Management of Incidental High-grade Dedifferentiated Chondrosarcoma by Triple Vertebrectomy and Anterior Reconstruction Using a Posterior-only Approach

Introduction. Chondrosarcomas are malignant bone tumors that are characterized by the production of chondroid tissue. Since radiation therapy and chemotherapy have limited effect on chondrosarcoma, treatment of most patients depends on surgical resection. We conducted this study to identify independent predictive factors and survival characteristics for conventional central chondrosarcoma and dedifferentiated central chondrosarcoma.Methods. A systematic literature review was performed in September 2014 using the Pubmed, Embase, and Cochrane databases. Subsequent to a beforehand-composed selection procedure we included 13 studies, comprising a total of 1114 patients.Results. The prognosis of central chondrosarcoma is generally good for the histologically low-grade tumors. Prognosis for the high-grade chondrosarcoma and the dedifferentiated chondrosarcoma is poor with lower survival rates. Poor prognostic factors in conventional chondrosarcoma for overall survival are high-grade tumors and axial/pelvic tumor location. In dedifferentiated chondrosarcoma the percentage of dedifferentiated component has significant influence on disease-free survival.Conclusion. Despite the fact that there are multiple prognostic factors identified, as shown in this study, there is a need for prospective and comparative studies. The resulting knowledge about prognostic factors and survival can give direction in the development of better therapies. This could eventually lead to an evidence-based foundation for treating chondrosarcoma patients.

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Development and external validation of nomograms to predict sarcoma-specific death and disease progression after surgical resection of localized high-grade conventional primary central chondrosarcoma and dedifferentiated chondrosarcoma

The Bone & Joint Journal ◽

10.1302/0301-620x.102b12.bjj-2020-0810.r1 ◽

2020 ◽

Vol 102-B (12) ◽

pp. 1752-1759

Author(s):

Yusuke Tsuda ◽

Kim Tsoi ◽

Jonathan D. Stevenson ◽

Minna Laitinen ◽

Peter C. Ferguson ◽

...

Keyword(s):

Disease Progression ◽

Histological Grade ◽

External Validation ◽

Surgical Margin ◽

Calibration Plot ◽

High Grade ◽

Dedifferentiated Chondrosarcoma ◽

Study Population ◽

Grade Ii ◽

Definitive Surgery

Aims Our aim was to develop and validate nomograms that would predict the cumulative incidence of sarcoma-specific death (CISSD) and disease progression (CIDP) in patients with localized high-grade primary central and dedifferentiated chondrosarcoma. Methods The study population consisted of 391 patients from two international sarcoma centres (development cohort) who had undergone definitive surgery for a localized high-grade (histological grade II or III) conventional primary central chondrosarcoma or dedifferentiated chondrosarcoma. Disease progression captured the first event of either metastasis or local recurrence. An independent cohort of 221 patients from three additional hospitals was used for external validation. Two nomograms were internally and externally validated for discrimination (c-index) and calibration plot. Results In the development cohort, the CISSD at ten years was 32.9% (95% confidence interval (CI) 19.8% to 38.4%). Age at diagnosis, grade, and surgical margin were found to have significant effects on CISSD and CIDP in multivariate analyses. Maximum tumour diameter was also significantly associated with CISSD. In the development cohort, the c-indices for CISSD and CIDP at five years were 0.743 (95% CI 0.700 to 0.819) and 0.761 (95% CI 0.713 to 0.800), respectively. When applied to the validation cohort, the c-indices for CISSD and CIDP at five years were 0.839 (95% CI 0.763 to 0.916) and 0.749 (95% CI 0.672 to 0.825), respectively. The calibration plots for these two nomograms demonstrated good fit. Conclusion Our nomograms performed well on internal and external validation and can be used to predict CISSD and CIDP after resection of localized high-grade conventional primary central and dedifferentiated chondrosarcomas. They provide a new tool with which clinicians can assess and advise individual patients about their prognosis. Cite this article: Bone Joint J 2020;102-B(12):1752–1759.

