Secondary Chondrosarcoma

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Patrick P. Lin, MD, et al Table 1 Major Series on Secondary Chondrosarcoma Study No. of Cases Preexisting Lesion (No.) Grade 14 (No. of Cases) 52 Solitary OC (21), HME (4), Coley and N/A Higinbotham 6 enchondroma (23), Ollier disease (4) Garrison et al 7 75 Solitary OC (40), HME (35) 1 (64), 2 (10), 3 (1) Hudson et al 8 15 Solitary OC (12), HME (3) 1 (11), 2 (3), 3 (1) Sun et al 12 5 Maffucci syndrome (all) 1 (1), 2 (3), N/A (1) Liuetal 9 12 Ollier disease (all) 1 (10), 2 (2) Merchan et al 10 4 HME (all) 1 (4) Wuisman 45 Solitary OC (16), HME (29) 1 (30), 2 (13), 3 (2) et al 13 Schaison 29 Solitary OC (17), Ollier 1(9),2(18),N/A(2) et al 11 disease (12) Ahmed et al 4 107 Solitary OC (61), HME (46) 1 (97), 2 (10) Altayetal 5 32 Solitary OC (14), HME (10), enchondroma (6), Ollier disease (1), Maffucci syndrome (1) 1(28),2(4) HME = hereditary multiple exostosis, N/A = not available, OC = osteochondroma Table 2 Risk Factors for Secondary Chondrosarcoma Clinical Pain Increasing size of a palpable lesion Male predominance (2:1) Location in the pelvis or hip Peak age in mid 30s Radiographic Surface irregularity Blurriness of the border Osteochondroma >5 cm Increase in size of osteochondroma Cartilage cap >2 cm Inhomogeneous mineralization of large cartilage cap Genetic Hereditary multiple exostosis (EXT1, EXT2, EXT3 mutations) Ollier disease Maffucci syndrome (5.5%) underwent dedifferentiation. 2 Most cases of dedifferentiated chondrosarcomas appear to originate from central primary chondrosarcoma; it is estimated that the fraction of primary chondrosarcomas undergoing dedifferentiation may be as high as 15.4%. 3 In most cases, the prognosis of patients with dedifferentiated chondrosarcoma is dismal, which is quite unlike the prognosis of patients with secondary chondrosarcoma. Secondary chondrosarcoma is a well-recognized clinical diagnosis. However, relatively little has been published on the subject, perhaps as a result of the rarity of the condition. In a review of the literature, we found only 10 studies pertaining directly to the subject that were not case reports (Table 1). Clinical Presentation The most frequent presenting complaints with secondary chondrosarcoma are a palpable mass and pain. In many cases, the signs and symptoms are subtle, and the complaints are not severe. Large masses in the pelvis and thigh are easily hidden from patient and clinician alike. New onset of pain in a preexisting osteochondroma should alert the physician to the possibility of enlargement of the cartilaginous cap. Rarely, some tumors in the pelvis may cause urinary and bowel irregularities. A male predilection for secondary chondrosarcoma seems to be evident. Based on combined data from the published series, 63% of patients with the disease are male and 37% are female, resulting in a male:female ratio of approximately 2:1. 4-13 The mean age of persons with secondary chondrosarcoma is 34 years, which is notably younger than the average age of persons with primary conventional chondrosarcoma. 4-13 The most common site of involvement is the pelvis, followed by the proximal femur. The scapula and proximal humerus are also relatively common sites. Clinical, radiographic, and genetic features associated with secondary chondrosarcomas are summarized in Table 2. Our review of the major published series indicates that the great majority of cases arise in osteochondromas (88%), with solitary osteochondroma being slightly more common than multiple osteochondromas (ie, hereditary multiple exostosis). 4-7,11,13 Secondary chondrosarcoma can also occur in persons with Ollier disease (ie, multiple enchondromas) and Maffucci syndrome (ie, multiple enchondromas associated with softtissue hemangiomas). Other benign cartilaginous lesions that have been reported, although rarely to result in secondary chondrosarcoma include solitary enchondroma, synovial chondromatosis, and chondromyxoid fibroma. 6,15-20 These lesions have been noted mostly in case reports, and there may be some question whether some cases were malignant at the outset. 6,15-20 October 2010, Vol 18, No 10 609
Secondary Chondrosarcoma Figure 1 Secondary chondrosarcoma arising from a pelvic osteochondroma. A, Preoperative AP pelvic radiograph demonstrating a large calcified tumor arising from the iliac wing. The surfaces of the calcified areas are indistinct and not well-delineated. B, Postoperative AP pelvic radiograph following wide excision of the tumor. C, Photograph of the cut specimen after resection demonstrating a thick layer of white, glistening hyaline cartilage. The central area shows a small area of ossified bone (arrow), which represents the stalk of the preexisting osteochondroma. D, T1-weighted axial magnetic resonance image demonstrating fatty marrow in the central portion of the sessile stalk emanating from the iliac crest, indicating the presence of an ossified central portion of the original osteochondroma. E, T2-weighted axial magnetic resonance image demonstrating the thick layer of cartilaginous tumor surrounding the central ossified stalk, which appears dark. F, Gadolinium-enhanced fat-saturated T1-weighted magnetic resonance image showing a subtle speckled pattern of enhancement within the tumor. Radiologic Features Conventional radiography may offer important clues regarding the diagnosis of secondary chondrosarcoma. Osteochondromas have well-defined bony edges, including the subchondral bone of the cartilaginous cap. In contrast, secondary chondrosarcomas demonstrate irregularity or blurriness of the surface of the osteochondroma (Figure 1). A soft-tissue mass outside the osseous portion of the osteochondroma may be subtly appreciable, and this mass may exhibit scattered foci of calcification. 4,5 CT and MRI are important in demonstrating the malignant features of the tumor. Most critically, they reveal an abnormally large cartilaginous cap on the osteochondroma (Figure 1, D through F). The portion of the osteochondroma that becomes malignant is the cartilaginous tissue that forms the cap of the lesion. The osseous tissue that makes up the base or stalk is not thought to 610 Journal of the American Academy of Orthopaedic Surgeons
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Secondary Chondrosarcoma

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