Primary Cartilage-Forming Tumors Flashcards
(29 cards)
What is a Chondroma/Enchondroma?
Benign, solitary tumors made of hyaline cartilage either inside bone (enchondroma) or on the bone surface (juxtacortical chondroma).
What age group is commonly affected by Chondroma/Enchondroma?
People between 20–40 years old.
Where are Chondromas commonly located?
Small bones of hands and feet, long bones, and pelvis.
What syndromes are associated with multiple enchondromas?
Ollier disease and Maffucci syndrome.
What mutation is involved in multiple enchondromas?
IDH (isocitrate dehydrogenase) point mutations.
What risk is associated with multiple enchondromas?
Malignant transformation to chondrosarcoma in 1/3 of cases.
How does a Chondroma appear morphologically?
As a well-circumscribed mass of mature cartilage.
What is an Osteochondroma (Exostosis)?
A benign cartilage-capped bone outgrowth near the growth plate.
Are Osteochondromas usually solitary or multiple?
They can be single or inherited as multiple (Hereditary Multiple Exostoses).
Where do Osteochondromas commonly arise?
From metaphysis near the growth plate, mostly around the knee.
What gene mutations are associated with Osteochondromas?
Inactivating mutations of EXT1 or EXT2 genes.
What is the risk of malignant transformation for solitary Osteochondromas?
Very rare (<2%).
What increases the risk of malignancy in Osteochondromas?
Hereditary Multiple Exostoses (up to 10%) or a thick cartilage cap (>2-3 cm).
What is the histology of an Osteochondroma?
Cartilage cap overlying bone and bone marrow, may become ossified.
What is a Chondrosarcoma?
A malignant tumor where mesenchymal cells produce cartilaginous matrix.
What age group is commonly affected by Chondrosarcoma?
Middle-aged and older adults (40–60 years).
Is Chondrosarcoma more common in males or females?
More common in males (M>F).
Where is Chondrosarcoma commonly located?
Axial skeleton (pelvis, shoulder, ribs) and proximal femur; rarely distal extremities.
Is Chondrosarcoma usually primary or secondary?
Mostly primary; secondary cases arise from multiple enchondromas or osteochondromas.
How does Chondrosarcoma appear grossly?
As a glistening mass in the medullary cavity.
What is seen in the histology of Chondrosarcoma?
Chondrocytes with variable pleomorphism and binucleation, no osteoid matrix.
What determines prognosis in Chondrosarcoma?
The tumor grade.
