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Virchows Archiv
https://doi.org/10.1007/s00428-024-03975-6
REVIEW
Primary osseous tumors oftheorbit
MarielBedell1· RanaNaous1
Received: 31 July 2024 / Revised: 29 September 2024 / Accepted: 6 November 2024
© The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2024
Abstract
This review article focuses on the various primary osseous tumors of the orbit. Due to overlapping clinical, radiologic, and
histologic features, differentiating these entities can pose significant challenges diagnostically. In this review, emphasis is
placed on key distinguishing clinical, morphologic, immunophenotypic, and molecular characteristics. Also described are
important prognostic details, recurrence risks, and the gold standard treatment methods for each entity. Relevant genetic
syndrome associations are additionally covered. Orbital bone entities discussed include osteoma, osteoid osteoma, osteo-
blastoma, ossifying fibroma, fibrous dysplasia, aneurysmal bone cyst, osteosarcoma, Ewing sarcoma, and mesenchymal
chondrosarcoma.
Keywords Bone tumors· Primary· Orbit
Introduction
Osseous tumors of the orbital bone are rare. Although both
benign and malignant bone tumors can affect the orbit, the
majority of orbital bone tumors are benign. Their clinical
presentation is variable and includes headaches, double
vision, vision loss, proptosis, facial pain or deformity, sinus-
itis, or sometimes asymptomatically. On some occasions,
there is a delay between symptom onset and diagnosis. The
treatment of almost all osseous orbital tumors is surgical. We
provide here a review of osseous tumors originating from the
orbit. Osteoma, osteoid osteoma, osteoblastoma, ossifying
fibroma, fibrous dysplasia, and aneurysmal bone cyst are all
benign primary bone tumors found in the orbit. Malignant
primary bone tumors of the orbit include mainly osteosar-
coma, Ewing sarcoma and mesenchymal chondrosarcoma.
We will review the clinicopathologic and radiologic features
as well as molecular characteristics, treatment options, and
recurrence risk of each of these lesions.
Osteoma
Osteomas of the orbit are benign, slow growing, and com-
monly asymptomatic lesions that are incidentally found on
imaging studies secondary to their indolent growth and small
size (< 2cm). When symptomatic they may cause facial
deformity, facial pain, sinusitis, and headache [1]. On imag-
ing, osteomas are uniformly dense, well-marginated lesions
with a broad base of attachment to underlying cortex without
any evidence of cortical invasion [2] (Fig.1A). Osteomas
predominantly affect bones formed by membranous ossifi-
cation and commonly present in the skull bones. They tend
to localize to the surface of craniofacial bones most often
in the fourth through sixth decades of life, equally in males
and females. They are well-circumscribed tumors composed
of mature compact, spongiotic/trabecular or mixed compact
and spongiotic bone with usually inconspicuous osteoblas-
tic and osteoclastic activity and set in a loose fibrovascular
stroma (Fig.1B). Sino-orbital osteomas may have zones
indistinguishable from an osteoblastoma with characteristic
conspicuous osteoblastic and osteoclastic activity mimicking
osteoblastoma (Fig.1C), thus labeling them as osteoblas-
toma-like osteomas [3]. However, this diagnosis may be sub-
jective and varies among institutions as some would refer to
such lesions with a predominant osteoblastoma-like pattern
as osteoblastomas or osteoblastomas with areas of sclerosis.
Although most are considered hamartomas, a subset of
osteomas is associated with APC gene mutations [4, 5] or
* Rana Naous
naousr@upmc.edu
Mariel Bedell
bedellml@upmc.edu
1 Department ofPathology, University ofPittsburgh Medical
Center (UPMC), Shadyside Hospital, 5230 Centre Avenue,
WG02.6, Pittsburgh, PA15232, USA
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