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Dedifferentiated Chondrosarcoma with a High-Grade Mesenchymal Component Mimicking a Gastrointestinal Stromal Tumor

Open Journal of Pathology ◽

10.4236/ojpathology.2012.23017 ◽

2012 ◽

Vol 02 (03) ◽

pp. 90-95

Author(s):

Toshihiro Akisue ◽

Kenta Kishimoto ◽

Teruya Kawamoto ◽

Hitomi Hara ◽

Masahiro Kurosaka

Keyword(s):

Gastrointestinal Stromal Tumor ◽

Stromal Tumor ◽

High Grade ◽

Dedifferentiated Chondrosarcoma

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Targeted Next-Generation Sequencing Identifies Molecular and Genetic Events in Dedifferentiated Chondrosarcoma

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2020-0379-oa ◽

2020 ◽

Author(s):

Calixto-Hope G. Lucas ◽

James P. Grenert ◽

Andrew Horvai

Keyword(s):

Dna Sequencing ◽

Copy Number ◽

High Grade ◽

Copy Number Alterations ◽

Next Generation ◽

Dedifferentiated Chondrosarcoma ◽

Tert Promoter ◽

Pathologic Features ◽

Tert Promoter Mutations ◽

Promoter Mutations

Context.— Dedifferentiated chondrosarcoma is a rare adult bone tumor with a dismal prognosis and is composed of a conventional chondrosarcoma juxtaposed to high-grade nonchondrogenic sarcoma. Dedifferentiated chondrosarcomas may represent tumor progression from a differentiated to a primitive histotype. Objective.— To determine the genetic and molecular events that drive progression from a conventional chondrosarcoma to high grade nonchondrogenic sarcoma. Design.— We analyzed the genomic landscape of paired conventional and dedifferentiated components of 11 dedifferentiated chondrosarcoma using targeted next-generation DNA sequencing with immunohistochemical validation. Clinical, radiographic, and pathologic features of tumors were reviewed. Capture-based DNA sequencing targeting the coding regions of 479 cancer genes and select introns was performed. Results.— The tumors arose in the femur (n = 4; 36%), scapula (n = 3; 27%), pelvis (n = 3; 27%), and humerus (n = 1; 9%) of 7 men (64%) and 4 women (36%; median age, 61 years). DNA was adequate for sequencing from all 11 dedifferentiated components (100%) and 9 paired conventional chondrosarcoma components (82%). All tumors (100%) harbored either IDH1 p.R132 or IDH2 p.R172S hotspot mutations. Seven tumors (64%) displayed COL2A1 alterations. TERT promoter mutations were present in 5 of 9 pairs (56%) and 2 (22%) additional unpaired dedifferentiated components. IDH1/2, COL2A1, and TERT mutations were identical in both components of the paired samples. Pathogenic missense or truncating mutations in TP53 and large-scale copy number alterations were more common in dedifferentiated components than in those of matched conventional components. Conclusions.— The results support IDH1/2, COL2A1, and TERT promoter mutations being common in dedifferentiated chondrosarcoma and as likely early events in progression, whereas inactivating mutation of TP53 and high-level copy number alterations may be later events in the dedifferentiated phenotype.

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Dedifferentiated Chondrosarcoma with Epithelial Differentiation: A Case Report and Review of the Literature

Journal of Surgical Oncology ◽

10.31487/j.jso.2020.02.08 ◽

2020 ◽

pp. 1-4

Author(s):

Humaid Al Farii ◽

Marc-Antoine Ricard ◽

Motaz AlAqeel ◽

Robert Turcotte ◽

...

