Outcome of advanced, unresectable conventional central chondrosarcoma

Maldegem, Annemiek M. van; Gelderblom, Hans; Palmerini, Emanuela; Dijkstra, Sander; Gambarotti, Marco; Ruggieri, Pietro; Nout, Remi A.; Sande, Michiel A. J. van de; Ferrari, Cristina; Ferrari, Stefano; Bovée, Judith V.M.G.; Picci, Piero

doi:10.1002/cncr.28845

Cited by 89 publications

(89 citation statements)

References 12 publications

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“…Treatment is mainly based on surgery and chemotherapy is less effective because of a low mitotic index and poor vascularity [4, 5]. Radiotherapy is effective but requires substantial dosage [6].…”

Section: Introductionmentioning

confidence: 99%

Survival and prognostic factors in conventional central chondrosarcoma

Fromm¹,

Klein²,

Baur‐Melnyk

et al. 2018

BMC Cancer

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BackgroundChondrosarcoma is the second most frequent primary malignant bone tumor. Treatment is mainly based on surgery. In general, wide resection is advocated at least in G2 and G3 tumors. But which margins should be achieved? Does localization as for example in the pelvis have a higher impact on survival than surgical margins themselves?MethodsFrom 1982 to 2014, 87 consecutive patients were treated by resection. The margin was defined as R0 (wide resection), R1 (marginal resection) or, R2 if the tumor was left intentionally. All patients were followed for evidence of local recurrence or distant metastasis. Overall and recurrence-free survival were calculated, significance analysis was performed.ResultsIn 54 (62%) cases a R0 resection, in 31 (36%) a R1 and in 2 (2%) patients a R2-resection was achieved. Histology proved to be G1 in 37 patients (43%), G2 in 41 (47%) and G3 in 9 cases (10%). 5-year local recurrence-free survival (LRFS) was 75%. Local recurrence-free survival showed a significant association with the margin status and the localization of the tumor with pelvic lesions doing worst. Metastatic disease was initially seen in 4 patients (4.6%), 19 others developed metastatic disease during follow-up. Overall survival of the entire group at 5 and 10 years were 79 and 75%, respectively. The quality of surgical margins and the presence of local recurrence did not influence overall survival in a multivariate analysis. Pelvic lesions had a worse prognosis as did higher grades of the tumor, metastatic disease and age.ConclusionsThe mainstay of therapy in Chondrosarcoma remains surgery. Risk factors as grading, metastatic disease, age and location significantly influence overall survival. Margin status (R0 vs. R1) did influence local recurrence-free survival but not overall survival. Chondrosarcomas of the pelvis have a higher risk of local recurrence and should be treated more aggressively.

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Section: Introductionmentioning

confidence: 99%

Survival and prognostic factors in conventional central chondrosarcoma

Fromm¹,

Klein²,

Baur‐Melnyk

et al. 2018

BMC Cancer

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“…For instance, the anti-apoptotic proteins Bcl-2 and Bcl-XL are highly expressed in all CS subtypes and the BH-3 mimetic ABT-737 renders CS cell lines sensitive to the conventional chemotherapeutic agents doxorubicin and cisplatin [10,40]. Survivin, a member of the inhibitor of apoptosis protein family, is expressed in CS samples and RNA interference targeted on survivin results in cell cycle arrest and increased apoptotic rates in CS cell lines [42].…”

Section: Discussionmentioning

confidence: 99%

Comprehensive analysis of published studies involving systemic treatment for chondrosarcoma of bone between 2000 and 2013

2014

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BackgroundThe majority of patients with chondrosarcoma of bone have an excellent overall survival after local therapy. However, in case of unresectable locally advanced or metastatic disease the outcome is poor and limited treatment options exist. Therefore we conducted a survey of clinical phase I or II trials and retrospective studies that described systemic therapy for chondrosarcoma patients.Materials and methodsUsing PubMed, clinicaltrials.gov, the Cochrane controlled trial register and American Society of Clinical Oncology (ASCO) abstracts a literature survey was conducted. From the identified items, data were collected by a systematic analysis. We limited our search to semi-recent studies published between 2000 and 2013 to include modern drugs, imaging techniques and disease evaluations.ResultsA total of 31 studies were found which met the criteria: 9 phase I trials, 11 phase II and 8 retrospective studies. In these studies 855 chondrosarcoma patients were reported. The tested drugs were mostly non-cytotoxic, either alone or in combination with another non-cytotoxic agent or chemotherapy. Currently two phase I trials, one phase IB/II trial and three phase II trials are enrolling chondrosarcoma patients.ConclusionBecause chondrosarcoma of bone is an orphan disease it is difficult to conduct clinical trials. The meagre outcome data for locally advanced or metastatic patients indicate that new treatment options are needed. For the phase I trials it is difficult to draw conclusions because of the low numbers of chondrosarcoma patients enrolled, and at different dose levels. Some phase II trials show promising results which support further research. Retrospective studies are encouraged as they could add to the limited data available. Efforts to increase the number of studies for this orphan disease are urgently needed.

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“…However, in the latter case, they represent a therapeutic challenge because of their locally aggressive behaviour, while the metastatic risk is low. 5 Histological subtype and grade influence the prognosis and the choice of treatment.…”

Section: Chondrosarcomamentioning

confidence: 99%

Systemic therapy for selected skull base sarcomas: Chondrosarcoma, chordoma, giant cell tumour and solitary fibrous tumour/hemangiopericytoma

Colia

Provenzano

Hindi

et al. 2016

Reports of Practical Oncology & Radiotherapy

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IntroductionSkull base tumours include a large number of benign and malignant entities. This review focuses on chondrosarcoma, chordoma, giant cell tumour of the bone (GTCB) and solitary fibrous tumour/hemangiopericytoma (SFT), i.e., those sarcomas which are relatively "typical" of the skull base. Each represents a very rare disease, with an incidence of less than 1/1,000,000/year (considering all primary sites). 1,2 Of course, * Corresponding author. Tel.: +39 0223902803; fax: +39 0223902404.E-mail address: silvia.stacchiotti@istitutotumori.mi.it (S. Stacchiotti).all sarcoma subtypes can occasionally arise from the skull base.In principle, the essential criteria for medical treatment of sarcomas arising from the skull base are basically independent from the primary site. On the other hand, as a general rule, given their rarity and heterogeneity, sarcoma patients should always be approached by a multidisciplinary team at a referral institution. 3 Chondrosarcoma, chordoma and GTCB are all bone sarcomas that can arise from the bone component of the skull base, http://dx

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Outcome of advanced, unresectable conventional central chondrosarcoma

Cited by 89 publications

References 12 publications

Survival and prognostic factors in conventional central chondrosarcoma

Survival and prognostic factors in conventional central chondrosarcoma

Comprehensive analysis of published studies involving systemic treatment for chondrosarcoma of bone between 2000 and 2013

Systemic therapy for selected skull base sarcomas: Chondrosarcoma, chordoma, giant cell tumour and solitary fibrous tumour/hemangiopericytoma

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