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S100-Protein-positive Sustentakularzellen in pulmonalen Karzinoiden und thorakalen Paragangliomen

Differenzialdiagnostische und prognostische Bedeutung

S100 protein positive sustentacular cells in pulmonary carcinoids and thoracic paragangliomas

Differential diagnostic and prognostic evaluation

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Zusammenfassung

Die klassische Histomorphologie von Paragangliomen ist als so genanntes „Zellballenmuster“ beschrieben. Die nestartig angeordneten Zellverbände sind hierbei von S100-Protein-exprimierenden Sustentakularzellen (SZ) umgeben, welche mittels ihrer Zellfortsätze ein Netzwerk mit stacheldrahtähnlicher Morphologie ausbilden. In adrenalen und extraadrenalen Paragangliomen korreliert die Prävalenz von SZ invers mit der Prognose der Patienten. Um die Prävalenz sowie die bisher unklare differenzialdiagnostische und prognostische Relevanz S100-positiver Zellen in pulmonalen Karzinoiden zu analysieren, wurden 26 Tumorlets, 147 typische und atypische pulmonale Karzinoide und 10 thorakale Paragangliome immunhistochemisch untersucht. Dabei zeigte sich eine ähnliche Verteilung der Zellen in pulmonalen Karzinoiden und thorakalen Paragangliomen. Alle Patienten mit pulmonalen Karzinoiden und schlechtem klinischem Verlauf wiesen geringe Zahlen S100-positiver Zellen im Tumorgewebe auf, was analog zu Paragangliomen auf eine mögliche prognostische Relevanz hindeutet. Zusammenfassend erscheinen S100-positive Zellen in der Differenzialdiagnose thorakaler Paragangliome und pulmonaler Karzinoide kein verlässliches Kriterium zu sein, könnten jedoch bei der prognostischen Einordnung dieser Entität hilfreich sein.

Abstract

Paragangliomas have a classical histomorphology comprising a so-called “Zellballen” or nesting pattern with surrounding S100 protein positive sustentacular cells (SC) which form a meshwork with a wire-fence appearance. In adrenal and extra-adrenal paragangliomas the prevalence of SC is inversely associated with the patients’ outcome. In order to get more insight into the prevalence as well as the prognostic and differential diagnostic value of this cell population in pulmonary carcinoids, we investigated a panel of 26 tumorlets, 147 typical and atypical pulmonary carcinoids and ten thoracic paragangliomas immunohistochemically. We were able to demonstrate that S100 protein positive cells are similarly distributed in both thoracic paragangliomas and pulmonary carcinoids. Hence, the presence and distribution of these cells does not appear to represent a reliable criterion in differential diagnosis. Moreover, all pulmonary carcinoid patients with a worse outcome had low numbers of or no S100 protein positive cells in their tissue specimens. Thus, the prevalence of these cells may potentially aid in prognostic assessment of pulmonary carcinoids, especially in biopsies.

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Warth, A., Krysa, S., Zahel, T. et al. S100-Protein-positive Sustentakularzellen in pulmonalen Karzinoiden und thorakalen Paragangliomen. Pathologe 31, 379–384 (2010). https://doi.org/10.1007/s00292-010-1293-2

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