures characteristic of epithelial cells, an infiltrated plaque on the abdominal skin of this patient was interpreted as cutaneous involvement of multiple system Langerhans cell histiocytosis because the immunohistochemical staining of the lesional cells for CD1a, S100, PNA, CD4, EN-4, and HLA-DR was positive, and numerous Birbeck granules were ultrastructurally identified in some lesional cells. Other clinical data included the presence of scaly erythematous skin lesions on the forehead and lytic osseous lesions in the maxilla, which were also histologically diagnosed as Langerhans cell histiocytosis. The absence of any internal malignancy in this patient readily ruled out the other diagnostic possibility of a metastatic adenocarcinoma showing glandular differentiation with brush border morphogenesis. The possibility that the desmosome-linked lesional epithelioid cells were actually cells of sweat glands entrapped in the histiocytic proliferation was also ruled out. The functional significance of the desmosomes and microvillous structures in the present case of Langerhans cell histiocytosis remains to be clarified. Awareness of this variant of Langerhans cell histiocytosis will be important for averting potential misdiagnosis in favor of epithelial tumors, especially metastatic adenocarcinomas.
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PMID:Langerhans cell histiocytosis (histiocytosis X) with morphologic expression of desmosomes and microvillous structures. 924 66

PURPOSE. The study was performed to evaluate the efficacy of fine needle aspiration biopsy (FNAB) in orbital lesions. METHODS. Seventeen patients with orbital masses who had been fully investigated by non-invasive techniques participated in this study. FNAB was performed by standard technique, as an outpatient procedure, with ultrasound guidance in lesions posterior to the equator. A trained cytologist analysed all the smears. RESULTS. Specific results were obtained in 14 of the 17 (82%) patients studied by FNAB. Ten cases were neoplastic (8 malignant and 2 benign), 3 were inflammatory and 1 was a case of histiocytosis X. In 3/17 cases the results were non-specific. These were treated as pseudotumours and responded well to systemic steroids. In 7 cases the clinical and radiological diagnosis was confirmed by FNAB. Non-invasive investigations like USG, CT and MRI, however, failed to provide accurate diagnosis in the other 7 (41%) cases. In these patients, FNAB yielded a pathological diagnosis (histiocytosis X, cryptococcosis, non Hodgkin's lymphoma, adenocarcinoma, pleomorphic adenoma, Schwannoma and cysticercosis), helping us to modify treatment with an excellent response. No significant complications were encountered following the aspiration biopsies. CONCLUSION. FNAB proved to be a reliable method for distinguishing between malignant and non-malignant lesions. It was found to be rapid, accurate, cost-effective, safe and a valuable addition to ultrasound, CT scan and MRI in the diagnosis of orbital lesions. This tool may help in avoiding a traumatic surgical intervention.
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PMID:Fine needle aspiration biopsy in orbital lesions. 1204 31

There exists a rarely observed association between pulmonary histiocytosis X and bronchopulmonary cancer. However, the frequency of bronchopulmonary cancer in these patients is higher than in the general population. A 28-year-old patient who currently smokes ten packs of cigarettes a year came to our department of pneumology with complains of cough and hemoptysis. An x-ray of the thorax revealed bilateral cysts and a shadow in the upper part of the right pulmonary field. In addition, a chest tomography showed multiple cysts dispersed throughout the two pulmonary fields and an irregular mass with a diameter of four centimetres in the upper right lobe. Bronchopulmonary adenocarcinoma was diagnosed during a cytologic exam of the bronchial washing. We decided to perform a thoracotomy on the patient, since there was no far metastasis. An upper lobectomy and wedge resection of the upper segment of the lower right lobe, which had been invaded by the tumour, were performed. Histology confirmed the diagnosis of adenocarcinoma. A pulmonary biopsy was carried out on the tumour-free site and showed the presence of histiocytosis X. There is a hypothesis that a neoplasm developed on the pulmonary fibrosis could be an epiphenomenon of bronchopulmonary cancer in patients who smoke and have pulmonary histiocytosis X. It is interesting to note that histiocytosis X and bronchopulmonary cancer were diagnosed at the same time, since the bronchopulmonary cancer may have occurred within a few years following the diagnosis of histiocytosis X, even if she was a smoker. Hemoptysis, which is found in 5% of patients with histiocytosis X, may suggest cancer. This young patient, a smoker, who complained of hemoptysis, is a particularly rare case of the association between pulmonary histiocytosis X and bronchopulmonary cancer whose pathogenesis is not clear cut. It is thus important to note that smoking can have major consequences, even in young people.
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PMID:[Histiocytosis X and bronchopulmonary adenocarcinoma: a rare coexistence]. 1252 89

