Incomplete Currarino syndrome with a presacral leiomyosarcoma.
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Biomedical subjects
Publications and source records attributed to L Bostad.
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A case of isolated vasculitis in the mammary gland of a 68-year-old female is described. Over a few months tender lumps occurred in both breasts. There were no signs of generalized disease. The sedimentation rate was normal and mammographic examination revealed no signs of malignant disease. The tumours were removed and histological examination showed inflammation, thrombosis and necrosis of medium and small arteries. We discuss the possibility of an isolated polyarteritis nodosa localized to the breast.
In a follow-up study of 193 adult patients with renal cell carcinoma diagnosed in northern Norway 1974-1980, ESR as a prognostic factor was studied with the Cox regression model. In 71 patients (37%) metastatic disease was known at diagnosis. In patients without metastatic disease an elevated ESR greater than 15 mm/h and renal vein involvement were significant prognostic factors indicating short survival. Multivariate survival analyses of all patients showed the presence of metastatic disease and elevated ESR (greater than 15 and greater than 30 mm/h) as significant prognostic factors indicating high-risk patients. This study concludes that ESR deserves attention as a prognostic discriminator in renal cell carcinoma.
Eight hundred sixty-nine primary malignant or borderline ovarian tumors reported to the Norwegian Cancer Registry were reviewed. The histologic slides were randomly distributed to six observers and classified according to the World Health Organization classification of ovarian tumors. By rotation of slides, each tumor was successively reviewed by three observers. Each observer was given approximately 40 duplicates of slides he or she had typed before, mixed in with the slides for the third review. A contracted version of the classification with 27 entries was used in the analysis. Mean intraobserver reproducibility was 62% (kappa, 0.53), varying from 50% to 75% (kappa, 0.34 to 0.70) for the individual observers. The mean rate of agreement between two observers was 56% (kappa, 0.46), varying from 46% to 65% for the individual pairs of observers. The rate of full agreement among three observers was 41%. The most common disagreements were between different specific types of carcinoma, between undifferentiated and differentiated carcinoma, between borderline and malignant tumors, between unclassified and classified carcinoma, and between mixed and pure types of carcinoma. Very low reproducibility was obtained for mixed and unclassified carcinoma.
Lesions of the gastrointestinal tract with massive tissue eosinophilia may present a difficult diagnostic problem. In a series of 250 gastrointestinal lymphomas drawn from the files of St Bartholomew's and St Mark's Hospitals there were 28 cases of a lymphoma with distinctive histological features, characterized by a massive tissue eosinophilia. Two of these tumours were present in the stomach and 26 in the small intestine. Eight of the latter were associated with coeliac disease. On low power examination a zoning phenomenon was regularly seen and fissuring ulceration, with perforation and fistula formation, was a common finding. The tumour cells were large and pleomorphic with irregular nuclear morphology and prominent nucleoli. Eosinophils were the predominant inflammatory cell associated with the lymphoma but plasma cells, epithelioid histiocytes and small lymphocytes were also present. Vascular changes were prominent. Involved lymph nodes showed gross expansion of the paracortex by tumour. Immunohistochemical studies showed that this lymphoma was probably of T-cell origin.
40 patients with their first gastric ulcer diagnosed gastroscopically 4-8 years ago and who have not since undergone surgery for their ulcer disease or been on medical treatment for active ulcer were reexamined with endoscopy. In 22 patients one or more gastric ulcers were found. At the follow-up endoscopy, 14 of these patients were without dyspeptic complaints, 4 had minimal symptoms and did not need antacids, and 4 were taking antacids for the relief of dyspepsia. A high frequency of asymptomatic gastric ulcer in patients with previous symptomatic disease has not been reported before, and the natural course of gastric ulcer disease should be re-evaluated by long-term prospective studies.
During the period 1976-83, anti-mitochondrial antibodies (AMA) were detected in 68 patients out of about 48 000 sera (0.14%) analyzed for a repertoire of autoantibodies at the Department of Immunology, University Hospital of Tromsø. Fifty-five of these patients were women, and only 10 had unequivocal primary biliary cirrhosis (PBC). At follow-up in 1984, 48 out of these 68 patients were accessible for complementary testing. The AMA test became negative in 17 of these 48 patients during the observation period. Eleven of these 17 had originally a titer of 50. Seven of the 31 patients with persistent AMA were without detectable liver pathology. One patient had antibodies against smooth muscle, one against cell nucleus, whereas 35 had an increased serum IgM level. In conclusion, most patients with AMA do not have obvious PBC, a low AMA titer is likely to be transient, and there is a strong association between AMA and an increased serum IgM level.
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A 54-year-old female with a 3 year history of coeliac disease suddenly deteriorated. Clinical investigation and bone marrow aspirate were consistent with malignant histiocytosis. The patient died and the following postmortem examination confirmed the diagnosis. The significance of the concomitant occurrence of coeliac disease and malignant histiocytosis is discussed.
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Tonsillar tissue from individuals in the early stages of HIV-1 infection was studied during the natural course of infection and during antiretroviral therapy with and without a protease inhibitor in order to investigate markers of clinical progression and evaluate the effects of therapy. Tonsillar biopsies and blood samples were collected at regular intervals during 3 years and clinical observations were noted. Tonsillar morphology was evaluated and the fragmentation of the follicular dendritic cell network was quantified by standardised follicular fragmentation rate (FR) analysis. Lymphocyte subsets were phenotyped by flow cytometry, and viral load was calculated by limiting dilution assay. The FRs were higher in the HIV-1-infected individuals than in the uninfected controls, although tonsillar tissue from both groups contained follicular fragmentation. During HIV-1 infection, the FR increased and the tonsillar CD4/CD8 ratio declined. During maximum viral suppression, FR approached that of controls while tonsillar T cell subsets and blood CD4 cell counts normalised. Even when virus suppression was incomplete, tonsillar improvements were observed in parallel with a resolution of the HIV-1-related dermatological disorders. However, persistent viral replication paralleled distortion of the tonsillar architecture. We suggest that a normalisation of the lymphoid tissue may have important functional and clinical implications in HIV-1 infection.
The youngest reported case of laryngeal granular cell tumor is presented together with transmission electron micrographs of the tumor cells. Whereas subglottic involvement is unusual in adults, a review of the literature reveals that three of the seven youngest patients (including the present patient) with laryngeal granular cell lesions had a subglottic tumor immediately below the anterior commissure.