Clear-cell chondrosarcoma. A case report suggesting a malignant variation of chondroblastoma.

Komiya, Setsuro; Inoue, Akio; Nakashima, Masanori; Takagi, Hisao; Ikuta, H.; Irie, Kohji; Morimatsu, Minoru

doi:10.2739/kurumemedj.33.131

Cited by 4 publications

(1 citation statement)

References 13 publications

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“…[5]198547N/A9 × N/A2–323 (median 72)5 × N/A16; 34%567; 15%42–84 (median 48)5 × lung22 × NED23 × R03 × bone8 × AWD19 × R18 × DODKomiya et al. [31]198611 × LowDOD54R20–148Bones / lungOhno et al. [32]198611 × LowNED39R00–0––Present et al.…”

Section: Resultsmentioning

confidence: 99%

Clear cell chondrosarcoma is an underestimated tumor: Report of 7 cases and meta-analysis of the literature

Klein¹,

Tauscher²,

Birkenmaier³

et al. 2019

Journal of Bone Oncology

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IntroductionClear cell chondrosarcoma (CCC) is a rare subtype of chondrosarcoma and it is commonly considered a low-grade tumor and less aggressive than atypical cartilaginous tumor (grade 1 central chondrosarcoma). However, the experience even of musculoskeletal tumor centres with this rare entity is limited. The aim of this study is to analyse our own treatment results and those of the literature regarding the therapy and outcome of this lesion.Material and Methods7 cases of CCC have been treated in our department between 2003 and 2015. Their follow-up data were collected retrospectively. 187 literature cases with histopathological and clinical characteristics were retrieved by means of a PubMed search with the key word “clear cell chondrosarcoma”. The data pertaining to treatment and follow up were extracted. We analysed the survival of patient and the risk factors for local recurrence (LR) as well as metastatic disease (MD).ResultsThe mean age at the time of diagnosis was 40 years. Two thirds of the patients were male. The mean follow-up time was 109 months. To our surprise, there was a high rate of LR (30%) and of MD (20%) when compared to low-grade conventional chondrosarcomas. 15% of LR and 20% of metastatic disease were observed after more than 10 years follow-up. Uncommon locations of MD such as in the spine is a unique observation in chondrosarcomas and underlines the high aggressiveness of this tumor. 10-year overall survival was almost 80%, 10-years disease free survival 60%. Positive margins (p = 0.038) and metastases (p = 0.006) impaired the overall survival significantly. The rate of local recurrence was significantly dependent on resection margin (p < 0.001); however there was no correlation with the grade of differentiation of the tumor. The development of MD was affected by local recurrence (p = 0.006), but we could not detect a significant association with margin status (p = 0.184).ConclusionsA wide resection is the advocated treatment option. Long term follow-up for at least 10 years is necessary in order to not overlook late LR or MD. This work demonstrates for the first time the apparent aggressiveness of the CCC.

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Section: Resultsmentioning

confidence: 99%

Clear cell chondrosarcoma is an underestimated tumor: Report of 7 cases and meta-analysis of the literature

Klein¹,

Tauscher²,

Birkenmaier³

et al. 2019

Journal of Bone Oncology

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Clear cell chondrosarcoma of the pelvis in a skeletally immature patient

et al. 1999

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We report on a case of clear cell chondrosarcoma (CCCS) of the left iliac bone in a 12-year-old skeletally immature boy. Radiographic examination revealed an aggressive osteolytic lesion with areas of mineralization. Fluid-fluid levels were seen on T2-weighted MR images. Laboratory data showed slight elevation of serum alkaline phosphatase. The biopsy specimen showed histological features of CCCS with some resemblance to osteosarcoma, such as prominent irregular osteoid formation among clear tumor cells. Surgical treatment was accomplished without pre- or post-operative chemotherapy. Because of the patient's age, elevated serum alkaline phosphatase, and histopathology with prominent osteoid production, this case could be confused with osteosarcoma. Although CCCS is an extremely rare bone tumor in children, it is important to be aware that it may arise in a skeletally immature patient. CCCS, unlike osteosarcoma, is not treated with neo-adjuvant chemotherapy.

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