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. 2022 Mar;85(3):1065-1074.
doi: 10.1002/jemt.23976. Epub 2021 Nov 10.

Stromal cells of giant cell tumor of bone show primary cilia in giant cell tumor of bone

Tomás Castiella  1   2 , Pablo Iruzubieta  3 , Eva Monleón  3   2 , Mª José Cardiel  1 , Jesús Gómez-Vallejo  4 , Marta Monzón  3   2 , Mª Concepción Junquera  3   2
Affiliations

Stromal cells of giant cell tumor of bone show primary cilia in giant cell tumor of bone

Tomás Castiella et al. Microsc Res Tech. 2022 Mar.

Abstract

Giant cell tumor of bone (GCTB) is a locally aggressive primary bone neoplasm composed by tumoral stromal cells (SCs) and a reactive component that consists of monocytic/histiocytic cells that give rise by fusion to osteoclast-like multinucleated cells. Recently, specific Histone 3.3 mutations have been demonstrated in SCs of GCTB. Many of the pathways related to bone proliferation and regulation depend on the primary cilium, a microtubule-based organelle that protrudes outside the cell and acts as a sensorial antenna. In the present work, we aimed to study the presence and role of primary cilia in GCTB. Ultrastructural, immunohistochemical, and immunofluorescence studies were performed in order to demonstrate, for the first time, that the primary cilium is located in spindle-shaped SCs of GCTB. Moreover, we showed Hedgehog (Hh) signaling pathway activation in these cells. Hence, primary cilia may play a relevant role in GCTB tumorogenesis through Hh signaling activation in SCs. RESEARCH HIGHLIGHTS: Transmission electron microscopy allows describing and differentiating cellular subpopulations in giant cell tumor of bone (GCTB). The primary cilium is present in some tumoral stromal cells of GCTB. Hedgehog signalling is activated in these cells.

Keywords: Hh signaling pathway; giant cell tumor of bone; primary cilium; ultrastructural study.

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