Giant rhabdomyoma of the right ventricle.

A giant intrathoracic mass causing foetal dysrhythmias, polyhydramnios and foetal hydrops necessitated a caesarean section in a male infant of 35 weeks gestation. Despite the benign histology of cardiac rhabdomyomas and the observation of spontaneous regression, there may be significant associated morbidity and mortality, especially in neonates. There is a high incidence of associated tuberous sclerosis.

Slight cognitive impairment and magnetic resonance imaging abnormalities but normal school levels in children treated for acute lymphoblastic leukemia with chemotherapy only.

OBJECTIVES: To investigate persistent neuropsychologic late effects in children treated for acute lymphoblastic leukemia at a young age with chemotherapy only by means of serial neuropsychologic assessments (NPAs), magnetic resonance imaging (MRI) of the brain, and evaluation of school levels. STUDY DESIGN: Consecutive patients (n = 17) had 2 extensive NPAs (12 psychometric measures) after cessation of therapy. Test results were compared with those of both healthy control subjects and 28 previously treated children who received cranial irradiation. MRI findings were related to test scores. School levels were evaluated in the patients and their healthy siblings. RESULTS: Initial participation (n = 17) and availability of the study group after 8 years of follow-up were 100%. Significant group differences between patients who received chemotherapy and healthy control subjects were found for memory and fine-motor functioning. The 17 patients combined showed 16 deficits on various test measures. MRI abnormalities were seen in 6 children, but these did not correlate with cognitive performance. No differences in school levels were seen when the patients who received chemotherapy were compared with their siblings. The current nonirradiated patients demonstrated significantly better test results and significantly fewer learning disabilities and MRI abnormalities than did the previously irradiated group. CONCLUSION: Treatment with chemotherapy only may be associated with some cognitive impairment. However, these children attained normal school levels.

Preoperative staging of non-small-cell lung cancer with positron-emission tomography.

BACKGROUND: Determining the stage of non-small-cell lung cancer often requires multiple preoperative tests and invasive procedures. Whole-body positron-emission tomography (PET) may simplify and improve the evaluation of patients with this tumor. METHODS: We prospectively compared the ability of a standard approach to staging (computed tomography [CT], ultrasonography, bone scanning, and, when indicated, needle biopsies) and one involving PET to detect metastases in mediastinal lymph nodes and at distant sites in 102 patients with resectable non-small-cell lung cancer. The presence of mediastinal metastatic disease was confirmed histopathologically. Distant metastases that were detected by PET were further evaluated by standard imaging tests and biopsies. Patients were followed postoperatively for six months by standard methods to detect occult metastases. Logistic-regression analysis was used to evaluate the ability of PET and CT to identify malignant mediastinal lymph nodes. RESULTS: The sensitivity and specificity of PET for the detection of mediastinal metastases were 91 percent (95 percent confidence interval, 81 to 100 percent) and 86 percent (95 percent confidence interval, 78 to 94 percent), respectively. The corresponding values for CT were 75 percent (95 percent confidence interval, 60 to 90 percent) and 66 percent (95 percent confidence interval, 55 to 77 percent). When the results of PET and CT were adjusted for each other, only PET results were positively correlated with the histopathological findings in mediastinal lymph nodes (P<0.001). PET identified distant metastases that had not been found by standard methods in 11 of 102 patients. The sensitivity and specificity of PET for the detection of both mediastinal and distant metastatic disease were 95 percent (95 percent confidence interval, 88 to 100 percent) and 83 percent (95 percent confidence interval, 74 to 92 percent), respectively. The use of PET to identify the stage of the disease resulted in a different stage from the one determined by standard methods in 62 patients: the stage was lowered in 20 and raised in 42. CONCLUSIONS: PET improves the rate of detection of local and distant metastases in patients with non-small-cell lung cancer.

Quantification of liver iron concentration with magnetic resonance imaging by combining T1-, T2-weighted spin echo sequences and a gradient echo sequence.

