Primary intrathoracic lymphangioma masquerading as teratoma

Two cases of cystic hygroma arising as a primary lesion in the chest are presented. Because of calcifications discovered on CT and because of the anterior location of the mass in both cases, teratoma was the first diagnostic consideration. However, after surgical removal, histologic study determined that both lesions were cystic hygromas with areas of scattered calcification probably secondary to hemorrhage. These cases are presented because of the remarkable similarity of their imaging features to those of teratoma.     

Segmental reflex sympathetic dystrophy: clinical and scintigraphic criteria

Clinical and scintigraphic criteria are proposed for the diagnosis of segmental reflex sympathetic dystrophy. Eight patients met previously described clinical criteria for reflex sympathetic dystrophy with involvement limited to only a portion of the hand. The delayed phase of the three-phase radionuclide bone scan was found to be highly sensitive (100%) for this small group of patients. Consecutive bone scans (n = 127) performed during a 6-month period for a variety of upper extremity problems were reviewed, and a segmentally diffuse pattern of tracer uptake was found to be highly specific (98%) for segmental reflex sympathetic dystrophy. Recognition and documentation of a more localized form of reflex sympathetic dystrophy will allow earlier recognition and treatment, which is an important factor in a successful outcome for managing pain dysfunction disorders.     

Global folds of highly deuterated, methyl-protonated proteins by multidimensional NMR

The development of 15N, 13C, 2H multidimensional NMR spectroscopy has facilitated the assignment of backbone and side chain resonances of proteins and protein complexes with molecular masses of over 30 kDa. The success of these methods has been achieved through the production of highly deuterated proteins; replacing carbon-bound protons with deuterons significantly improves the sensitivity of many of the experiments used in chemical shift assignment. Unfortunately, uniform deuteration also radically depletes the number of interproton distance restraints available for structure determination, degrading the quality of the resulting structures. Here we describe an approach for improving the precision and accuracy of global folds determined from highly deuterated proteins through the use of deuterated, selectively methyl-protonated samples. This labeling profile maintains the efficiency of triple-resonance NMR experiments while retaining a sufficient number of protons at locations where they can be used to establish NOE-based contacts between different elements of secondary structure. We evaluate how this deuteration scheme affects the sensitivity and resolution of experiments used to assign 15N, 13C, and 1H chemical shifts and interproton NOEs. This approach is tested experimentally on a 14 kDa SH2/phosphopeptide complex, and a global protein fold is obtained from a set of methyl-methyl, methyl-NH, and NH-NH distance restraints. We demonstrate that the inclusion of methyl-NH and methyl-methyl distance restraints greatly improves the precision and accuracy of structures relative to those generated with only NH-NH distance restraints. Finally, we examine the general applicability of this approach by determining the structures of several proteins with molecular masses of up to 40 kDa from simulated distance and dihedral angle restraint tables.     

Optic chiasm astrocytomas of childhood. 1. Long-term follow-up

There are many in vivo animal models for studying airway mucus secretion and hypersecretion, each with advantages and disadvantages. Use of a particular test system will depend upon the aspect of secretion to be modelled. Airway hypersecretory diseases exhibit chronic mucus hypersecretion, of which the clinical impact is predominantly in the distal airways. The majority of documented test preparations study acute secretion, invariably using tracheal preparations, but have been invaluable in elucidating the normal physiology of airway mucus secretion. Chronic models of the hypersecretory state in the distal airways have been developed, but are predominantly histologic in nature (for example quantification of increased goblet cell number). There are few investigations of mucus hypersecretion. Examination of the 'antisecretory' potential of pharmaceutical compounds has been investigated predominantly in chronic histologic models with the drug being given 'prophylactically' rather than 'therapeutically'. Refinement of chronic hypersecretory models should lead to elucidation of the connection between airway irritation, inflammation, MUC gene expression, mucous cell hyperplasia/metaplasia, airway hypersecretion and bronchial hypersecretory disease.     

Does the establishment of momentum lead to athletic improvement?

PURPOSE: To determine the potential efficacy of radio-frequency (RF) ablation of liver metastases during long-term follow-up. MATERIALS AND METHODS: Sixteen patients with 31 hepatic metastases were treated with percutaneous, ultrasound-guided RF ablation. RF was applied to monopolar electrodes (2-3-cm tip exposure) either individually or within a multiprobe array (two to four probes) for 6 minutes at 90 degrees C over one to four treatment sessions per metastasis. RESULTS: In only one of 75 sessions, a moderate complication, self-limited intraperitoneal hemorrhage, was observed. Four patients (four lesions) underwent surgical resection 15-60 days after RF treatment. Residual, viable tumor was seen in all of these patients. The remaining 12 patients were followed up for 9-29 months (mean, 18.1 months). In these patients, 18 of 27 lesions remained stable or decreased in size and showed no enhancement at computed tomography and/or magnetic resonance imaging for at least 9 months. Two patients died of disseminated disease at 13 months and one at 16 months. Disease-free survival was achieved in eight patients. CONCLUSIONS: RF ablation appears to be a simple, safe, and potentially effective treatment for selected patients with liver metastases and may become a less invasive alternative to surgical therapy.     

