Pigmented ganglioneuroblastoma: a tumour cell 'storage disease'?
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Biomedical subjects
Publications and source records attributed to E F Gaffney.
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Currently, pleomorphic rhabdomyosarcoma (RMS) in adults is considered to be extremely rare or nonexistent. The authors have identified 11 cases of pleomorphic RMS using the following criteria: pleomorphic sarcoma occurring within voluntary muscle, large polygonal or strap-like cells with copious eosinophilic cytoplasm, desmin and myoglobin immunoreactivity, or ultrastructural evidence of sarcomeric differentiation. Ten patients were male, the median age at presentation was 56 years (range, 27-84), and the thigh (seven cases) was the most common site. Of eight cases with clinical follow-up, one patient is alive at 20 months, and seven died 2 to 28 months following diagnosis. The tumors were generally patternless, but several had storiform areas. Cross-striations were not identified. Immunostaining for muscle-related antigens was positive as follows: desmin (in 10 of 11 cases), myoglobin (in 10 of 11 cases), actin HHF-35 (in all 11 cases), smooth-muscle actin (in six of eight cases), sarcomeric actin (in six of nine cases), and fast myosin (in five of five cases). Staining for S-100 protein was negative in all cases. On electron microscopy (six cases), two tumors had well-differentiated rhabdomyoblasts with sarcomeres, Z-disks, and hexagonal arrays of myofilaments; three were poorly differentiated; and one contained immature mesenchymal cells only. Pleomorphic RMS can be distinguished from other pleomorphic sarcomas provided that well-fixed tumor tissue is available for immunohistochemical staining and electron microscopy. We consider that this distinction is important in view of the poor prognosis associated with pleomorphic RMS in this series.
Solid undifferentiated carcinoma was the major microscopic pattern in 24 prostatic carcinomas, 12 of which were clinically recurrent. Tumour cells were uniform, with moderately hyperchromatic nuclei and indistinct cytoplasm, and were arranged in solid or focally irregular aggregates. In areas, the tumour cells were large with vesicular nuclei, nucleoli and more abundant cytoplasm. In previous specimens, solid undifferentiated carcinoma was absent or was a minor pattern. Twenty of 23 cases showed prostate specific antigen and prostatic acid phosphatase immunoreactivity, and nine of 17 cases contained scattered argyrophilic or chromogranin-immunoreactive cells. On proliferating cell nuclear antigen immunostaining of 12 specimens, the mean tumour proliferative fraction in solid undifferentiated carcinoma (range: 10.5-18%) was greater than in areas of grade 3 prostatic carcinoma (range: 3-6%). In all 22 stage C and D cases, there was a close correlation with clinical evidence of tumour progression, and the overall 2-year survival rate was only 16.7%. It is concluded that a major solid undifferentiated pattern correlates with increased biological aggressiveness and a poor prognosis in locally advanced prostatic carcinoma.
Post-ischemic renal failure is associated with a zone of vascular hyperaemia in the outer medulla of the kidney. The effect of this lesion on regional renal perfusion is, however, unclear. Acute unilateral renal ischemia was applied to four groups of ten adult male Wistar rats for a period of 60 min, followed by revascularisation for 0, 15, 30 or 60 min. The aorta was then clamped and Microfil was injected at a standard pressure to fill the renal vasculature. Gross and histological examinations of the renal parenchyma and vasculature were then performed. Regional renal Microfil perfusion was quantified by examination of unstained histological sections, giving rise to a vascular perfusion index (VPI) for each vascular region of the kidney. The VPIs were similar in control and ischemic kidneys that were not subjected to reflow (group 1). In contrast, the VPI was markedly decreased in the inner stripe and inner medulla in animals in which revascularisation had occurred (groups 2-4), and the vasculature in these regions was histologically shown to be packed with red blood cells. Post-ischemic renal failure is associated with hyperperfusion of the medulla resulting from blockage of the vasculature that occurs during revascularisation.
Sarcomas of the soft tissues and viscera are a diverse group of uncommon neoplasms that often present difficult diagnostic and management problems. The Dublin Soft Tissue Tumour Panel prospectively reviewed pathology material from 137 patients of four Dublin teaching hospitals between January 1989 and August 1990. The prevalence of sarcomas in Irish patients was found to be similar to that estimated for the United States. The most common sarcoma of soft tissues was malignant fibrous histiocytoma (14) and the most common visceral sarcoma was leiomyosarcoma (10). The most problematic diagnoses were 3 peripheral neuroepitheliomas (extraskeletal Ewing's sarcomas) which occurred in patients aged 19-27 years, distinctly younger than the typical adult sarcoma patient. Clinical follow-up is in progress and will be combined with the findings of a linked retrospective study, to facilitate evaluation of all aspects of management.