BACKGROUND: The aim of the study was to quantify hepatic iron by MRI for practical use. METHODS: In twenty-three patients with various degrees of iron overload, measurements were carried out with a 1.5 Tesla MR unit. A combination of pulse sequences (T1, T2 and gradient echo) enabled us to quantify smaller amounts of liver iron as accurately as larger amounts of liver iron. The gradient echo sequence provided us with a good correlation when detecting smaller amounts of iron in the liver where the T1 sequence provided a good correlation when larger amounts of iron were present. RESULTS: The combination of the three sequences showed a nice correlation (r=-0. 93, P<0.001) and provided us with an accurate estimate of the liver iron content (LIC). This correlation was achieved with a LIC from the lower range of normal up to LIC of 146 mmol/kg dry weight, which seems the highest measurable liver iron content for a 1.5 Tesla MRI. Measuring in the lower range makes it possible to decide whether further invasive diagnostic investigations by a liver biopsy are indicated. CONCLUSION: MRI is a useful tool to quantify iron overload non-invasively. In cases where a liver biopsy is hazardous MRI can easily be used to obtain reliable, quantitative information about the initial LIC. Quantification by MRI could also be used for follow up of the iron content during depletion treatment by phlebotomy or iron chelation. The stronger the magnet the more sensitive the detection of concentrations up to 150 mmol/kg is. A semi-quantitative judgement will only be possible with severe iron overload over 150 mmol/kg. If such an iron excess is found, a liver biopsy should be performed to exclude cirrhosis.

Familial congenital bilateral agenesis of the acromion: a radiologically illustrated case report.

Familial congenital bilateral acromion absence was found in four members of one family. Only one of them presented with gradually increasing pain in his left shoulder, resembling a shoulder impingement syndrome. The other members did not have any symptoms. This is the first report of familial occurrence of this extremely rare congenital anomaly.

[A swelling in the distal part of the thigh]. / Een zwelling distaal in het bovenbeen.

A swelling in the distal thigh may be caused by a tumour of bone or soft tissues, an aneurysm or an inflammation. Signs of inflammation may render interpretation more difficult. Four males aged 60, 48, 15 and 81 years had had a swelling in the popliteal space or above the knee, in some cases with pain. Supplementary examinations, especially MRI scanning and arteriography did not always result in a diagnosis. The diagnosis could be made, however, during operation: an empyemic thrombosed popliteal artery aneurysm, a liposarcoma, pyomyositis and suture dehiscence of a plastic prosthesis of the popliteal artery. After treatment, consisting of or started during the operation, uneventful recovery occurred.

11C-tyrosine position emission tomography and 1H magnetic resonance spectroscopy of the response of brain gliomas to radiotherapy.

We monitored 10 patients with unresected (9) or partially resected (1) supratentorial gliomas with 11C-tyrosine position emission tomography (TYR-PET) before and after radiotherapy. TYR-PET tumour volumes were measured using a threshold technique. In seven patients the tumour volume decreased after radiotherapy, although all gliomas persisted on TYR-PET images. In eight patients the tumour protein synthesis rate (PSR) was calculated using a dynamic study protocol in combination with a PATLAK analysis. There were no changes in PSR after radiotherapy, but the PSR was calculated on the remaining tumour volume using the same threshold technique as before therapy, i.e. the decrease in tumour volume was not taken into account. In eight patients the PET data were compared with magnetic resonance spectroscopic imaging (1H-MRSI) performed simultaneously. Although there was no statistically significant correlation between TYR-PET volume changes and 1H-MRSI choline level we observed a simultaneous decrease in volume and choline in four patients.

Radiological and functional assessment of radiation-induced lung injury in the rat.

The purpose of this study is to develop an experimental model to measure localized radiation-induced lung injury using multiple end-points including breathing frequency, high-resolution computed tomography (CT), and radionuclide perfusion. The rats were anesthetized and the right lung irradiated with a single dose of 18 Gy using 200-kVp x-rays. The lung function of the animals was measured every 2 weeks after irradiation with the breathing rate assay. CT scanning and radionuclide lung perfusion assay were performed prior to and 2, 4, 10, 16, and 34 weeks after irradiation. Significant elevation in breathing rate occurred after 16 weeks, with a maximal increase between 22 and 28 weeks. An increase in the right lung density started 4 weeks after irradiation. Regional measurements indicated a relatively uniform increase in density at 4 and 10 weeks, while foci of high-density areas were observed at the later time points. Changes in rat lung volume indicated shrinkage of the irradiated right lung and accompanying compensatory hypertrophy of the shielded left lung. Radionuclide perfusion assay showed significant decrease in relative blood flow in the irradiated right lung 4 weeks after hemithoracic irradiation. Changes in breathing rate provide an index of overall lung function while changes in lung density, volume, and perfusion are of particular importance for evaluating loco-regional differences in lung sensitivity. This study is the first demonstration that CT can be used to measure volume changes after thoracic irradiation in rats.

