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Pacheco et al. J Cancer Metastasis Treat 2020;6:49 Journal of Cancer
DOI: 10.20517/2394-4722.2020.85 Metastasis and Treatment

Review Open Access

Primary malignant tumors of bone surface: a review
with emphasis in differential diagnosis

Marina Pacheco, Alberto Righi

Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna 40136, Italy.
Correspondence to: Dr. Alberto Righi, Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna 40136, Italy.
E-mail: alberto.righi@ior.it

How to cite this article: Pacheco M, Righi A. Primary malignant tumors of bone surface: a review with emphasis in differential
diagnosis. J Cancer Metastasis Treat 2020;6:49. http://dx.doi.org/10.20517/2394-4722.2020.85

Received: 27 Aug 2020 First Decision: 09 Nov 2020 Revised: 16 Nov 2020 Accepted: 07 Dec 2020 Published: 18 Dec 2020

Academic Editor: Ian Judson, Lucio Miele Copy Editor: Whitney Xu Production Editor: Jing Yu

Abstract
Surface tumors of the bone are broadly defined as a diverse group of osteogenic and chondrogenic benign and
malignant neoplasms that arise adjacent to the outer surface of cortical bone. They may be a cause of diagnostic
difficulty due to a degree of histological overlap, rarity, and nomenclature. In this review we summarize the different
histological types of primary malignant tumors of bone surface, namely, secondary peripheral chondrosarcoma,
periosteal chondrosarcoma, parosteal osteosarcoma, dedifferentiated parosteal osteosarcoma, periosteal
osteosarcoma, and high-grade surface osteosarcoma. We provide a comprehensive updated review of their
pathogenesis and highlight radiological, macroscopic, and histopathological features and recently available ancillary
diagnostic tools that may aid in the differential diagnosis.

Keywords: Secondary peripheral chondrosarcoma, periosteal chondrosarcoma, parosteal osteosarcoma, periosteal
osteosarcoma, high-grade surface osteosarcoma

INTRODUCTION
Primary malignant tumors of the bone surface are a group of osteogenic and chondrogenic neoplasms with
different clinical behaviors, which depend greatly on their histologic type. Their overall incidence is very
[1]
low, constituting approximately 3.5% of all primary bone tumors . Their rarity, overlapping morphology,
and the therapeutic implications of their correct identification make their diagnosis better rendered
by expert hands in an integrated, multidisciplinary setting. The aims of this review are to outline the

© The Author(s) 2020. Open Access This article is licensed under a Creative Commons Attribution 4.0
International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use,
sharing, adaptation, distribution and reproduction in any medium or format, for any purpose, even commercially, as long
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