Several premalignant and malignant neoplasms clinically appear as chronic eczematous lesions of the perianal region and have to be distinguished from benign processes. Anal intraepithelial neoplasia (AIN) presenting as bowenoid papulosis and perianal Bowen disease is a precursor lesion for invasive squamous cell carcinoma. When AIN is widespread, persistent or progressive, an underlying immunosuppression should get excluded. Verrucous carcinoma of the perianal region is a highly differentiated variant of squamous cell carcinoma characterized as a slowly growing but locally invasive tumor. Extramammary Paget disease (EMP) is an intraepithelial adenocarcinoma of the skin with apocrine differentiation. Along with EMP, basal cell carcinoma, Langerhans cell histiocytosis and cutaneous T-cell lymphoma should be included in the differential diagnosis for lesions simulating chronic perianal dermatitis. In such situations, biopsies and histopathologic examination are imperative to avoid delayed diagnosis and assure prompt therapy.
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PMID:[Differential diagnosis of chronic perianal dermatitis. Premalignant and malignant disorders]. 1502 33

Pseudoachalasia is a rare clinical entity which has clinical, radiographic and manometric features often indistinguishable from achalasia. A small primary adenocarcinoma arising at the gastroesophageal junction or a tumor of the distal esophagus are the most frequent causes. Rarely, processes other than esophagogastric cancers may lead to the development of pseudoachalasia. We present three cases of pseudoachalasia in which the primary cause of the disease was not an esophagogastric cancer. The causes were a pancreatic carcinoma, a breast cancer and an histiocytosis X. Aspects of these three patients' diagnostic and therapeutic course are discussed in detail.
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PMID:Pseudoachalasia: not only esophago-gastric cancer. 1743 2

We report a rare case of coexisting pulmonary adenocarcinoma and Langerhans' cell histiocytosis (LCH) in a 78-year-old woman who did not smoke. During follow-up of diabetes mellitus, she had complained of chest pain and was found to have a nodular lesion in S9 of the left lower lobe, which was resected surgically. No abnormal laboratory findings were obtained. Before surgical resection, needle biopsy specimens confirmed the existence of adenocarcinoma. The resected tumor in the left lower lobe was 3.0 x 1.8 x 3.0 cm, and histologically both acinar and bronchioloalveolar cell subtypes of adenocarcinoma were found in cancer foci. In addition to pulmonary adenocarcinoma, Langerhans' cell proliferation associated with marked eosinophil infiltration was incidentally found in a small nodule, approximately 3 x 2 mm in size in the subpleural region. The Langerhans' cells contained interdigitated nuclei, exhibiting rather clear nucleoplasm and cytoplasm; they were positive for S-100 protein, CD1a, and also CD4. Massive eosinophil infiltration was found around the focus of Langerhans' cell proliferation. This nodule appeared to be LCH. The adenocarcinoma and LCH were adjacent, and cancer cells were infiltrated only in the peripheral parts of LCH. The coexistence of adenocarcinoma and LCH appeared to be incidental. The association of adenocarcinoma and LCH is rare, and only several reports of it can be found in the English literature.
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PMID:A rare case of coexistence of pulmonary adenocarcinoma with Langerhans' cell histiocytosis. 1880 45


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