Noninvasive metabolic assessment of human donor livers: prognostic value of 31P-magnetic resonance spectroscopy for early graft function.

BACKGROUND: The purpose of this study was to investigate whether phosphorus-31 magnetic resonance spectroscopy (31P-MRS) of the isolated donor liver can serve as a viability indicator with prognostic value for transplantation outcome. METHODS: Forty human donor livers preserved with University of Wisconsin solution were studied shortly before transplantation. The respective spectral peak areas of the isolated donor liver were correlated with the amount of hepatocellular graft damage and liver metabolic function shortly after implantation. RESULTS: The individual phosphomonoesters, inorganic phosphate, phosphodiesters, and nicotine adenine dinucleotide peaks were not prognostic for postoperative hepatocellular damage or liver metabolic capacity. The presence of adenosine triphosphate, however, predicts a significantly better metabolic capacity to eliminate bilirubin, to synthesize fibrinogen and antithrombin III, and to maintain a better prothrombin time after transplantation. Furthermore, this study is probably the first 31P-MRS demonstration in the human liver of phosphocreatine. CONCLUSIONS: In the clinical setting described, metabolic assessment using 31P-MRS did not result in a reliable noninvasive test to predict primary graft dysfunction. Study of the role of phosphocreatine in liver metabolism during cold storage is needed.

Cystic hygroma of the shoulder region. A case report.

Cystic hygroma is a rare congenital malformation of the lymphatic system. The lesion is characterized by the formation of a cystic mass of variable size. Most lesions are present in infancy or early childhood and are found predominantly in the region of the head and neck. This report describes a case with an unusual location of the lesion: A 12-year-old boy with a cystic hygroma in the shoulder presented with shoulder pain and swelling. The diagnosis was made by magnetic resonance imaging and confirmed after histologic examination of the resected specimen. This uncommon localization and condition of cystic hygroma should be included in the differential diagnosis of shoulder pain in children.

Angiographic response of locally advanced soft-tissue sarcoma following hyperthermic isolated limb perfusion with tumor necrosis factor.

BACKGROUND: Hyperthermic isolated limb perfusion (HILP) with tumor necrosis factor-alpha (TNF-alpha), interferon-gamma (IFN-gamma), and melphalan is associated with a dramatic anti-tumor effect in which the neo-vascularization of the tumor is supposed to be the major target. The aim of the present study was to correlate the angiographic findings with the pathological response in patients undergoing HILP for locally advanced soft-tissue sarcoma. PATIENTS AND METHODS: Twenty-five patients, 14 male and 11 female, mean age 47 years (range 18-80) were studied. Angiographies were performed before and a median period of 7 weeks (range 4-14 weeks) after HILP. Eight weeks after perfusion, the residual tumor mass was resected and pathologically examined. The changes in tumor vascularization after treatment were scored and compared with the pathological response. RESULTS: All baseline angiograms showed a hypervascular tumor. After HILP, a normal angiography result (NA) was observed in 18 patients (72%) and an abnormal angiography result (AA) was observed in seven patients (28%). All patients with an NA showed a pathologically complete response (pCR) or a pathological partial response with > 90% necrosis of the tumor. Of seven patients with an AA, pathological examination showed a pCR in one patient, 10-50% viable tumor volume in four patients, and no pathological response after perfusion in two patients. A good correlation was seen between angiographic and pathological classification (p < 0.001). CONCLUSION: An angiography performed after hyperthermic isolated limb perfusion with TNF-alpha and melphalan provides a good indication, regardless of whether a good pathological response is expected.

Pelvic girdle chondrosarcoma and inferior vena cava thrombosis.