Keyword(s):

Case Report ◽

Giant Cell Tumor ◽

Fibrous Histiocytoma ◽

Cell Tumor ◽

High Grade ◽

Review Of The Literature ◽

Dedifferentiated Chondrosarcoma ◽

Well Differentiated ◽

Definition Of ◽

Rare Instance

Case: We describe a case of 72-year-old male with dedifferentiated chondrosarcoma with osteosarcomatous and epithelioid (squamous) dedifferentiation. As first described in the literature by Dahlin and Beabout, a well-differentiated benign chondral lesion abruptly juxtaposed to a high-grade non-cartilaginous component is the classical definition of a chondrosarcoma [1]. The non-cartilaginous component is typically an osteosarcoma, fibrosarcoma or malignant fibrous histiocytoma with rare instances of leiomyosarcoma, giant cell tumor and rhabdomyosarcoma [2]. Conclusion: We report our rare instance of squamous dedifferentiation of a dedifferentiated chondrosarcoma along with other five cases that have been reported so far in the literature

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A European treatment protocol for bone sarcoma in patients older than 40 years

Journal of Clinical Oncology ◽

10.1200/jco.2009.27.15_suppl.10516 ◽

2009 ◽

Vol 27 (15_suppl) ◽

pp. 10516-10516 ◽

Cited By ~ 1

Author(s):

S. Ferrari ◽

S. Smeland ◽

S. Bielack ◽

A. Comandone ◽

P. Dileo ◽

...

Keyword(s):

Complete Remission ◽

Treatment Protocol ◽

International Study ◽

Bone Sarcoma ◽

Poor Responders ◽

High Grade ◽

Dedifferentiated Chondrosarcoma ◽

Peripheral Neurotoxicity ◽

Central Location ◽

Synchronous Metastases

10516 Background: EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S.) is the first prospective multicenter international study for patients 41 - 65 years old with high-grade bone sarcoma. Methods: Patients with HG Osteosarcoma (OS), HG sarcoma NOS (S), Fibrosarcoma, MFH, Leiomyosarcoma, Dedifferentiated Chondrosarcoma (DCh) were included. Chemotherapy: Combinations of cisplatin/doxorubicin (CDP 100mg/m2/ADM 60mg/m2), ifosfamide/CDP(IFO 6g/m2/CDP 100mg/m2) and IFO/ADM (IFO 6g/m2/ADM 60mg/m2) were repeated three times (9 cycles). Surgery was planned after 3 cycles. Methotrexate (8g/m2) was postoperatively added in poor responders. Immediate surgery was allowed and 9 cycles with CDP, ADM, IFO were postoperatively given. Results: In December 2007, 140 patients were registered (median age 51 years). OS (51%), S (16%), and DCh (11%) were the more frequent histotypes. Synchronous metastases in 30 (21%) patients, central location of tumor in 45 (32%). Surgical complete remission (SCR) was achieved in 84% of patients, (localized 91%, metastatic 37%) without difference among the histology groups. One surgical-related and one chemotherapy-related death were reported. Grade 4 WBC and PLT incidence was 55% and 17%.Renal toxicity and peripheral neurotoxicity were reported in 16% and 20% of patients. With a median follow-up of 25 months (4–68) 3 year OS was 58% (95%CI 48–68%) [7% (95%CI 0–19%) without SCR]. In patients with SCR, 3 year OS and EFS were 46% (95%CI 9–83%) and 0% in case of synchronous metastases and 69% (95%CI58–80%) and 45% (95%CI33–57%) for localized patients; 50% (95%CI 29–71%) and 40% (95%CI 20–59%) for patients with central tumor, 73% (95%CI61–85%) and 44% (95%CI31–57%) for those with extremity tumor; 68% (95%CI 52–83%) and 46% (95%CI 32–54%) for OS, 64% (95%CI 42–85%) and 48% (95%CI 25–71%) for S, 48% (95%CI 13–82%) and 27% (95%CI 1–54%) for DCh. Conclusions: The protocol is feasible, but the chemotherapy-related toxicity is remarkable. Surgical complete remission is the main factor influencing survival. Central location and synchronous metastases are negative prognostic factors, but 50% 3-year OS can be achieved with aggressive local and systemic treatment. Osteosarcoma and high-grade sarcoma NOS benefit from chemotherapy more than patients with dedifferentiated chondrosarcoma. No significant financial relationships to disclose.

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