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Tenosynovialer Riesenzelltumor

Morphologische, ultrastrukturelle und immunhistochemische Befunde sowie Differenzialdiagnose riesenzellhaltiger Tumoren des Weichgewebes

Tenosynovial giant cell tumor

  • Schwerpunkt: Weichgewebstumoren
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Zusammenfassung

Die morphologischen, ultrastrukturellen und immunhistochemischen Befunde an 12 tenosynovialen Riesenzelltumoren (TSRZT) der diffusen Form/pigmentierte villonoduläre Synovialitis (PVNS) wurden im Vergleich zur Morphologie von 30 Tumoren der lokalisierten Form (Riesenzelltumoren der Sehnenscheide) erstellt sowie im Hinblick auf die formale Pathogenese, Abgrenzung der beiden Varianten und Differenzialdiagnose diskutiert.

Die diffuse Form des TSRZT/PVNS ist charakterisiert durch ein auffallendes Vaskularisationsmuster mit teils abnormem Strukturmuster (lückenhafte Endothel-, Perizytenanordnungen). Die Tumorproliferate des TSRZT führen offenbar zu stärkeren Kompressionsphänomenen der Gefäße, sodass zusammen mit der sehr ausgeprägten Vaskularisation bei ultrastrukturell nachweisbaren teils „fragilen“ Gefäßwandstrukturen eine vermehrte Einblutung des Gewebes mit Hämosiderose und Ansammlung mehrkerniger (Osteoklasten-artiger) Riesenzellen gefördert wird. Mikrohämorrhagiefoci mit nahezu immer angrenzenden mehrkernigen Riesenzellen waren in 83% der diffusen Tumoren sowie in 67% der lokalisierten Tumoren zu belegen. Neben den dünnwandigen ektatischen, teilweise dicht gelagerten Gefäßstrukturen waren als typische morphologische Befunde des diffusen TSRZT „Riesenhämosideringranula“, meist zahlreiche „Ringsiderophagen“ sowie pseudoalveoläre Spalträume und irregulär anastomosierende Synovialiszotten nachzuweisen. Weder die mitotische Aktivität, die Anzahl der Riesenzellen noch die Kernanzahl der Riesenzellen (als Maß für die Zellgröße) zeigten statistisch signifikante Unterschiede zwischen lokalisierter und diffuser Form des TSRZT. Immunhistochemisch fand sich in der diffusen Variante neben CD68 fokal eine Expression von CD31 (in 75%) und Calretinin (in 63%).

Abstract

Morphological, ultrastructural, and immunohistochemical findings of 12 diffuse type-tenosynovial giant cell tumors/pigmented villonodular synovitis are presented compared to 30 localized tenosynovial giant cell tumors (giant cell tumor of tendon sheath).

Diffuse-type-tenosynovial giant cell tumor is characterized by a striking vascularisation pattern composed of densely arranged thin-walled, partly slit-like and partly hyalinized small blood vessels within the papillary synovial fronds. These vessels may show abnormal structures with incompletely arranged endothelial cells/pericytes. The fibrohistiocytic tumor cells probably cause considerable compression/distortion or destruction of the small vessels which might be responsible for an increased blood deposition and massive hemosiderosis. Accompanying multinucleated osteoclast-like giant cells seemingly are recruited from circulating blood monocytes. Microhemorrhagic foci with multinucleated giant cells could be detected in 83% of diffuse-type and 67% of localized-type tumors. Apart from the described vessels, typical morphological findings in diffuse-type tenosynovial giant cell tumors included “giant” hemosiderotic granules, (at least 2–3 times the diameter of an erythrocyte) “giant” siderophages, pseudoalveolar clefts and irregularly anastomosing synovial fronds. Neither mitotic rate nor the amount of giant cells/amount of nuclei of giant cells revealed statistically significant differences between localized-type and diffuse-type of tenosynovial giant cell tumor. Immunohistochemically, the diffuse-type exhibited focal expression of CD31 (in 75% of tumors) and calretinin (in 63%) besides CD68-staining.

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Danksagung

Die Autoren danken Frau Cornelia Troske für das Fotodesign.

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Authors and Affiliations

  1. Institut für Pathologie, Register für Gliedmaßentumoren, Berufsgenossenschaftliche Kliniken Bergmannsheil, Ruhr-Universität Bochum

    C. Kuhnen & K.-M. Müller

  2. Berufsgenossenschaftliches Forschungsinstitut für Arbeitsmedizin, Berufsgenossenschaftliche Kliniken Bergmannsheil, Ruhr-Universität Bochum

    S. Rabstein

  3. Max-Planck-Institut für Molekulare Physiologie Dortmund

    A. Kasprzynski & P. Herter

  4. Institut für Pathologie, Berufsgenossenschaftliche Kliniken Bergmannsheil, Bürkle-de-la-Camp-Platz 1, 44789, Bochum

    C. Kuhnen

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  1. C. Kuhnen
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  4. A. Kasprzynski
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Correspondence to C. Kuhnen.

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Kuhnen, C., Müller, KM., Rabstein, S. et al. Tenosynovialer Riesenzelltumor. Pathologe 26, 96–110 (2005). https://doi.org/10.1007/s00292-004-0740-3

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  • DOI: https://doi.org/10.1007/s00292-004-0740-3

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