Chondrosarcoma

Chondrosarcoma is a malignant bone tumor characterized by the production of a cartilaginous matrix. This bone tumor most commonly affects adults over the age of 50. Chondrosarcoma usually presents with a slowly increasing mass (or swelling) with a dull achy pain. Depending on the tumor’s location, this malignancy may also be associated with symptoms of nerve compression. The diagnosis is established based on imaging characteristics and tissue biopsy. The mainstay of treatment is surgical excision. Prognosis depends on the aggressiveness of the tumor, which is determined by the histological grading system.

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Overview

Definition

Chondrosarcoma is a malignant bone tumor characterized by the production of a cartilaginous matrix.

Epidemiology

  • Primary malignant bone tumors:  
    • 6th most common neoplasm in children
    • 3rd most common neoplasm in adolescents and young adults
    • Approximately 3,000 primary malignant bone tumors are diagnosed annually in the United States.
    • Approximately 1,500 deaths result from primary malignant bone tumors annually.
  • Chondrosarcoma:
    • 20%–25% of primary bone malignancies
    • Highest incidence in adults over 50
    • Pelvis and long bones are most commonly affected.

Etiology

  • Can arise:
    • De novo from normal bone
    • Via malignant transformation of enchondroma/osteochondroma
  • Myc amplification, multiple mutations, and chromosomal structural anomalies have been implicated in pathogenesis.
  • Predisposing inherited disorders:
    • Ollier disease 
    • Maffucci syndrome

Pathophysiology

  • Mutations and chromosomal abnormalities play a major role in pathogenesis.
  • Normally, chondrocytes within a growth plate undergo hypertrophy, differentiation, and apoptosis to allow for the ingrowth of vessels and osteoblasts (allowing for bone formation):
    • This process becomes dysregulated in chondrosarcoma formation.
  • There are many histological subtypes:
    • Most common subtype is conventional (hyaline cartilage producing) chondrosarcoma, accounting for 80%–90% of these tumors.
  • Morphology:
    • Translucent, lobular, cartilaginous cut surface with cystic/mucoid areas
    • Abundant cartilage matrix production
  • Tumor’s aggressiveness determines histological grade:
    • Tumors will be graded 1–4.
Low-grade chondrosarcoma

Microscopic findings in low-grade chondrosarcoma (H&E):
a: Hypocellular chondroid lobules on low-power magnification
b: Host bone permeation is a hallmark of chondrosarcoma.

Image: “Microscopic findings in low grade chondrosarcoma” by Department of Orthopaedics and Traumatology, Bozok University, Yozgat, Turkey. License: CC BY 4.0

Clinical Presentation

  • Longstanding dull, achy pain with swelling and redness
  • Progressive increase in the size of the mass
  • Neurological symptoms if the tumor is close to a neurovascular bundle:
    • Lumbosacral plexus in the pelvis
    • Cranial nerves in the base of the skull
  • Limitation of range of motion (if close to the joint)
  • Pathologic fracture
  • Most will grow slowly and have low metastatic potential.
  • When spreading occurs, the spread is primarily to the lungs.
Chondrosarcoma on shoulder

Chondrosarcoma of the scapula:
Tumor measures 38 x 33 x 30 cm (14.96 x 12.99 x 11.81 in).

Image: “Scapular chondrosarcoma” by Department of Orthopedic Surgery, the second hospital of Jilin University, Changchun, China. License: CC BY 2.0

Diagnosis

History and physical exam

  • Vague pain/swelling
    • Gradually increasing
    • May be worse at night
  • Neurologic symptoms (if nerve compression is present)
  • History of relevant congenital diseases
  • Palpable mass

Imaging

  • X-ray:
    • Calcification in the medullary cavity (popcorn calcification)
    • Radiolucency
    • Usually, no pronounced periosteal reaction
  • MRI: 
    • Test of choice for primary tumor evaluation
    • Evaluates the extent of the tumor, marrow, and soft tissue involvement
  • CT and bone scan: 
    • For primary tumor and distant metastatic disease evaluation
    • May show matrix mineralization, endosteal scalloping, and/or cortical breach
Pelvis chondrosarcoma

Chondrosarcoma:
Anteroposterior view shows tumor at the right pubis and periacetabulum that extends to the left pubic area.

