Need for follow up in coeliac disease

The use of follow up studies was evaluated in 128 patients with coeliac disease during their first visit to a department for adults. The original diagnosis had been made in childhood in all patients. Fifty eight (45%) of the subjects were following a gluten free diet, 23 (18%) were following a gluten free diet but with occasional gluten consumption, and 47 (37%) had adopted an unrestricted, gluten containing diet for a mean of 11.2 years. There was no correlation in individual subjects between the presence of symptoms, biochemical and immunological abnormalities, severity of histological findings, and the amount of dietary gluten, despite the greater frequency of symptoms in the group following an unrestricted diet than in the other two groups. Short stature and epilepsy with cerebral calcifications only occurred in patients following an unrestricted diet. As only diagnosis based on two or three biopsy samples and regular follow up correlated positively with dietary compliance, it is suggested that a histologically confirmed diagnosis of coeliac disease and regular lifelong follow up are essential in the management of these patients.     

Radiotherapy in the management of giant cell tumor of bone

PURPOSE: To evaluate the outcomes of patients with giant cell tumor of bone (GCTB) treated with radiotherapy (RT) with or without surgical resection. METHODS AND MATERIALS: We performed a retrospective review of the records from 25 consecutive patients with pathologically confirmed GCTB who had undergone RT between 1956 and 2000. RESULTS: Patients ranged in age from 11 to 69 years (median 32); 16 were female and 9 were male. The anatomic distribution of lesions was as follows: cervical spine, 3; temporal bone, 1; thoracic or lumbar spine, 9; sacrum, 8; ilium, 1, and humerus, radius, and thumb metacarpal, 1 each. Tumors ranged in size from 2 to 20 cm (median 9.5) at their maximal dimension. Thirteen patients had been referred for RT for primary GCTB and 12 had been referred with locally recurrent disease after having undergone one or more other treatments. Fourteen patients had undergone RT for gross disease, and the remaining 11 had been treated with RT after gross total resection. In 10 of these 11 patients, the treatment margins were positive or uncertain. Radiation doses ranged from 25 to 65 Gy (median 46). At a median follow-up of 8.8 years (range 0.67-34), 7 patients had developed isolated local recurrence, 2 had developed isolated distant recurrence, and 3 had developed both. The actuarial 5-year overall and disease-free survival rate was 91% and 58%, respectively, and the actuarial 5-year local control and distant metastasis-free survival rate was 62% and 81%, respectively. Univariate analysis suggested that treatment for recurrent disease correlated with a lower disease-free survival rate (83% vs. 33%, p = 0.06), distant metastasis-free survival rate (100% vs. 64%, p = 0.08), and local control rate (83% vs. 42%, p = 0.08) at 5 years. Of the 12 cases of recurrence, 7 were ultimately successfully treated with additional salvage therapy. In 4 of these patients, salvage therapy included interferon-alpha 2b. CONCLUSION: RT should be considered an adjuvant to surgery or as alternative therapy in cases of GCTB that are unresectable or in which excision would result in substantial functional deficits. When RT is used as primary therapy, the rate of local control seems to be satisfactory. In heavily pretreated patients, however, RT delivered as it was in this series can result in poor local control, and alternative therapies should be considered.     

Surgical margins and reresection in the management of patients with soft tissue sarcoma using conservative surgery and radiation therapy

BACKGROUND: Patients with localized soft tissue sarcoma (STS) who present to specialist centers after undergoing apparent macroscopic total resection often have a significant incidence of residual tumor and may benefit from reresection of the tumor bed. The potential benefits of such reresection have not been documented adequately. METHODS: The clinicopathologic features and disease outcome for 666 consecutive patients with localized STS who presented after undergoing apparent macroscopic total tumor resection were analyzed to elucidate the relative merits of reresection. Actuarial univariate and multivariate methods were used to compare disease outcome of patients who presented with positive or uncertain microscopic resection margins according to whether they underwent reresection. All patients received adjuvant radiation therapy. RESULTS: Two hundred and ninety-five patients underwent reresection of their tumor bed, and residual tumor was found in 136 patients (46%), including macroscopic tumor in 73 patients (28%). Final resection margins among patients who underwent reresection were negative in 257 patients (87%), positive in 35 patients (12%), and uncertain in 3 patients (1%). Patients who did not undergo reresection had final margins that were negative in 117 patients (32%), positive in 47 patients (13%), and uncertain in 207 patients (56%). Local control rates at 5 years, 10 years, and 15 years for patients who underwent reresection were 85%, 85%, and 82%, respectively; for patients who did not undergo reresection, the respective local control rates were 78%, 73%, and 73% (P = 0.03). Reresection remained a significant determinant of local control when other prognostic factors were incorporated into a multivariate proportional hazards regression analysis. A similar beneficial effect of reresection was found for metastasis free survival and disease specific survival. CONCLUSIONS: Patients with localized STS who were referred to a specialist center after undergoing apparent macroscopic total resection of their tumor had a high incidence of residual tumor in their tumor bed and benefited from undergoing reresection, even if radiation was administered routinely.     

Acute experimental cerebral ischemia: MR enhancement using Gd-DTPA

The effects of a paramagnetic contrast agent, gadolinium-DTPA (Gd-DTPA), on magnetic resonance (MR) imaging of acute cerebral ischemia was investigated in a feline model of middle cerebral artery occlusion. Imaging was performed both before and after administration of an intravenous dose of 0.2 mmol/kg of Gd-DTPA. The animals were then sacrificed for pathologic correlation. No changes in intensity or relaxation times were noted before or after Gd-DTPA administration in two animals with 2 hours of occlusion. Infarcts were noted before and after contrast enhancement in all six cats with ischemia of greater than 16-hours duration. Gd-DTPA caused significant increase in intensity of infarct but not in that of normal cerebral tissue. Rapid enhancement was visible in infarcts of 16-24 hours, but such enhancement was slower in infarcts of 72-168 hours, presumably owing to slowed inflow caused by increased vasogenic edema in the latter group. Contrast enhancement of acute cerebral ischemic lesions with Gd-DTPA offers no improvement in sensitivity of MR imaging, although the conspicuity of the lesion may be improved. Additionally, contrast media may provide potential temporal and pathophysiological data for better characterization of cerebral ischemia.