Pelvic girdle chondrosarcomas in children and adolescents are extremely rare. These high grade malignant chondrosarcomas with a locally aggressive growth pattern have a high tendency for thrombus formations in the afferent tumour vessels, associated with an increased risk of inferior vena cava thrombosis, pulmonary embolism and metastatic spread to the lungs. This tumour location is generally non-resectable for cure and, since adjuvant chemotherapy is ineffective, patients die from the tumour thrombus formation causing tumour emboli and metastatic disease to the lungs. Effective local tumour control can be achieved with high-dose hyperfractionated radiation therapy.

Randomised controlled trial of magnetic-resonance pelvimetry in breech presentation at term.

BACKGROUND: Pelvimetry is widely used in women with breech presentation at term to select those for whom planned vaginal delivery is appropriate. However, its clinical value has never been established. We evaluated pelvimetry in a randomised controlled trial. The main outcome measures were the elective and emergency caesarean-section rates and the early condition of the neonate. METHODS: Magnetic-resonance (MR) pelvimetry was done on 235 women. The women were then randomly assigned to two groups--for the study group (n = 118), the pelvimetry results were reported to the responsible obstetricians, who used them as the basis for decisions on whether to schedule elective caesarean or trial of labour; for the control group (n = 117), the pelvimetry results were not disclosed until 8 weeks post partum, and decisions about obstetric management were made on the basis of clinical factors only. FINDINGS: 35 women (15 [13%] study group, 20 [17%] control group) had abnormalities on pelvimetry. The overall caesarean-section rates did not differ significantly between the study and control groups (50 [42%] vs 59 [50%], p = 0.24) but the emergency caesarean-section rate was significantly lower in the study group than in the control group (22 [19%] vs 41 [35%], p = 0.0052). The mean 1 min Apgar scores in the study and control groups were 8.1 and 8.0 (p = 0.93) and the mean 3 min scores 9.5 and 9.4, respectively (p = 0.28). There were no significant differences in the early neonatal outcome for infants born vaginally, by emergency caesarean section, or by elective caesarean section in the two groups, except for a significantly lower Apgar score in the six infants born vaginally to control-group women who had pelvic abnormalities. INTERPRETATION: The use of MR pelvimetry in breech presentation at term did not significantly reduce the overall caesarean-section rate. However, it allowed better selection of the delivery route, with a significantly lower emergency caesarean-section rate. Neonatal outcome was not compromised by use of the pelvimetry data.

Assessment of quantitative hypertrophy scores in hypertrophic cardiomyopathy: magnetic resonance imaging versus echocardiography.

To compare the diagnostic value of spin-echo magnetic resonance (MR) imaging and transthoracic echocardiography in quantitative assessment of the extent of hypertrophy in patients with hypertrophic cardiomyopathy (HCM), we examined 52 consecutive patients with HCM. The Spirito-Maron and Wigle hypertrophy scores were calculated with wall thickness measurements obtained by both imaging modalities. MR imaging yielded complete assessment of anatomic features and allowed calculation of hypertrophy scores in 49 patients (94%). Adequate echocardiograms were obtained in 33 patients (63%) and correlated well with MR imaging for wall thickness measurements and for determination of the two hypertrophy scores (both r> 0.9). MR imaging provided additional information not available by echocardiography in 16 patients (31%). We conclude that the Spirito-Maron and Wigle hypertrophy scores correlated well between echocardiography and MR imaging. Because echocardiography was of insufficient quality for calculating adequate hypertrophy scores in 19 (37%) patients, MR imaging provided the most comprehensive diagnostic information in patients with HCM.

Fracture of the thumb sesamoid bone: a report of three cases and a review of the English-language literature.

Fracture of the sesamoid bone of the thumb is a rare injury. In this report three additional cases with fracture of an ulnar sesamoid bone of the thumb are reported. The anatomic considerations, the mechanisms of injury, the differential diagnoses, and management are discussed with the English-language literature.

Localised proton spectroscopy and spectroscopic imaging in cerebral gliomas, with comparison to positron emission tomography.