Primary prevention of malignant melanoma in the Stockholm Cancer Prevention Programme

The relation between intracoronary thrombus and endothelin-1 (ET-1) was studied. In a canine model, acute myocardial infarction (MI) was induced by coronary occlusive thrombus produced at a mock atheromatous plaque. Blood samples were collected from the aorta (A) and coronary vein (V). Twenty-eight open-chest dogs divided into three groups were studied. Group I (n = 15): acute MI was induced by coronary occlusive thrombus, and thrombolysis was obtained by urokinase two hours after MI. Group II (n = 8): nonocclusive thrombus was produced without inducing MI. Group III (n = 5): coronary artery was ligated for two hours and reperfused by release of ligation. In Group I, ET-1 was significantly increased after MI in A and V, and ET-1 in V was significantly more elevated than in A during thrombolysis, suggesting ET-1 production in the coronary vessels by thrombolysis. In Group II, ET-1 increased slightly during thrombus formation, but there was no difference in A and V. In Group III, ET-1 was elevated significantly after MI without A and V difference. These results indicate that there is no detectable ET-1 production with coronary thrombus formation, whereas coronary ET-1 production is detected during thrombolysis, most probably because resolved thrombus releases a more potent stimulus to ET-1 production.     

Human chromosome 5 sequence primer amplifies Alu polymorphisms on chromosomes 2 and 17

Members of the Alu family of repetitive elements occur frequently in the human genome and are often polymorphic. Techniques involving Alu element mediated polymerase chain reactions (Alu PCR) allow the isolation of region-specific human DNA fragments from mixed DNA sources. Such fragments are a source of region-specific Alu elements useful for the detection of Alu-related polymorphisms. A clone from human chromosome 5, corresponding to locus D5F40S1, was isolated using Alu PCR differential hybridization. Alu elements within this clone were investigated for the presence of potentially polymorphic 3' polyA tails. Primers were devised to amplify the 3' polyA tail of an Alu element present within the clone. One primer, D5F40S1-T, was specific to the DNA flanking the 3' end of the Alu element, and the other primer was homologous to sequences within the element. When these primers were used in PCR reactions, products from chromosomes 2 and 17 (loci D2F40S2 and D17F40S3) were amplified in addition to the expected product from chromosome 5. The most likely explanation for this nonspecific amplification is that the D5F40S1-T primer is located within a low-copy repetitive element that is 3' of the Alu element. This phenomenon presents a potential problem for the identification of region-specific Alu polymorphisms.     

Pediatric malignant glioma with tubuloreticular inclusions and MYCN amplification. Report of a case with immunohistochemical, ultrastructural, flow cytometric, karyotypic, and Southern blot analysis

BACKGROUND: The authors described unusual pathologic features in a left frontal lobe malignant glioma in a 31/2-year-old boy. The pathology was similar in the initial excision and two subsequent recurrences at 9 and 11 months and at autopsy, when extensive subarachnoid spread was noted. METHODS: The tumor was studied by conventional histology, immunohistochemistry, flow cytometry, transmission electron microscopy (TEM), immune electron microscopy (IEM), and cytogenetic and Southern blot analysis. RESULTS: The tumor revealed two different histologic patterns. One component showed large cells with eosinophilic cytoplasm, vesicular nuclei with prominent nucleoli, eosinophilic perinuclear inclusions, and immunoreactivity for glial fibrillary acidic protein (GFAP) and vimentin. The other component consisted of undifferentiated cells with hyperchromatic nuclei and scanty cytoplasm. By TEM, the perinuclear aggregates were composed of tubuloreticular inclusions, which were also observed in endothelial cells within the tumor vasculature. By IEM, the intermediate filaments in the tumor cell cytoplasm were decorated with GFAP. Flow cytometric results revealed a marked increase in the S-phase (48%), whereas cytogenetic analysis of short-term cultures showed an abnormal karyotype containing marker chromosomes and double minutes. In the second resection, additional karyotypic abnormalities were noted, including 1p- and several additional markers. The first and second resections showed MYCN amplification by Southern Blot analysis in the 60- to 80-fold range. CONCLUSIONS: This tumor presents unique histologic, ultrastructural, and cytogenetic findings as well as MYCN amplification that is notable for a pediatric malignant glioma. Tubuloreticular inclusions were a prominent feature in this tumor, which again is unique for a glioma.