Leiomyosarcoma of somatic soft tissues: a tumor of vascular origin with multivariate analysis of outcome in 42 cases.

Leiomyosarcomas of the somatic soft tissues (SST) are rare compared with their retroperitoneal and cutaneous counterparts and, therefore, have not been extensively studied. We have analyzed the clinicopathologic features of 42 SST leiomyosarcomas referred in consultation to determine what factors affect outcome. Cutaneous, visceral, retroperitoneal, uterine, gastrointestinal, and major vessel leiomyosarcomas were excluded. By definition all lesions possessed at least focal cytologic atypia and mitotic activity, although the latter varied from <1/10 high power fields to 66/10 high power fields. The patients (21 females and 21 males) ranged in age from 26 to 86 years (mean 60 years); tumors developed in the lower (n = 28) or upper extremity (n = 11) and trunk (n = 3). Most arose in deep (n = 27) as opposed to superficial (n = 15) soft tissue; 39 arose from a small vein. During the follow-up period (mean 47 months, range 9-162 months), 3 of 38 (8%) patients developed local recurrence and 17 of 38 metastasized (45%) mostly to the lungs. In a univariate analysis age >62 years, size >4 cm, extensive necrosis, modified updated French Federation of Cancer Centers (FFCC) grade, and whether the tumor had been "disrupted" by a previous incisional biopsy or incomplete excision were significantly correlated with metastasis. AJCC stage also approached significance (p = 0.096) but could not be reliably tested because of the sparseness of the data. In multivariate analyses the logistic regression model that best predicted metastasis at 36 months incorporated the effects of age, FFCC grade, and disruption and had a sensitivity of 94.1% and a specificity of 95.2%. Disruption was the only significant risk factor for metastasis in a multivariate analysis (relative risk 2.70; p = 0.0001) but was strongly correlated with large size and deep location. Other parameters did not improve the predictive power of the model significantly. We concluded that the majority of SST leiomyosarcomas are actually of vascular origin, an observation that has clinical and possibly biologic ramifications. Our histologic definition of leiomyosarcoma to include atypia and any level of mitotic activity appears warranted by the biologic outcome in our cases. The risk of metastasis can be calculated from a model incorporating age, FFCC grade, and disruption. Because disruption correlates with size and depth, it could represent a surrogate as opposed to causal marker for metastasis. Nevertheless, in view of their vascular origin, the possibility that tumor disruption may facilitate or promote access to the bloodstream merits further study.     

An epidemiological study of pulmonary function tests in geriatric population of central India

We have studied Pulmonary Function Tests (PFTs) namely Vital Capacity (VC). Forced Vital Capacity (FVC), and Forced Expiratory Volume in First Second (FEV1). Forced Expiratory Flow(FEF 25-50%) in 1200 elderly subjects above 60 years of age of which 570 were females and 630 males. Mean age was 69.22 +/- 5.57 years in males and 68.77 +/- 5.44 in females. The mean value of ventilatory parameters were as follows-1) VC 2.99 +/- 0.5 lt in males and 1.89 +/- 0.29 lt in females. 2) FVC 2.69 +/- 0.58 lt. in males and 1.76 +/- 0.21 lt in females. 3) FEV1/FVC% 83.82 +/- 10.62% in males and 83.37 +/- 11.93% in females. 4) FEF 25-75% was 2.81 +/- 1.20 lt/sec in males and 2.13 +/- 1.27 lt/sec. Physical as well as ventilatory parameters were less in females than for males. The correlation of age with VC and FEV1 was highly significant (P < 0.01) but with FVC was not significant (P > 0.05). The correlation of height, weight and body surface area was not significant with any ventilatory parameter (P > 0.05). Multiple regression equations for VC, FVC and FEV1 were formulated for males and females taking height and age as variables. The predicted values correlated excellently with observed values.     

