April 11, 2024

Chondrosarcoma is a malignant tumor of cartilage-producing cells which consist of a heterogeneous group of lesion with diverse morphological features and clinical behavior.

Chondrosarcoma is also called as tumor with hyperglycemia

It is the most common sarcoma of the bone greater than 20 years (25% of all sarcomas)

WHO Classification of Chondrosarcoma

  1. Primary and Secondary Chondrosarcoma
  2. Juxtacortical (Periosteal) Chondrosarcoma
  3. Dedifferentiated Chondrosarcoma
  4. Mesenchymal Chondrosarcoma
  5. Clear cell Chondrosarcoma

Primary Chondrosarcoma

90% of chondrosarcoma

Primary Chondrosarcoma is a malignant cartilage tumor arising centrally in a previously normal bone

It is 3rd most common after Myeloma and Osteosarcoma

It is also called Central / Conventional Chondrosarcoma

Etiology

Age: Adulthood ( Peak in 3rd- 7th decade)

Male > Female = 3:2

Site:- Pelvis is the most common site (Ilium), Proximal femur, Proximal humerus, distal femur, ribs

It is uncommon in small bones of hands and feet

Clinical Features

Pain

Pathological Fracture

X-Rays:

Expansion of the medullary portion of bone and thickening of the cortex

Periosteal reaction: absent/ scant

Endosteal scaffolding as well as annular, punctate, or comma-shaped stippled calcification (ring and arcs appearances)

Similar to Enchodromas

Endosteal scalloping of more than 2/3rd of the cortical thickness is suggestive of a more aggressive lesion.

It shows the different radiological lesions

AdequateAggressive
Cortical Expansion with handling thickeningCortical disruption + soft tissue masses

Predictor of malignancy:- Localization of axial skeletal:- Size larger than 5 cm

Symptomatic intramedullary cartilaginous tumor but doesn’t shows adaptive aggressive signs: Low-grade chondrosarcoma/ Enchrondomas

The appearance of lysis in the previously calcified area: herald tumor prognosis

CT Scan

It helps in the detection and characterization of lesions in the anatomical complex areas, especially the sacrum, and spine.

MRI Scan

The intramedullary extension is best demonstrated

Bone Scan

Radionucleotide uptake in the lesion ( Grade from 1-3)

GradeUptake
Grade 1Uptake less than that in ASIS
Grade 2Uptake similar to ASIS
Grade 3Uptake greater than ASIS

PET Scan: To detect differentiation from benign

Laboratory Diagnosis

Non-specific

Not contract CT: Lung most common site for metastasis

Pathology

Gross features

Glistening, translucent, blue-gray, or blue-white

Calky white area of calcification

Microscopic features

Irregular lobules of cartilage which permeates the host

Hyaline cartilaginous matrix with a variable amount of myxoid changes

As per cellular atypia (3- Grades)

GradeCellular atypia
Grade 1Moderately cellular (vis-a-vis enchondroma)
Grade 2More cellular shows more nuclear atypia
Grade 3Very cellular and shows pleomorphic and anaplastic tumor cells
Clinico-Pathological Grading

Treatment

Surgery is the only reliable treatment

It is not sensitive to chemotherapy and radiotherapy

Grade 1

In long bones (aggressive radiological features:- Wide excision)

Extended Intralesional curretage

It has advantageous function results that are superior to resection

The cartilaginous lesion in the pelvis and sacrum: Intralesional excision (Frequent reoccur)

Radiotherapy: Limited

Prognosis:

Most pulmonary metastasis within 5 years of initial presentation

Predictor of metastasis: Histological grade

Secondary Chondrosarcoma

Secondary chondrosarcoma is known to occur after being a precursor lesion

  • Osteochondroma
  • Enchondroma

Develop earlier than primary chondrosarcoma

Peripheral Chondrosarcoma: Malignant transformation within the cartilaginous cap

Clinical Features

The sudden onset of pain and increase in size suggests a malignant transformation in an enchondromas

X-Ray Features

Irregular mineralization and increased thickness of cartilaginous cap in osteochondromas

Pre-existing enchondromas: reveals destructive permeating of bone and development of a soft tissue mass

CT Scan/ MRI

Invasion of the surrounding tissue and marked myxoid changes

Pathology

Invasion of the surrounding tissue and marked myxoid changes

Treatment

Wide surgical excision of the lesion

Should not be violated ( cartilaginous cap):- during resection of tumor arising in osteochondromas

Periosteal Chondrosarcoma

It is also called Juxta-cortical chondrosarcoma

It arises from the outer surface, the highest incidence is seen in 4th decade

Radiological Features

Round in shape and lies on the surface of the bone with indistinct and irregular

Radiolucent lobulated lesion: Stippled calcification and tends to affect the metaphyseal cortex

Dedifferentiated Chondrosarcoma

Dedifferentiated Chondrosarcoma is the most malignant of all chondrosarcoma and has a very poor prognosis

It has two different pathological tissue components

  • Well-differentiated cartilage tumor (Low-grade chondrosarcoma)
  • High-grade non-cartilaginous sarcoma

Average age: 50-60 years

Male=Female

Mesenchymal Chondrosarcoma

Mesenchymal Chondrosarcoma is a biomorphic pattern- highly differentiated, small round cells and an island of well-differentiated hyaline cartilage

Craniofacial bones: Jaw Bones

Meninges: Most common site for extraskeletal involvement

Clear Cell Chondrosarcoma

Clear Cell Chondrosarcoma has a clinical behavior that is less aggressive than conventional chondrosarcoma

Predilection: Epiphyseal End of Long Bone

Histological it is characterized by the presence of bland clear cells in addition to the hyaline cartilage

Age predisposition: 25-50 years

2/3rd of the lesion: Femoral head or humerus head

Chordoma

Chordoma is a rare, low to the intermediate-grade malignant bone tumor of the cranium and spine.

1- 4 % of all bone malignancy

Age:

Spheno-occipital tumors (35-40%): Usually develop in young adults

Sacro-coccygeal tumors (40-50%): Develop after 40 years of age

No age in immune

Male: Female = 1.8:1

Clinical Features

Chordoma is a slow-growing tumor that is insidious in onset

Sacrococcygeal tumors show symptoms due to expansions and bone destruction

Presents with pain in the anal and rectal region

Pain is mild and intermittent in nature

Other features :

  • Constipation
  • Urinary difficulty

Neurological Deficit: More likely to occur in chordomas of the mobile spine than in the sacrococcygeal region

Skull bone Chordomas present with cranial nerve palsies and endocrinopathies and rarely epistasis and intracranial hemorrhages

X-Ray Features

X-ray features show mid-line lesion and often appears on plain radiographs as a destructive bone lesion with an epicenter in the vertebral body and surrounding soft tissue mass

Pathology

Microscopic Lobulated and shows fibrous septa

Have abundant: Clear cytoplasm ( Physaliferous cells)

Chondroid variant: Hyaline, myxoid cartilages

Tumor cells: S100 protein, pan-cytokeratin, EMA

Treatment options

Complete En-bloc surgical resection

Surgery is extensive, and necessary, sacrificing several sacral nerves at times resection of bowel loops with colostomy, complete sacral and bony reconstruction with allograft

The tumor is radioresistant, radiotherapy is done only for surgically inaccessible sites.

See also: Ewing Sarcoma

See also: Osteosarcoma

See also: Musculoskeletal Malignancy: Management

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