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)

The light-microscopic and ultrastructural findings in a case of so-called giant cell tumor of soft parts, localized at the dorsal side of the left foot of a 23-years-old male are described. An amputation of the lower extremity was performed and subsequently chemotherapy with adriamycin was given for 3 months. Despite the histology and cytologic malignant appearance and the evident vascular invasion, already present at the time of the first excision, the last known status 2 years later seems favorable. There are no pathologic lymph nodes in the groins and no signs of metastases on chest X-rays. From the electron-microscope study no definite conclusion can be drawn as regards the histogenesis of this tumor. we feel, as do others, that many of the principal mononuclear tumor cells are poorly differentiated mesenchymal cells. Some of the neoplastic cells, however, show ultrastructural features suggestive of chondro- or osteoblasts (a well-developed r.e.r. containing electron-dense material; multiple Golgi complexes; masses of glycogen; interdigitating cell membrane villi; cytoplasmic filaments; an extracellular amorphous matrix). Some of the larger tumor cells have the submicroscopic aspects of histiocytes as described in osseous, cutaneous, or pulmonary lesions of the histiocytosis X group. As yet undetermined cytoplasmic inclusion bodies constitute another rare observation in our material.
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PMID:Giant cell tumor of soft parts. An ultrastructural study. 18 82

Rhabdomyosarcoma usually presents as a soft tissue mass, which may invade adjacent bone. However, the patient presents occasionally with bony metastases. Recognition of these is important for staging and management. Fifty-eight cases have been reviewed; 14 of these had local bone invasion by the soft tissue tumour. All bones involvel were flat bones; 12 showed permeated bone destruction and two showed geographic destruction. Bone expansion was seen in half the involved bones. Twelve of the 58 cases showed secondary bone deposits, which were the presenting feature in five. Although 10 cases had permeated bone destruction, two were very well defined with a wide range of radiological appearances. The radiological differential diagnosis includes neuroblastoma, leukaemic infiltration, lymphoma, histiocytosis X, solitary and multifocal osteosarcoma and other deposits.
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PMID:The radiology of bone changes in rhabdomyosarcoma. 62 1

Histiocytosis X describes a disease characterized by histiocytic infiltration of the reticuloendothelial system, skin, bones, and pituitary gland. The disseminated form frequently occurs in infants and children. Chemotherapy has significantly improved the prognosis in this disorder. Sixty-three per cent of survivors, however, have some residual disability related to fibrosis of tissues previously infiltrated by histiocytes. In instances of liver involvement, healing by fibrosis may result in cirrhosis with portal hypertension and bleeding esophageal varices. Clinical findings include hepatosplenomegaly, jaundice, ascites, hypoalbuminemia, prolonged prothrombin time, and Bromsulphalein retention. Histologic examination of the liver shows a characteristic dense "macronodular" periportal cirrhotic pattern. Three children with portal hypertension and bleeding varices due to healed histiocytosis X were sucessfully managed by portosystemic shunt procedures. Portacaval, mesocaval, and central splenorenal shunts were equally effective in relieving poral hypertension. These children had neither recurrence of bleeding nor evidence of encephalopathy. Two children remain well whereas in one patient a primary hepatoma developed fourteen years posthung and he died of pulmonary metastases. Portosystemic shunt procedures effectively relieve the threat of potentially fatal variceal hemorrhage and improve the opportunity for long-term survival in children with cirrhosis and portal hypertension due to healed histiocytosis X.
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PMID:Portal hypertension in infants and children with histiocytosis X. 108 50

The endoscopic examination of the tracheobronchial tree is most helpful in the diagnosis and staging of bronchial carcinoma. Tumors that are endoscopically visible may be confirmed in more than 95% of the cases. In localized peripheral tumors, the diagnostic yield of bronchoscopy is significantly lower; for peripheral metastases, only about 10%. In diffuse interstitial pulmonary diseases other than malignancies, some infections, and histiocytosis X, bronchoscopy including transbronchial biopsy is less successful.
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PMID:The diagnostic yield of bronchoscopy. 204 23

A retrospective study was undertaken of 1005 normal contrast-enhanced head computed tomographic (CT) scans in children to determine normal standards for pituitary stalk diameter, basilar artery diameter, and their ratio for age and gender. The pituitary stalk enlarges with age, and girls have larger stalks than do boys after age 8. The pituitary stalk-to-basilar artery ratio is easily estimated visually. Ratios greater than or equal to 1 are unusual in normal children. A ratio greater than or equal to 1 should prompt direct measurement of the stalk and comparison with age-matched normal values. If the stalk measurement is greater than 2 SD above the age-matched mean, it is presumably abnormal and deserves further evaluation for numerous possible pathologic conditions including histiocytosis X, pituitary adenoma, hypothalamic lesions, Rathke cleft cysts, metastases, sarcoidosis, hypothyroidism, and infection.
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PMID:Normal pituitary stalk size in children: CT study. 387 41

Seven cases are presented in which enlargement of the pituitary stalk was demonstrated by computed tomography (CT). Histiocytosis X, sarcoidosis, and metastatic cancer were the proven or presumed causes. The discovery of pituitary stalk enlargement prompted radiation treatment in three patients and led to the diagnosis of previously unsuspected diabetes insipidus in one.
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PMID:CT of the abnormal pituitary stalk. 642 Nov 24

We describe 12 patients with simultaneous bilateral spontaneous pneumothorax (SBSP). They represent 4 percent of patients with spontaneous pneumothorax seen at our hospital from 1971 to 1990. Five of the 12 had no underlying lung disease. In the seven remaining patients, SBSP was secondary to histiocytosis X, lymphangioleiomyomatosis, osteogenic sarcoma with pleural and pulmonary metastases, Hodgkin's disease, mesothelioma, cystic fibrosis, or miliary tuberculosis. Nineteen of the 56 patients with SBSP (34 percent) described in the literature (this series included) had pulmonary disease related to disorders of cells of mesenchymal origin. Emphysema and bullous lung disease were not associated with SBSP. Long-term prognosis was a function of pulmonary status. Four of the patients described herein died during the period reviewed. All suffered from severe underlying disease. In no case was SBSP the main cause of death. With timely treatment, the short-term prognosis is benign even for patients with underlying lung disease. Surgical pleurectomy should be attempted early, especially in SBSP secondary to underlying lung disease.
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PMID:Simultaneous bilateral spontaneous pneumothorax. 816 40

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

In 1973, a 10 year old boy presented with numerous bilateral lung nodules, diagnosed as histiocytosis X by open lung biopsy. The patient was treated with prednisone until 1984. In 1993, he developed severe pain in the neck. A biopsy of the spine revealed the same tumour morphology as was seen in the lung in 1973. Immunohistological examination of the former and present biopsy led to the definitive diagnosis of epithelioid haemangioendothelioma of the lung with metastases to spine and liver. Epithelioid haemangioendothelioma of the lung is a rare soft tissue tumour of vascular origin, readily mistaken for carcinoma or, as in this case, histiocytosis. The tumour has an intermediate malignant potential. Although metastases of epithelioid haemangioendothelioma of the lung are well-known, metastatic spread to bones, as in our case, has not previously been mentioned in the literature.
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PMID:Epithelioid haemangioendothelioma of the lung: clinical and pathological pitfalls. 857 93

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


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