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Chondrosarcoma

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Tumors and Tumor-Like Lesions of Bone

Abstract

Chondrosarcoma (CHS) represents 20–30 % of all malignant bone neoplasms and is the second most common solid malignant tumor of bone after ostegosarcoma. CHS is classified as primary or secondary if the tumor arises de novo or develops in a preexisting benign cartilage neoplasm (enchondroma or osteochondroma), respectively. By the same token, CHS is classified according to its anatomic location within a bone as central when it is located in the medullary cavity of a long bone (80–85 % of cases) or peripheral (about 10–15 % of cases) or juxtacortical or periosteal when the tumor is located on the surface of a bone (<1 % of cases). Microscopically, CHS is a group of different clinicopathological entities in which the common denominator is the presence of hyaline cartilage. The most common is the primary or conventional CHS that represents 85 % of all the CHSs and is a tumor composed predominantly of hyaline cartilage in various stages of differentiation. Its variants include a specific cell component such as a high-grade sarcoma component in dedifferentiated CHS, clear cells in clear-cell CHS, and a small-cell component in mesenchymal CHS. Each one of these subtypes has distinct clinical, radiological, histological, and genetic features.

Secondary chondrosarcoma may arise in a sporadic osteochondroma, in a genetic syndrome characterized by multiple osteochondromas (hereditary multiple exostosis), or in skeletal enchondromatosis (Ollier or Maffucci diseases). The prognosis of CHS depends on the histological grade, stage, and location of the tumor. According to the National Cancer Data Base Report, the relative 5-year survival rate of CHS is approximately 75 %, and patients who survive the first 10 years after diagnosis of conventional CHS will die of other causes rather than from CHS-related events. High-grade (grade 3) conventional CHS and dedifferentiated and mesenchymal CHSs account for those cases with less favorable prognosis.

Dedifferentiated chondrosarcoma is a rare malignant neoplasm characterized by a high-grade, nonchondroid sarcoma such as malignant fibrous histiocytoma, osteosarcoma, fibrosarcoma, rhabdomyosarcoma, or angiosarcoma associated with and apparently arising from a low-grade cartilage-forming tumor. Slightly more males than females are affected. The age range is wide, but the majority of tumors occur in patients over the fourth decade. The peak age incidence is the sixth decade of life. Sites of involvement are similar to ordinary chondrosarcoma. Radiographically, the existence of the two components (low- and high-grade) of dedifferentiated chondrosarcoma can frequently be recognized. Histologically, the hallmark of dedifferentiated chondrosarcoma is the coexistence of its two components: a low-grade cartilaginous precursor lesion and a high-grade sarcoma. Treatment: In general, the dedifferentiated component is more resistant to chemotherapy and radiation therapy than de novo malignant fibrous histiocytoma or osteosarcoma.

Mesenchymal chondrosarcoma is a rare distinct malignant neoplasm characterized by the presence of solid, highly cellular areas composed of round or slightly spindled primitive mesenchymal cells with foci of cartilaginous differentiation. The noncartilaginous elements usually predominate, and such lesions can be confused histologically with Ewing’s sarcoma, malignant lymphoma, or hemangiopericytoma. There is no sex predilection. Usually, it affects young adults and teenagers. The peak incidence is during the fourth followed by the third decade of life but can be seen at any age. Sites of involvement: It favors the maxilla, mandible, ribs, and vertebrae. Radiologically, a lytic defect that often contains stippled densities corresponding to small islands of mineralized cartilage is present. Radical surgery is the best therapy.

Clear-cell chondrosarcoma is a rare low-grade malignancy characterized by rounded cells with conspicuous clear or vacuolated cytoplasm and by a cartilaginous or chondroid matrix in at least some areas. Scattered osteoclast-type giant cells, occasional bone trabeculae, and aneurysmal bone cyst–like areas may also be present. It is a rare tumor that accounts for less than 2 % of all chondrosarcomas. The reported sex ratio, male to female, is 2:1. The peak incidence is in the fourth and fifth decades of life; however, individual cases are reported from the second to the seventh decades. The most common location is distal femur, followed by proximal humerus, proximal femur, proximal tibia, vertebrae, and ribs. Radiographically, one of the most constant findings is the propensity for the tumor to involve the epiphysis of a long bone and extend to the articular cartilage. The most common roentgenographic pattern is that of a purely lucent lesion. En bloc resection including a margin of normal bone and soft tissue is the treatment of choice.

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Authors and Affiliations

  1. Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, TX, USA

    Sergio Piña-Oviedo MD

  2. Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Weill Cornell University, Houston, TX, USA

    Jae Y. Ro MD

  3. Department of Pathology and Genomic Medicine, Methodist Hospital Physician Organization, Houston, TX, USA

    Alberto G. Ayala MD

  4. Houston Methodist Research Institute, Houston, TX, USA

    Alberto G. Ayala MD

  5. Weill Cornell Medical College of Cornell University, Houston, TX, USA

    Alberto G. Ayala MD

  6. Department of Pathology, Kyung Hee University School of Medicine, Seoul, South Korea

    Yong-Koo Park MD, PhD

Authors
  1. Sergio Piña-Oviedo MD
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  2. Jae Y. Ro MD
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  3. Alberto G. Ayala MD
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  4. Yong-Koo Park MD, PhD
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Correspondence to Alberto G. Ayala MD .

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  1. Laboratory of Orthopaedic Pathology, Buenos Aires, Argentina

    Eduardo Santini-Araujo

  2. Laboratory of Orthopaedic Pathology, Buenos Aires, Argentina

    Ricardo K. Kalil

  3. Villa Erbosa Hospital, University of Bologna, Bologna, Italy

    Franco Bertoni

  4. Department of Pathology, Kyung Hee University Hospital, Seoul, Korea, Republic of (South Korea)

    Yong-Koo Park

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Piña-Oviedo, S., Ro, J.Y., Ayala, A.G., Park, YK. (2015). Chondrosarcoma. In: Santini-Araujo, E., Kalil, R., Bertoni, F., Park, YK. (eds) Tumors and Tumor-Like Lesions of Bone. Springer, London. https://doi.org/10.1007/978-1-4471-6578-1_23

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