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> Table of Contents > Volume 1 > Section I - Skin, Soft Tissue, Bone, and Joints > 8 - Bone Tumors > Tables > TABLE 8.7 Clinicopathologic Features of the Different Types of Chondrosarcoma

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BONE SARCOMAS
condensed chromatin and are evenly dispersed. Binucleated cells are infrequent, and mitoses are absent. Occasionally, degenerative features such as ischemic necrosis or calcifications are found (Fig. 8.8). The
[+] STAGING OF distinction between an enchondroma and ACT/CS1 can be difficult radiologically (12). Moreover, this distinction is also difficult on histology (13), causing high interobserver variability (14,15). Mucomyxoid matrix
BONE SARCOMAS degeneration and infiltrative growth (entrapment of preexisting host bone) are most predictive of ACT/CS1 (14). Immunohistochemistry or molecular diagnostics cannot help in this differential diagnosis. Radiologic
presentation, localization, and age should also be taken into account. For instance, in the phalanges, the matrix of enchondromas can contain more myxoid features, and the lesion may be more cellular. At this
[+] CONDITIONS
location, the main characteristics of malignancy are the presence of mitoses, cortical breakthrough, and soft-tissue involvement (11). Malignant transformation of enchondromas is thought to be extremely rare (<1%).
SIMULATING
Multiple enchondromas are seen in the context of nonhereditary Ollier disease (multiple enchondromas with a unilateral predominance) (Fig. 8.9) and Maffucci syndrome (multiple enchondromas combined with
NEOPLASMS OF
[spindle cell] hemangiomas) (16) caused by somatic mosaic mutations in the IDH1 or IDH2 gene (17,18). In these conditions, the risk of malignant transformation is increased to 18% to 46%, depending on the extent
BONE
and localization of the lesions (19). Solitary enchondromas also harbor mutations in the IDH1 or IDH2 gene in approximately 50% (20).
-
ACKNOWLEDGMENTS
FIGURE 8.7 Enchondroma. The lesion consists of lobular, relatively cell-poor hyaline cartilage, often demarcated by a zone of reactive bone formation (encasement)
- REFERENCES indicating slow growth.
[+] Figures

[-] Tables

- TABLE 8.1
Common
Spatial
View Figure
Distribution of
Bone Tumors in
Long Bones

- TABLE 8.2
Tumor
Syndromes in
Which Bone
Tumors Are
Frequently FIGURE 8.8 Enchondroma. The cells have nuclei with condensed chromatin and are irregularly dispersed. Binucleated cells are infrequent, and mitoses are absent.
Encountered Necrosis and irregular calcifications can be prominent.

- TABLE 8.3
Specific
Genetic
Changes in
Bone Tumors
That Can Be View Figure
Used in
Diagnosis

- TABLE 8.4
Bone Sarcoma
Grading

- TABLE 8.5
Musculoskeletal
Tumor Society
Periosteal Chondroma
Staging System Periosteal chondroma is a rare, benign hyaline cartilage neoplasm of bone surface that arises from the periosteum and is often located on the fingers or proximal humerus (1). On
for Bone P.286
Tumors radiographic imaging, a small (<5 cm), sharply demarcated tumor is seen on the surface of bone, with possible erosion of the exterior cortex, whereas marrow invasion is absent. In contrast to enchondroma, the
mixture of low and highly cellular areas, the presence of binucleated cells, hyperchromasia of the nuclei, myxoid change of the matrix, and nuclear pleomorphism are histologically tolerated, whereas these features
- TABLE 8.6 would lead to a diagnosis of chondrosarcoma if the tumor were located outside the periosteal area (21,22). The distinction between a periosteal chondroma and a periosteal chondrosarcoma is mainly based on the
TNM Staging presence of cortical destruction in the case of a periosteal chondrosarcoma. Furthermore, periosteal chondrosarcomas are usually more than 5 cm in size (23). Approximately 70% of periosteal chondromas harbor
System for mutations in IDH1 (20).
Bone Tumors

- TABLE 8.7
Clinicopathologic FIGURE 8.9 Ollier disease. Conventional radiograph of the hand demonstrating multiple enchondromas affecting multiple bones.
Features of the
Different Types
of
Chondrosarcoma

- TABLE 8.8
Osteoclastic
Giant Cell
Containing
Tumors and
Tumorlike
View Figure
Lesions of
Bone

- TABLE 8.9
Vascular
Tumors Subungual Exostosis and Bizarre Parosteal Osteochondromatous Proliferation of Bone (Nora Lesion)
Occurring in Bizarre parosteal osteochondromatous proliferation (BPOP) is an osteochondromatous proliferation involving the surface of the small bones of the hands and feet (1,24,25). There is a wide age range, with no sex
Bone predilection. In contrast to osteochondroma, the lesion is not continuous with the underlying cortex and medulla. Histologically, bone and spindle cells are seen, with hypercellular cartilage with enlarged
chondrocytes and irregular bone-cartilage interfaces (Fig. 8.10). A characteristic, strikingly blue color can be found, even after decalcification (“blue bone”). There is marked proliferative activity. In contrast to
[+] Section II - Breast
chondrosarcoma, the cartilaginous matrix is typically fibrillary. The lesion may rapidly recur. A recurrent chromosomal translocation t(1;17) has been found (26).

Subungual exostosis is a benign osteochondromatous proliferation involving the distal phalanx (1). The histology resembles BPOP, although the classic blue bone is lacking, and the lesion is more organized with a
[+] Section III - Central
gradual maturation from peripheral spindle cell proliferation, to hyaline cartilage, to trabecular bone (Fig. 8.10). Subungual exostosis, as the name implies, is typically located at the distal phalanx, whereas BPOP is
Nervous System
not located at that site. In contrast to osteochondroma, which is extremely rare on the digits, the cartilaginous matrix is highly fibrillary. Subungual exostosis is characterized by a recurrent chromosomal
[+] Section IV - Endocrine translocation t(X;6), rearranging the COL12A1 and COL4A5 genes in chromosome bands 6q13-14 and Xq22 (27), upregulating expression of the IRS4 gene (28).
System

[+] Section V -
FIGURE 8.10 Highly cellular cartilage, with a fibrocartilaginous extracellular matrix, displaying a gradual transition into cellular fibroblastic stroma, can be seen in
Hematopoietic and
both subungual exostosis and bizarre parosteal osteochondromatous proliferation (Nora lesion).
Lymphatic Systems

[+] Section VI - Head and

Neck

[+] Section VII -

Intrathoracic Organs and
Blood Vessels View Figure

[+] Volume 2

BACK OF BOOK ↑

[+] Index

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