MRI demonstration of unsuspected intraventricular rupture of pyogenic cerebral abscesses in patients being treated for meningitis

We report three patients with pyogenic meningitis who showed a partial response to medical treatment. MRI demonstrated an unsuspected brain abscess with intraventricular rupture, a possible cause of the partial response to treatment.     

Demonstration of scolex in calcified cysticercus lesion using gradient echo with or without corrected phase imaging and its clinical implications

AIM: This study was performed to determine the magnetic resonance imaging (MRI) sequence that was best suited to demonstrate the scolex in a calcified lesion and to seek the explanation for the appearance of a negative phase in a calcified scolex on corrected gradient refocused echo (GRE) phase imaging. MATERIALS AND METHODS: Forty-nine patients with single/multiple computed tomography (CT) documented homogeneous calcified lesions and/or calcified scolices in cysts were studied with conventional spin echo and corrected GRE phase imaging. Calcium and different paramagnetic substances from cysticerci scolices of a sample of infected swine muscle were quantified. RESULTS: The scolex could be demonstrated in 29/39 patients with single calcified lesion. GRE imaging with an echo time of 35ms was the only sequence that demonstrated scolex in all these 29 cases. 15/29 patients with a single calcified lesion, in all 10 patients with multiple calcified lesions and infected swine muscle with multiple cysts and calcified scolex, corrected GRE phase imaging showed negative phase in all these scolices. Estimation of minerals from the calcified scolices from the swine muscle showed by spectroscopic techniques 41.2% of the total mineral contents as paramagnetic substances. CONCLUSION: We conclude that GRE imaging is the imaging method of choice for demonstration of the scolex in a CT calcified lesion. The negative phase on corrected GRE phase imaging is due to the presence of large amount of paramagnetic substances.     

Utility of databases and information technology in pediatric neurology

Significant number of neurological patients in the pediatric age group have genetic and/or metabolic basis. It is difficult to remember details of each of them as their number is very large and the disorders are encountered infrequently. This impracticality necessitates the use of various websites and data base search. The internet has become a tool by which one can obtain and disseminate information. It has enhanced the medical person's ability to know at the earliest the developments in different medical specialities. Furthermore, these rare disorders are being recognized on the basis of specialized tests available only at selected centres which deal with few of these disorders. Our objective is to provide pediatric neurologists easy access to the expanding body of medical information and also to make them aware of the advancements in information technology, which is likely to facilitate telemedicine as a future consultancy service. Information about these diseases can also be facilitated by e-consultations.     

Valley sign in duchenne muscular dystrophy: importance in patients with inconspicuous calves

Several patients of Duchenne muscular dystrophy (DMD) do not demonstrate clinically remarkable calf hypertrophy. A new clinical sign visible behind the shoulders, which may be called 'valley sign', was tested for its utility in such cases as clinical diagnosis becomes difficult in these patients. Out of 142 DMD patients seen in the last 7 years, 12 were found to have inconspicuous calves. All the 12 patients had clinical, biochemical and/or genetic evidence of DMD. The new sign was examined by 3 independent observers in these 12 DMD patients and in 10 patients with other neuromuscular diseases. Eight DMD patients and none of the others showed positive sign. This signifies importance of this sign in the clinical diagnosis of DMD in those children in whom the calf muscle bulk is apparently normal.     

Inflammatory biomarkers,hormone replacement therapy,and incident coronary heart disease: prospective analysis from the Women's Health Initiative observational study

