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Chondrogenic Bone Tumors: the Importance of Imaging Characteristics Chondrogene Knochentumoren: Bildgebung Als Wegweiser?

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Chondrogenic Bone Tumors: the Importance of Imaging Characteristics Chondrogene Knochentumoren: Bildgebung Als Wegweiser? Published online: 2020-11-05 Review Chondrogenic Bone Tumors: The Importance of Imaging Characteristics Chondrogene Knochentumoren: Bildgebung als Wegweiser? Authors Hannes Engel1, Georg W. Herget2, Hannah Füllgraf3,RetoSutter4, Matthias Benndorf1, Fabian Bamberg1, Pia M. Jungmann1 Affiliations Methods Systematic PubMed literature research. Identifica- 1 Department of Radiology, Medical Center – University tion and review of studies analyzing and describing imaging of Freiburg, Faculty of Medicine, University of Freiburg, characteristics of chondrogenic bone tumors. Germany Results and conclusions The 2020 World Health Organiza- 2 Department of Orthopaedics and Traumatology, tion (WHO) classification system differentiates between be- Medical Center – University of Freiburg, Faculty nign, intermediate (locally aggressive or rarely metastasizing), of Medicine, University of Freiburg, Germany and malignant chondrogenic tumors. On imaging, typical 3 Institute for Surgical Pathology, Medical Center – findings of differentiated chondrogenic tumors are lobulated University of Freiburg, Faculty of Medicine, University patterns with a high signal on T2-weighted magnetic reso- of Freiburg, Germany nance imaging (MRI) and ring- and arc-like calcifications on 4 Department of Radiology, Balgrist University Hospital, conventional radiography and computed tomography (CT). Zurich, Switzerland; Faculty of Medicine, University of Depending on the entity, the prevalence of this chondrogenic Zurich, Switzerland pattern differs. While high grade tumors may be identified due to aggressive imaging patterns, the differentiation be- Key words tween benign and intermediate grade chondrogenic tumors cartilage, neoplasms, chondroma, osteochondroma, is challenging, even in an interdisciplinary approach. chondrosarcoma, diagnostic imaging Key Points: received 14.05.2020 ▪ The WHO defines benign, intermediate, and malignant accepted 09.09.2020 chondrogenic bone tumors published online 05.11.2020 ▪ Frequent benign tumors: osteochondroma and enchon- Bibliography droma; Frequent malignant tumor: conventional chondro- Fortschr Röntgenstr 2021; 193: 262–274 sarcoma ▪ DOI 10.1055/a-1288-1209 Differentiation between enchondroma versus low-grade ISSN 1438-9029 chondrosarcoma is challenging for radiologists and pa- © 2020. Thieme. All rights reserved. thologists ▪ Georg Thieme Verlag KG, Rüdigerstraße 14, Pain, deep scalloping, cortical destruction, bone expan- 70469 Stuttgart, Germany sion, soft tissue component: favor chondrosarcoma ▪ Potential malignant transformation of osteochondroma: Correspondence progression after skeletal maturity, cartilage cap thickness Priv.-Doz. Dr. Pia M. Jungmann (> 2 cm adult; > 3 cm child) This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. Department of Radiology, Medical Center – University ▪ Potentially helpful advanced imaging methods: Dynamic of Freiburg, Faculty of Medicine, University of Freiburg, MRI, texture analysis, FDG-PET/CT Germany, Hugstetter Straße 55, 79106 Freiburg, Germany Citation Format Tel.: +49/7 61/27 03 85 90 ▪ Engel H, Herget GW, Füllgraf H et al. Chondrogenic Bone [email protected] Tumors: The Importance of Imaging Characteristics. ABSTRACT Fortschr Röntgenstr 2021; 193: 262–274 Background Chondrogenic tumors are the most frequent ZUSAMMENFASSUNG primary bone tumors. Malignant chondrogenic tumors repre- Hintergrund Chondrogene Tumoren sind die häufigsten pri- sent about one quarter of malignant bone tumors. Benign mären Knochentumoren. Maligne chondrogene Tumoren re- chondrogenic bone tumors are frequent incidental findings präsentieren etwa ein Viertel der malignen Knochentumoren. at imaging. Radiological parameters may be helpful for identi- Benigne chondrogene Knochentumoren sind häufige radiolo- fication, characterization, and differential diagnosis. 262 Engel H et al. Chondrogenic Bone Tumors… Fortschr Röntgenstr 2021; 193: 262–274 | © 2020. Thieme. All rights reserved. gische Zufallsbefunde. Radiologische Parameter können hilf- radiologische Bild chondrogen differenzierter Tumoren ist reich sein zur Identifikation, Charakterisierung und Differen- gekennzeichnet durch ein lobuliertes Wachstumsmuster mit zialdiagnostik. einem hohen Signal in T2-gewichteten Sequenzen in der Mag- Methode Systematische Literaturrecherche mittels PubMed. netresonanztomografie (MRT) und durch ein meist ring- und Identifikation und kritische Beurteilung von Studien, welche bogenförmiges Verkalkungsmuster in der Projektionsradio- die Bildgebung chondrogener Knochentumoren analysieren grafie und der Computertomografie (CT). In Abhängigkeit oder beschreiben. der Entität ist dieses typische Muster unterschiedlich ausge- Ergebnisse und Schlussfolgerung Nach der World-Health- prägt. Während hochmaligne Tumoren häufig ein eindeutig Organization (WHO)-Klassifikation von 2020 werden benigne, malignes Wachstumsmuster zeigen, ist die Differenzierung intermediäre (lokal aggressiv oder selten metastasierend) und zwischen benignen und intermediären chondrogenen Tumo- maligne chondrogene Tumoren unterschieden. Das typische renauchinterdisziplinärschwierig. Introduction in the 2013 WHO classification and was assigned to the intermedi- ate group, both terms are used depending on location in the new Bone tumors and tumor-like lesions are frequent incidental find- 2020 WHO classification [6, 10]: When located in the appendicu- ings in the radiologist’s clinical routine. The World Health Organi- lar skeleton (long and short tubular bones), these tumors are ca- zation (WHO) classification of bone tumors is based on the histo- tegorized as ACT and assigned to the intermediate group; when logical structure of the primary tissue or the primary cell type. located in the axial skeleton (including the pelvis and base of the This article discusses chondrogenic bone tumors which represent skull), tumors with the same histology are designated as chondro- the largest group of bone tumors. A well differentiated chondroid sarcoma grade I and assigned to the malignant group to reflect matrix is characterized by a lobulated growth pattern and calcifi- the difference in biological behavior [11, 12]. cation patterns that are typical for cartilaginous tumors (▶ Fig. 1). The typical calcification patterns are the result of endochondral ossification. They are primarily popcorn-like or ring- and arc-like Benign chondrogenic tumors and can be visualized on conventional radiography and computed tomography (CT) [1]. However, according to Lodwick, the chon- Osteochondroma droid matrix is visible on conventional radiography only in 65 % of Osteochondromas (osteocartilaginous exostoses) are among the chondrosarcomas, 30 % of chondroblastomas, and 2 % of chon- most common benign bone tumors (approximately 20–50 %). It dromyxoid fibromas [2]. Due to its high water content, hydrophi- must be assumed here that the true prevalence is underestimated lic cartilage tissue is clearly hyperintense in T2-weighted MRI due to asymptomatic lesions [13]. Most osteochondromas are sequences [3, 4]. Calcifications are hypointense both in T2- and diagnosed in the second decade of life. Men are affected more T1-weighted sequences. The cartilage tissue is supplied with often than women [14]. Mutations in tumor suppressor genes blood via the capillaries in the septa and via the perichondrium EXT1 and EXT2 are usually found in the cartilage cap [15]. Osteo- so that ring- and arc-like contrast enhancement occurs after con- chondromas are often located in the region of the knee joint and trast administration [5]. the proximal humerus. The distal femur is most commonly affec- ted. The location is usually metaphyseal or metadiaphyseal. The WHO classification tumor can manifest either (i) in a broad-based manner (sessile) or (ii) as a pedunculated, spur-like expansile growth from the In the 2020 World Health Organization (WHO) classification, the bone surface. It is encompassed by a cartilage cap [9]. This expan- various entities of chondrogenic bone tumors are categorized as sile growth points in the direction of the diaphysis. There is benign, intermediate (locally aggressive and/or rarely metastasiz- pathognomonic continuity of the medullary cavity from the This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. ing), or malignant (▶ Table 1) [6]. In the 2013 WHO classification, bone to the osteochondroma as well as direct continuity of the the group of benign chondrogenic tumors including osteochon- cortex. This helps to differentiate Osteochondromas from paros- dromas and chondromas/enchondromas was expanded to in- teal osteosarcomas. The growth arises from the cartilage cap as clude osteochondromyxoma, subungual exostosis, and bizarre the actual tumor (component), which often shrinks on a relative parosteal osteochondromatous proliferation (BPOP, Nora’s lesion) basis during adolescence due to increasing ossification [16]. A [7]. Chondroblastomas and chondromyxoid fibromas were moved malignant transformation in children is extremely rare. Cartilage from the intermediate to the benign group in the 2020 WHO clas- cap thicknesses of > 3 cm in children and > 2 cm in adults are con- sification. Synovial chondromatosis was moved from the benign sidered suspicious [16]. In an analysis of 67 osteochondromas and to the intermediate group to reflect the locally aggressive growth 34 secondary peripheral chondrosarcomas, Bernard et al. did not pattern and the high risk for local recurrence
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