Image: “Anteroposterior radiograph of the pelvis” by Department of Orthopaedic Surgery, Faculty of Medicine, Siriraj Hospital, Mahidol University, 2 Prannok Road, Bangkok, 10700, Thailand. License: CC BY 2.0

Biopsy

  • Definitive diagnosis
  • Should be taken from the most aggressive-appearing portion of a lesion

Management

Surgery

  • Offers the only chance of cure
  • Wide surgical excision is the mainstay of treatment.
  • In some central low-grade lesions, the intralesional curettage with local phenolization or cryotherapy followed by bone grafting of the cavity can be performed.
  • For peripheral lesions arising in osteochondromas, complete removal of cartilage cap is recommended.

Radiation

  • Chondrosarcomas are relatively radioresistant.
  • Radiation can be used for palliation.
  • For patients with incomplete resection or when resection is not feasible

Chemotherapy

  • Chondrosarcomas are generally chemoresistant.
  • Not used as adjuvant or neoadjuvant treatment
  • Role in the treatment of metastatic disease is also limited.

Prognosis

  • Determined by grade
  • 83% 5-year survival rate for grade 1 tumors
    • Falls to 53% for grade 2 and grade 3 tumors

Differential Diagnosis

  • Ewing sarcoma: primary bone malignancy composed of poorly differentiated cells that most commonly affects adolescents. This malignancy presents with localized pain and swelling. Diagnosis is established with imaging and biopsy. Management is based on chemotherapy and surgical resection.
  • Osteosarcoma: a malignant bone tumor characterized by the production of immature bone. Most common in children and young adults. This tumor presents with pain, swelling, palpable mass, and pathologic fractures. Diagnosis is suspected on imaging and confirmed with tissue biopsy. Management includes surgical resection and systemic chemotherapy.
  • Bone metastases: metastatic bone lesions from primary cancers arising at other sites, most commonly prostate, breast, and lung. These metastases present with bone pain, pathologic fractures, and constitutional symptoms. Diagnosis is made based on clinical history and imaging. Treatment includes systemic chemotherapy as well as supportive measures aimed at minimizing pain and pathologic fractures.
  • Benign bone tumors: more common than malignant bone tumors. These types of tumors include enchondroma, osteochondroma, nonossifying fibroma, chondroblastoma, osteoid osteoma, osteoblastoma, periosteal chondroma, giant cell tumor, and chondromyxoid fibroma. Many benign bone tumors, however, may present with the risk of malignant transformation. 
  • Osteomyelitis: bone infection most commonly due to pyogenic bacteria in the setting of orthopedic injury or a chronic nonhealing wound that erodes into the bone. Presents with localized pain, signs of inflammation/infection, and fever; can progress to sepsis and limb loss. Treatment is aggressive antibiotic therapy with or without surgical debridement.
  • Orthopedic injury: strains, sprain, growing pain, and fractures are the precipitating events that bring bony tumors to clinical attention. However, a more innocent injury or process may be to blame when considering the possibility of a bony neoplasm. Orthopedic injury is characterized by localized pain and inflammation/swelling. Diagnosis is usually made clinically, and management is supportive.

References

  1. Gelderblom A.J., & Bovee J. VMG. (2021). Chondrosarcoma. Retrieved March 13, 2021, from https://www.uptodate.com/contents/chondrosarcoma
  2. Kasper, D., Fauci, A., Hauser, S., Longo, D., Jameson, J., & Loscalzo, J.  (2012). Harrison’s principles of internal medicine (18th edition.). New York: McGraw Hill Education.
  3. Lakshmanan P. (2020). Chondrosarcoma. Retrieved March 13, 2021, from https://emedicine.medscape.com/article/1258236-overview
  4. Kumar, V., Abbas, A. K., & Aster, J. C. (2015). Bones, joints, and soft tissue tumors. In Robbins and Cotran pathologic basis of disease (9th ed.), Bone tumors and tumor-like lesions (pp. 1197–1203). Elsevier Gezondheidszorg.
  5. Sticco K. (2020). Chondrosarcoma. Retrieved February 26, 2021, from https://www.statpearls.com/articlelibrary/viewarticle/19483/

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