Das Klarzellchondrosarkom - klinischpathologischer Bericht eines weiteren Falles

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.

show abstract

“…[35]198911 × LowNED70R11640––Welkerling et al. [36]199011 × LowNED22R00–0––Sreekantaiah et al. [37]199111 × LowNED11R00–0––Bosse 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

View full text Add to dashboard Cite

show abstract

Skelettsystem

Delling¹

1997

Pathologie 5

View full text Add to dashboard Cite

Morphological typing of chondrosarcoma: A study of 94 cases

Welkerling

Dreyer

Delling

1991

Vichows Archiv A Pathol Anat

View full text Add to dashboard Cite

Ninety-four chondrosarcomas of the Hamburg Bone Tumour Registry were reviewed in a retrospective study. The purpose of this study was to examine the morphological characteristics of different types of chondrosarcomas and to describe distinctive features of location, the age distribution and the male to female ratio. Central chondrosarcomas can be divided into classical chondrosarcomas, dedifferentiated chondrosarcomas, mesenchymal chondrosarcomas and clear-cell chondrosarcomas. Five periosteal chondrosarcomas were represented. Classical chondrosarcomas and clear-cell chondrosarcomas show a significant predominance of males; no sex predilection was seen in dedifferentiated and mesenchymal chondrosarcomas. Nearly 60% of classical and mesenchymal chondrosarcomas occur in the trunk. Eighty-five percent of dedifferentiated chondrosarcomas are located in the long bones of the limbs. Clear-cell chondrosarcomas arise in the proximal part of the femur. There is a marked predilection for mesenchymal chondrosarcomas in the second and third decades of life. The average age of patients with classical chondrosarcomas was 54 years, but clear-cell chondrosarcomas occur 10 years earlier and dedifferentiated chondrosarcomas 10 years later. Characteristically, classical chondrosarcomas produce a pure chondroid matrix with variable differentiation of tumour chondrocytes. The most important histological feature of the defifferentiated chondrosarcoma is the close association of two different cellular components. One of these consists of cartilage, which is generally well differentiated. In most of our cases the second component showed features of osteosarcoma (50%). Mesenchymal chondrosarcoma is characterized by concentric infiltration of cartilage islands by small tumour cells. Clear-cell chondrosarcomas show regions of cartilaginous tumour and areas of closely packed, glycogen-rich, large tumour cells with distinct boundaries. Osteoid formation and multinucleated giant cells are present in clear-cell areas. Knowledge of this group of tumours is indispensable for correct histological diagnosis and typing and is important in the design of surgical therapy and the prediction of biological behaviour.

show abstract

Das Klarzellchondrosarkom - klinischpathologischer Bericht eines weiteren Falles

Cited by 6 publications

References 0 publications

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

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

Skelettsystem

Morphological typing of chondrosarcoma: A study of 94 cases

Contact Info

Product

Resources

About