Aims-To examine the expression of HLA-DR and 2 microglobulin in medullary carcinoma and atypical medullary carcinoma of the breast to determine if the eVective presentation of tumour antigens to the immune system can diVerentiate between these two histopathologically similar entities. Methods-Expression of HLA-DR and 2 microglobulin was examined by immunohistochemical methods in five samples of medullary carcinoma of the breast, which has a relatively favourable prognosis, six samples of atypical medullary carcinoma of the breast, which has a prognosis closer to that of regular invasive duct carcinoma, and 20 samples of invasive duct carcinomas, 10 with an accompanying lymphocytic infiltrate. Results-A positive and significant correlation was found between tumour type and both HLA-DR and 2 microglobulin expression. Expression was most prominent in medullary carcinoma, followed by atypical medullary carcinoma and invasive duct carcinoma with and without lymphocytic infiltrates. The mean intensity and percentage of HLA-DR tumour immunostaining were significantly higher in medullary carcinoma than in the other three tumour groups, as was the mean intensity of 2 microglobulin immunostaining. Mean percentage of 2 microglobulin immunostaining was significantly higher in medullary carcinoma than in invasive duct carcinoma without lymphocytic infiltrates, and showed a trend to increase from invasive duct carcinoma with lymphocytic infiltrates to atypical medullary carcinoma and medullary carcinoma. Conclusions-Medullary
A 3.5-year-old boy with a multifocal primary lymphoma of the brain was treated successfully without neurotoxicity with a treatment regimen fhat did not include radiation. The protocol of Dexacort (dexamethasone), methotrexate, Oncovin (vincristine), and BCNU (carmustine) (DEMOB), which was developed with the use of MTX pharmocokinetic studies, was given over 7.5 months, and resulted in tumor disappearance on computerized tomography scans and marked improvement in clinical status. The patient remains in good health 3 years after diagnosis (March 1985).Cancer 57:6-11,1986.HE REALIZATION in the Iast few years that microgly-T omas are primary brain lymphomas' raised the hope that, with appropriate antilymphoma therapy, cure could be achieved. We report the first case that, to the best of o u r knowledge, has been treated successfully without radiotherapy. It has been 3 years since diagnosis and 30 months since therapy was completed. Case ReportA 3.5-year-old boy was referred for postoperative treatment of a primary brain tumor in January 1982 from the Neurosurgical Department of this hospital. He had been healthy until August 198 1, when he had an episode of vomiting and a 2-hour loss of consciousness. One week later he had a similar attack. Computerized tomography (CT) (Figs. 1A and IB) showed two foci; one in the left frontoparietal area and the other in the left temporo-occipital area. The frontoparietal focus was not visualized on a further CT 6 weeks later, but the temporo-occipital lesion had grown in size (Fig. 2).He experienced weakness of his left leg and arm and, after a further episode of vomiting and headache, underwent craniotomy with gross resection of the left temporal lesion (December 2 1, I98 I). The tumor was removed piecemeal. The internal border was less clearly demarcated than the superficial border, but no obvious tumor remained after the operation.
Clinically significant liver involvement in systemic amyloidosis, especially with cholestasis, is rare. We report a case of primary amyloidosis with severe intrahepatic cholestasis leading to terminal liver failure. The present case is the first of its kind reported involving Lambda light chains only as the associated paraprotein. Conventional treatment and a therapeutic trial with dimethyl sulphoxide were unsuccessful. The liver is known to be a common site of amyloid deposition, both in primary and secondary amyloidosis.' Hepatocellular failure and portal hypertension are rare, however, with cholestasis and jaundice found in only about 5% of cases.2 In primary amyloidosis with massive liver involvement the paraprotein consists characteristically of Kappa light chains.3 We report a case of primary amyloidosis of Lambda light chains with dominant liver involvement leading to cholestasis and terminal hepatic failure. CASE REPORT A 69 year old dentist was sent for evaluation of hepatomegaly found on a routine medical examination. He was asymptomatic and had been well throughout his life. On examination, a firm, nontender liver was palpated 8 cm beneath the right costal margin. The span was 19 cm on percussion. The tip of the spleen was palpable. Enlarged left axillary and bilateral inguinal lymph nodes were also palpated. The physical examination was otherwise unremarkable. A complete blood count (CBC), prothrombin time (PT), serum electrolytes, calcium, blood urea nitrogen (BUN), creatinine, albumin, globulin, alkaline phosphatase (ALP) and bilirubin were within normal limits.
Histologic and immunohistochemical properties of 53 medulloblastomas were analyzed with regard to clinical features and survival rate. No correlation was found between survival rate and histologic features of the tumor, such as desmoplastic reaction, number of mitoses, hemorrhages, necrosis, endothelial proliferation, glomerular arrangement, calcifications, rosettes, or oligodendroglial cells. However 82% of the patients with positive glial fibrillary acidic protein (GFAP) staining in numerous tumor cells survived more than 5 years; only 30% survived that long if their tumor cells were GFAP negative (P = 0.0093). This significant difference was not related to the mode of therapeutic protocol used. The authors suggest that GFAP staining may be a useful prognostic tool in medulloblastoma.
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