Chondrosarcoma (primary, secondary, periosteal)

Bone & joints

Chondrosarcoma

Author: Nat Pernick, M.D.

Topic Completed: 1 June 2005

Minor changes: 22 February 2021

Copyright: 2003-2021, PathologyOutlines.com, Inc.

PubMed search: chondrosarcoma [title]

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Table of Contents

Cite this page: Pernick N. Chondrosarcoma (primary, secondary, periosteal). PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/bonechondrosarcoma.html. Accessed March 4th, 2021.

Definition / general

Malignant cartilage forming tumor that does not produce osteoid
May arise from osteochondroma
Third most common bone malignancy after myeloma and osteosarcoma
Most common subtype of chondrosarcoma
Usually ages 30 - 60 years, 75% males
16% occur in patients age 20 years or less, may be higher grade and at different sites (Am J Surg Pathol 1987;11:930)
Often large painful tumors of long bones or ribs that grow rapidly during adolescence and reach 8 cm or larger
Associated with preexisting enchondroma, but not with chondroblastoma, osteochondroma, fibrous dysplasia or Paget’s disease
Childhood tumors usually involve extremities and are often chondroblastic osteosarcomas
Conventional tumors are divided by location into central, peripheral and juxtacortical / periosteal forms
Tumors often recur at a higher histologic grade
Poorly differentiated tumors are uncommon, recur locally due to satellite nodules; metastasize early to lungs, only rarely to lymph nodes
Sites: large bones - pelvis, ribs, femur, humerus, vertebrae; unusual in hands, feet, jaw, skull
Xray correlation: presume malignant if large tumor of long bones or grows rapidly during adolescence to 8 cm or more; have fluffy calcification, poorly defined margins, erosion or thickening of cortex; usually no periosteal new bone formation
Grading: based on cellularity and nuclear changes in chondrocytes; well, moderate or poorly differentiated correspond to grades 1 - 3; grade 4 is spindled tumor representing either chondroblastic osteosarcoma or dedifferentiated chondrosarcoma
- Well differentiated: less cellular with only a few double nucleated cells and mild / moderate atypia; not well circumscribed, lobulated architecture with abundant cartilaginous matrix separated by narrow fibrovascular bands; tumor cells resemble chondroma; permeate existing trabecular bone and fill marrow space; lie in lacunar space surrounding hyaline cartilaginous matrix; malignant features more obvious at growing edge of tumor; may have reactive thickening of cortex
- Poorly differentiated: marked hypercellularity, extreme pleomorphism with markedly hyperchromatic nuclei; bizarre tumor giant cells and small cells, frequent mitotic figures; usually mixed with other grades; tumor cells destroy cortex and form soft tissue mass

Diagrams / tables

Images hosted on other servers:

Chondrosarcoma

Clinical features

Approximately 10% - 15% of chondrosarcomas arise secondary to a preexisting condition, including exostosis (solitary or multiple), chondrodysplasia, multiple chondromas
- Usually low grade with excellent prognosis
- Patients usually younger than conventional chondrosarcoma
- Usually presents as change in size or symptoms of preexisting lesion

Radiology images

Contributed by Dr. Mark R. Wick

CT scan

Distal femur Xray

Femur Xray

Scapula Xray

AFIP images

Central chondrosarcoma

Pubic symphysis

Scapula

Prognostic factors

Grading important for 5 year survival: well differentiated - 78%, moderate - 53%, poorly differentiated - 22%
Distant metastases occur in 4% of well differentiated vs. 30% of higher grade tumors

Case reports

68 year old woman with squamous cell carcinoma (Hum Pathol 1986;17:317)

Treatment

Since often implants in soft tissue after biopsy, wide en bloc excision advocated except for well differentiated tumors, which are amenable to conservative therapy
Patients may have local recurrence or metastases up to 20 years later

Gross description

Pearly white or light blue, often with focal calcification
May have small cysts or myxoid change

Gross images

Contributed by Dr. Mark R. Wick

Grade 1 pubis

Proximal femur

AFIP images

Rib

Lobular configuration

Intramedullary tumor

Knobby appearance

Juxtacortical chondrosarcoma

Microscopic (histologic) description

Tumor cells produce cartilaginous matrix
Either well, moderate or poorly differentiated
May have only minor or focal atypia, but consider malignant if malignant radiologic features (see above)
No direct osteoid or bone formation by tumor cells (if present, classify as osteosarcoma, although may be nonneoplastic bone)
Intracytoplasmic hyaline globules common in low grade tumors (Hum Pathol 1994;25:1283)
May have rows of cuboidal cells in myxoid background, resembling chordom, but chondrosarcoma is keratin-

Microscopic (histologic) images

Contributed by Kelly Magliocca, D.D.S., M.P.H.

Chondrosarcoma

Contributed by Mark R. Wick, M.D.

Myxoid

Grade 1

Grade 2

Hyaline type grade 1

Hyaline type grade 2

Hyaline type grade 3

Invading bone

AFIP images

Well differentiated

Grade 1

Grade 2

Grade 3

Spindle cells

Reactive new bone

Edge infiltrates bone marrow

Penetrates cortex

Multinucleated stellate cell

Juxtacortical chondrosarcoma

Images hosted on other servers:

Grade 2 chondrosarcoma

Grade 3

Positive stains

S100 (nuclear and cytoplasmic)
Staining resembles adult cartilage in well differentiated tumors or fetal cartilage in poorly differentiated tumors
High grade tumors may be p53+

Negative stains

Neural type cadherin (Arch Pathol Lab Med 2002;126:425)

Electron microscopy description

Glycogen, lipid droplets, dilated cisternae of granular endoplasmic reticulum

Molecular / cytogenetics description

Often 20q+, 8q+

Differential diagnosis

Chondroma vs. well differentiated chondrosarcoma: xray is determinative; for chondrosarcoma, must see permeation of tumor through cortex into soft tissue
Osteosarcoma: tumor cells make bone