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  • 1
    In: Histopathology, Wiley, Vol. 80, No. 4 ( 2022-03), p. 729-735
    Kurzfassung: To investigate the histological diversity of salivary mucoepidermoid carcinoma (MEC), its clinicopathological features, and its associations with CRTC1 / 3 ‐ MAML2 fusions. Methods and results Salivary MEC cases ( n  = 177) were examined for CRTC1 / 3 ‐ MAML2 fusions, histological variants were classified, and tumours were graded according to four different grading systems. Adverse histological features considered to be unusual in MEC were also investigated. Of the 177 MEC cases, 110 were positive for CRTC1 / 3‐MAML2 fusions. The classical variant was the most frequent in the fusion‐positive case group, the fusion‐negative case group, and the total case group. The clear/oncocytic variant was the second most frequent in the fusion‐positive and total case groups. Oncocytic, Warthin‐like and spindle variants were seen in the fusion‐positive case group only. Clear cell, sclerosing, mucinous and central variants were seen in both the fusion‐positive case group and the fusion‐negative case group. No case was classified as a ciliated variant, as a mucoacinar variant, or as a high‐grade transformation. As compared with the classical variant, non‐classical variants were characterised by frequent CRTC1 / 3‐MAML2 fusions and a low clinical stage in all cases. Of the four histological features considered to be unusual in MEC, marked nuclear atypia, frequent mitoses ( 〉 10/10 high‐power fields) and extensive necrosis were found independently of the fusion status, and were present in 3–5% of all cases. However, none of the cases showed overt keratinisation. On comparison, the Armed Forces Institute of Pathology and modified Healey grading systems downgraded tumours, the Brandwein system upgraded tumours, and the Memorial Sloan Kettering system provided a moderate means of assessment. Conclusion Recognition of the histological diversity of MEC, its clinicopathological features and its associations with CRTC1 / 3 ‐ MAML2 fusions is helpful for an accurate diagnosis of this carcinoma.
    Materialart: Online-Ressource
    ISSN: 0309-0167 , 1365-2559
    URL: Issue
    RVK:
    Sprache: Englisch
    Verlag: Wiley
    Publikationsdatum: 2022
    ZDB Id: 2006447-0
    Standort Signatur Einschränkungen Verfügbarkeit
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  • 2
    In: Clinical Endocrinology, Wiley, Vol. 97, No. 1 ( 2022-07), p. 72-80
    Kurzfassung: We recently reported cases of adipsic hypernatremia caused by autoantibodies against the subfornical organ in patients with hypothalamic‐pituitary lesions. This study aimed to clarify the clinical features of newly identified patients with adipsic hypernatremia whose sera displayed immunoreactivity to the mouse subfornical organ. Design Observational cohort study of patients diagnosed with adipsic hypernatremia in Japan, United States, and Europe. Methods The study included 22 patients with adipsic hypernatremia but without overt structural changes in the hypothalamic‐pituitary region and congenital disease. Antibody response to the mouse subfornical organ was determined using immunohistochemistry. The clinical characteristics were compared between the patients with positive and negative antibody responses. Results Antibody response to the mouse subfornical organ was detected in the sera of 16 patients (72.7%, female/male ratio, 1:1, 12 pediatric and 4 adult patients). The prolactin levels at the time of diagnosis were significantly higher in patients with positive subfornical organ (SFO) immunoreactivity than in those with negative SFO immunoreactivity (58.9 ± 33.5 vs. 22.9 ± 13.9 ng/ml, p   〈  .05). Hypothalamic disorders were found in 37.5% of the patients with positive SFO immunoreactivity. Moreover, six patients were diagnosed with rapid‐onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation/neural tumor syndrome after the diagnosis of adipsic hypernatremia. Plasma renin activity levels were significantly higher in patients with serum immunoreactivity to the Na x channel. Conclusions The patients with serum immunoreactivity to the SFO had higher prolactin levels and hypothalamic disorders compared to those without the immunoreactivity. The clinical characteristics of patients with serum immunoreactivity to the subfornical organ included higher prolactin levels and hypothalamic disorders, which were frequently associated with central hypothyroidism and the presence of retroperitoneal tumors.
    Materialart: Online-Ressource
    ISSN: 0300-0664 , 1365-2265
    URL: Issue
    RVK:
    Sprache: Englisch
    Verlag: Wiley
    Publikationsdatum: 2022
    ZDB Id: 2004597-9
    Standort Signatur Einschränkungen Verfügbarkeit
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