In 32 patients with gliomas, one- and two-dimensional proton magnetic resonance spectroscopy (1H-MRS) has been conducted, the latter allowing reconstruction of spectroscopic data into a spectroscopic image (MRSI), showing the distribution of the various metabolite concentrations over the cross-sectional plane. For lack of absolute concentrations, the measured concentrations of phosphocholine (CHOL), N-acetyl-L-aspartate (NAA), and lactate (LAC) were conventionally expressed in ratios relative to that of creatine (CREAT). Compared to normal brain tissue, an increased CHOL/CREAT ratio was found in all groups of tumours, in glioblastomas, high-, middle- and low-grade astrocytomas both at the margin and the core of the tumours, but in oligodendrogliomas only at the margin. This is consistent with an increased phosphocholine turnover in relation to membrane biosynthesis by the proliferating cells. The NAA/CREAT ratio was decreased in all groups of tumours, both in the centre and at the margin, reflecting replacement of functioning neurons by neoplastic cells. The LAC/CREAT ratio was elevated in the core of malignant gliomas, which may be the result of a prevailing glycolysis, characteristic of tumours, possibly in conjunction with hypoxia/ischaemia. In the perifocal oedema, there was neither elevation of the CHOL/CREAT ratio nor decrease of the NAA/CREAT ratio; an increased LAC/CREAT ratio therefore rather reflected ischaemia/hypoxia probably due to locally elevated pressure and compromised regional perfusion. In the normal brain, the metabolite ratios of grey matter did not differ from those of white matter. The frontal lobe and basal ganglia showed lower NAA/CREAT ratios than the other cerebral areas.(ABSTRACT TRUNCATED AT 250 WORDS)

Ex vivo MRI in extracorporeal liver surgery.

Extracorporeal resection of hepatic tumors that were considered inoperable in the past is now possible in selected cases. Such procedures require high-quality preoperative images for the exact delineation of the tumor extent and for an optimal planning of the line of parenchymal division. In-vivo CT and MRI can not always depict the tumor adequately. In such cases, ex-vivo MRI may be a useful additional technique. It combines a high spatial resolution with the best possible soft tissue contrast, as was learned from previous studies on donor livers destined for transplantation. Ex-vivo MRI favours both a sufficiently radical resection as well as sufficient hepatic functional reserve to be present for reimplantation. A case history is reported together with details on the technical procedure.

On the principles underlying the diagnosis of brain tumours--a survey article.

A survey is given of the principles underlying the diagnosis of brain tumours. Traditionally diagnosis and localization of brain tumours have been based upon morphological criteria. Currently unsurpassed levels in imaging of anatomical details and topographical relations by the techniques of computed tomography (CT) and magnetic resonance imaging (MRI) have been achieved. The techniques of positron emission tomography (PET) and of magnetic resonance spectroscopy (MRS), which depict also metabolic and blood flow aspects, provide a refinement of our knowledge on the metabolism, structure and pathophysiological relations of a tumour to the surrounding parenchyma. Recent advances in the recording of function-related changes of the cerebral electro-magnetic field allow a better definition of critical functional areas.

Clinical applicability of human in vivo localized phosphorus-31 magnetic resonance spectroscopy of bone and soft tissue tumors.

BACKGROUND: Magnetic resonance imaging (MRI) is of restricted value for the in vivo characterization of tumor types. The applicability of phosphorus-31 (31P) magnetic resonance spectroscopy (MRS) in the diagnosis of bone and soft tissue tumors is unknown. METHODS: A total of 191 consecutive patients (85 females and 106 males; mean age 41 years, range 1-80) with a well-defined bone or soft tissue tumor on MRI were analyzed for additional 31P spectroscopy. Histology and/or cytology was obtained from all tumors. Because of low sensitivity of the 31P nucleus and the contamination of surrounding tissue, only large, superficially located tumors accessible to the surface coil could be accepted for MRS. RESULTS: Twenty-one patients (11%) could be included in the study. From this remaining group only 12 studies (57%) produced spectra with well resolved phosphorus peaks and an acceptable signal-to-noise ratio. However, these spectra did not allow differentiation between the benign and malignant nature of the lesions. The other 9 studies showed spectra with poor signal intensities and/or poorly defined peaks, making tumor differentiation impossible. CONCLUSION: Only 6% of the bone and soft tissue tumors produced well defined spectra, which implies that localized 31P MRS cannot be considered as a routine technique in the diagnostic and treatment evaluation of bone and soft tissue tumors.