Context  Postmenopausal hormone replacement therapy (HRT) has been shown to elevate C-reactive protein (CRP) levels. Several inflammatory biomarkers, including CRP, are associated with increased cardiovascular risk. However, whether the effect of HRT on CRP represents a clinical hazard is unknown. Objectives  To assess the association between baseline levels of CRP and interleukin 6 (IL-6) and incident coronary heart disease (CHD) and to examine the relationship between baseline use of HRT, CRP, and IL-6 levels as they relate to subsequent vascular risk. Design, Setting, and Participants  Prospective, nested case-control study of postmenopausal women, forming part of the Women's Health Initiative, a large, nationwide, observational study. Among 75 343 women with no history of cardiovascular disease or cancer, 304 women who developed incident CHD were defined as cases and matched by age, smoking status, ethnicity, and follow-up time with 304 study participants who remained event free during a median observation period of 2.9 years. Main Outcome Measure  Incidence of first myocardial infarction or death from CHD. Results  Median baseline levels of CRP (0.33 vs 0.25 mg/dL; interquartile range [IQR], 0.14-0.71 vs 0.10-0.47; P<.001) and IL-6 (1.81 vs 1.47 pg/mL; IQR, 1.30-2.75 vs 1.05-2.15; P<.001) were significantly higher among cases compared with controls. In matched analyses, the odds ratio (OR) for incident CHD in the highest vs lowest quartile was 2.3 for CRP (95% confidence interval [CI], 1.4-3.7; P for trend = .002) and 3.3 for IL-6 (95% CI, 2.0-5.5; P for trend <.001). After additional adjustment for lipid and nonlipid risk factors, both inflammatory markers were significantly associated with a 2-fold increase in odds for CHD events. As anticipated, current use of HRT was associated with significantly elevated median CRP levels. However, there was no association between HRT and IL-6. In analyses comparing individuals with comparable baseline levels of either CRP or IL-6, those taking or not taking HRT had similar CHD ORs. In analyses stratified by HRT, we observed a positively graded relationship between plasma CRP levels and the OR for CHD among both users and nonusers of HRT across the full spectrum of baseline CRP. Conclusions  These prospective findings indicate that CRP and IL-6 independently predict vascular events among apparently healthy postmenopausal women and that HRT increases CRP. However, use or nonuse of HRT had less importance as a predictor of cardiovascular risk than did baseline levels of either CRP or IL-6.      

Wegener granulomatosis – an atypical case

 An unusual sequence of the clinical manifestations of microvascular disease is described in a 15 year-old girl. She initially presented with acute renal failure caused by a crescentic glomerulonephritis associated with positive tests for MPO-ANCA. Eighteen months later she had pulmonary hemorrhage and respiratory failure. An open lung biopsy showed granulomas that were diagnostic for Wegener granulomatosis. We discuss the diagnostic dilemmas faced in attempts to distinguish infective causes of pulmonary granulomas, such as tuberculosis or fungi, from granulomas associated with vasculitis, in a patient previously treated with immunosuppressive therapy. Received: 4 August 1999 / Revised: 28 October 1999 / Accepted: 3 January 2000     

Biphasic illness pattern due to early relapse in Japanese-B virus encephalitis

Japanese-B virus encephalitis (JE) is considered a uniphasic illness with a variable outcome. Biphasic illness patterns have never been reported previously. From an endemic zone in India we observed six patients of JE (from 62 patients treated in 7 years) who had an early relapse resulting in the biphasic clinical course. Five had poor socio-economic status and three had laboratory evidence of nutritional deficiency. Two patients were adults and the other four were children. Fever, rigors, headaches, body aches, altered consciousness, rigidity and tremors predominated the first phase of illness. During the second phase, behavioural changes, dystonia, pen-oral dyskinesia, drooling, mutism and muscle wasting due to anterior horn cell involvement were the important features. Though the serial antibody titres against the JE virus showed a four-fold rise in the initial or late convalescent phases, there was no increase during the second phase of the illness as compared to the first phase. On MRI, fresh lesions appeared during the second phase at the sites known for their involvement in JE, suggesting recrudescence of the virus. One patient survived with major sequelae, two with minor sequelae and the other three had complete recovery. We conclude that some patients with JE may have an early relapse after partial recovery, giving rise to the biphasic illness pattern. A locally prevalent genetic variant of the virus or host factors may be responsible for the altered clinical course of the disease. Biphasic illness does not necessarily mean a bad prognosis.