Publication: Giant cell tumor of bone: An update, including spectrum of pathological features, pathogenesis, molecular profile and the differential diagnoses
Authors
Rekhi, Bharat ; Dave, Vinayak
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Publisher
Universidad de Murcia, Departamento de Biologia Celular e Histiologia
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DOI
https://doi.org/10.14670/HH-18-486
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info:eu-repo/semantics/article
Description
Abstract
Giant cell tumor of bone (GCTB) is an
enigmatic tumor. Despite its benign histological
appearance and clinical behavior in most cases, it is
associated with recurrences, uncommonly metastasis,
and rarely with a malignant transformation. During the
last few years, there has been a significant evolution in
the diagnosis and management of GCTB, including
discoveries related to the underlying pathogenesis
(RANK/RANK/OPG pathway), with treatment-related
implications in the form of denosumab (approved
inhibitor for targeting RANKL), leading to improved
surgical resections, especially in cases of recurrent, large
and borderline resectable tumors. Lately, a specific
Histone mutation, namely H3.3G34W underlying almost
all GCTBs has been discovered, further leading to the
identification of a highly sensitive and specific
immunohistochemical antibody marker, H3.3G34W,
which is very useful for an exact diagnosis of a GCTB,
including its differentiation from its various mimics,
which has significant implications. This review
describes clinicopathological features of a GCTB,
including its variable features, recent concepts,
underlying pathogenesis, post-denosumab related
changes and various entities that constitute its
differential diagnosis, including their molecular
signatures, with treatment-related implications
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Citation
Histology and Histopathology Vol. 38, nº2 (2023)
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