Piezoelectric second generation lithotriptors are an established means of administering extracorporeal shockwave lithotripsy (ESWL) enabling treatment to be performed without anaesthesia or analgesia, but higher shockwave doses and multiple or staged treatment are frequently required. The bioeffects of this modality of ESWL, therefore, require further assessment. Seven experimental groups of adult male rabbits were treated using the EDAP LT.01 in order to determine the acute and chronic bioeffects of clinical dose, excess dose, divided excess dose, high frequency and multiple treatment (X10) piezoelectric shockwaves (PSW). Renal function was measured before and after treatment using mercaptoacetyltriglycine (MAG 3) scans. Gross and histological morphological changes were assessed at one and 30 days following application of PSW. Application of single clinical dose PSW was not associated with any significant functional or morphological renal injury. Excess dose PSW caused transient gross renal contusion, which resolved in the majority of animals with no persistent microscopic abnormality. Divided excess dose PSW resulted in no gross or microscopic damage. High frequency PSW was associated with mild histological abnormality. Multiple PSW treatments caused small discrete fibrotic lesions in all cases, without any change in renal function.
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Chronic glomerulonephritis (GN) was induced in N/M mice by daily injections of human serum albumin (HSA). The glomerular lesion was similar to that observed in human membranous GN and was characterized by intense mesangial and capillary loop immunofluorescent staining for HSA, IgG and C3. Electron microscopic examination revealed numerous electron-dense deposits in the mesangium and along the subepithelial side of the glomerular basement membrane, the latter deposits being associated with membranous spikes. Chronically injected mice that had been treated with cyclosporin (CsA) from Day 1 had different patterns of immune complex deposition. Mesangial deposition was apparently unaltered but no subepithelial deposits or spikes were evident. In addition, only two out of 21 HSA-injected mice which began CsA treatment on Day 21 had subepithelial deposits. There was no significant difference in serum levels of HSA-specific IgG between the three groups of mice. CsA treatment would therefore appear to ameliorate the immunopathology of antigen-induced glomerulonephritis in this model without affecting serum antibody levels, and may be of therapeutic value in the treatment of human membranous GN.
A 63 year old man with pulmonary tuberculosis developed nephrotic syndrome secondary to immune complex nephritis. The nephrotic syndrome and renal lesion resolved with standard chemotherapy.
DNA from members of a three-generation pedigree of Irish origin, displaying an autosomal dominant simplex form of epidermolysis bullosa of the epidermolytic, simplex, or Koebner variety (EBS2), was analyzed for linkage with a set of markers derived from the long arm of chromosome 1. Two-point analysis revealed positive lod scores for five of these markers, AT3 (Z = 2.107, theta = 0), APOA2 (Z = 1.939, theta = 0.15), D1S66 (Z = 1.204, theta = 0), D1S13 (Z = 1.026, theta = 0.15), and D1S65 (Z = 0.329, theta = 0.15). Multilocus analysis, incorporating the markers D1S19, D1S16, D1S13, APOA2, D1S66, AT3, and D1S65, resulted in a lod score of 3 maximizing at AT3. These data strongly support previous tentative indications of linkage between EBS2 and genetic markers on the long arm of chromosome 1.
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A malignant sex cord-stromal tumour that occurred in a 56-year-old patient with the androgen insensitivity syndrome is reported. Although hamartomas composed of sex cord cells are common in the testes of patients with this syndrome, unequivocal neoplasms of sex cord type are rare. The tumour described herein most closely resembled, but lacked the overall morphology of, a juvenile granulosa cell tumour, and Charcot-Böttcher filaments, indicative of Sertoli cell differentiation, were seen on electron microscopy. The features of the androgen insensitivity syndrome and the various tumours that have been reported in patients with this syndrome are briefly reviewed.
Endocrine-paracrine (EP) cells are normal cellular constituents of the human genitourinary tract. We examined the distribution and immunostaining profile of EP cells in von Brunn's nests and glandular metaplasia in the supramontanal (proximal) prostatic urethra of adult males and in prostatic ducts of the male fetal urethra. In 15/17 transurethral prostatic resection specimens there were argyrophilic cells and/or serotonin and neuronspecific enolase containing cells in von Brunn's nests/glandular metaplasia and in the supramontanal prostatic urethra. All seven fetal specimens examined contained EP cells and five had focal calcitonin immunoreactivity in prostatic ducts and ductal buds. One adult resection specimen had focal staining for human chorionic gonadotrophin and no case showed staining for somatostatin. These results further support the contention that von Brunn's nests and glandular metaplasia are probably normal urothelial variants in the adult supramontanal prostatic urethra.
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Significant subfertility exists in patients following unilateral testicular torsion, implying bilateral testicular disease. Immunological activation has been detected after experimental torsion and the present study sought to demonstrate immunologically mediated effects on contralateral spermatogenesis following experimental torsion, as well as quantifying ipsilateral damage. Early and late effects of torsion on bilateral spermatogenesis were studied at 1 and 6 months in 10 groups each containing 20 rats. Gross and histological examination, direct immunofluorescence tests, vas deferens counts and copulation studies were performed. Severe ipsilateral damage was noted, even after brief torsion periods. No contralateral testicular effects, immunological or otherwise, were observed. Ipsilateral damage after torsion may have been underestimated. There is no damage to contralateral testicular exocrine function following unilateral experimental torsion.