Table of Contents
Definition / general | Diagrams / tables | Clinical features | Radiology images | Prognostic factors | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosisCite this page: Pernick N. Chondrosarcoma (primary, secondary, periosteal). PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/bonechondrosarcoma.html. Accessed January 22nd, 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
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
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
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.
Contributed by Mark R. Wick, M.D.
AFIP images
Images hosted on other servers:
Positive stains
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