Prognostic Factors in Dedifferentiated Chondrosarcoma: A Retrospective Analysis of a Large Series Treated at a Single Institution

Abstract

Background. Dedifferentiated chondrosarcomas (DDCSs) are highly malignant tumors with a dismal prognosis and present a significant challenge in clinical management. Methods. In an IRB approved retrospective protocol, we identified 72 patients with DDCS treated at our institution between 1993 and 2017 and reviewed clinicopathological characteristics, treatment modalities, and outcomes to analyze prognostic factors. Results. Femur (44.4%), pelvis (22.2%), and humerus (12.5%) were most commonly involved sites. Twenty-three patients (31.9%) presented with distant metastasis, and 3 (4.2%) of them also had regional lymph node involvement. The median overall survival (OS) was 13.9 months. On multivariate analysis, pathological fracture, larger tumor size, lymph node involvement, metastasis at diagnosis, extraosseous extension, and undifferentiated pleomorphic sarcoma component correlated with worse OS, whereas surgical resection and chemotherapy were associated with improved OS. For progression-free survival (PFS), pathological fracture and metastasis at diagnosis showed increased risk, while chemotherapy was associated with decreased risk. Among patients who received chemotherapy, doxorubicin and cisplatin were significantly associated with improved PFS but not OS. Among patients without metastasis at diagnosis, 17 (34.7%) developed local recurrence. Thirty-one (63.3%) developed distant metastases at a median interval of 18.1 months. On multivariate analysis, R1/R2 resection was related with local recurrence, while macroscopic dedifferentiated component was associated with distant metastasis. Conclusions. The prognosis of DDCS is poor. Complete resection remains a significant prognostic factor for local control. Chemotherapy with doxorubicin and cisplatin seems to have better PFS. More prognostic, multicenter trials are warranted to further explore the effectiveness of chemotherapy in selected DDCS patients.

Small cell carcinoma of esophagus: Case report

“…In most of the cases, a chondrosarcoma arising from an enchondroma is of grade I. 4,9 The present case however had a high grade cartilaginous component along with dedifferentiation into fibrosarcoma and osteosarcoma. Other components which can be seen are malignant fibrous histiocytoma, leiomyosarcoma, angiosarcoma, rhabdomyosarcoma, lipoblastoma and very rarely an epithelioid sarcoma.…”

“…Presence of dedifferentiated component in a chondrosarcoma is associated with high incidence of metastasis and a poor overall survival rate. [1][2][3][4][5] Characterized by a sharp juxtaposition of a cartilaginous component with a high grade sarcomatous area, accurate pre-operative diagnosis of dedifferentiated chondrosarcoma(DDCS) requires a careful radiological assessment followed by a multipoint biopsy. [6][7][8][9] We present a case of secondary chondrosarcoma of humerus in a middle aged man with dedifferentiation into fibrosarcoma and osteosarcoma which was surgically resected but had a fatal outcome.…”

Inhibiting Monocyte Recruitment to Prevent the Pro-Tumoral Activity of Tumor-Associated Macrophages in Chondrosarcoma

“…Patients diagnosed with chondrosarcomas (CHS) are subjected to large tumor resections to prevent local recurrences or metastasis, including amputations, with physical disabilities that highly affect daily life. Despite several preclinical studies and clinical trials aimed to identify druggable targets that may improve the prognosis of CHS patients, this bone sarcoma remains an orphan disease, mainly because all studies are performed with small numbers of patients [2][3][4]. Furthermore, no effective treatments exist for advanced CHSs due to their resistance to radiotherapy and chemotherapy.…”

“…Conventional CHS is the most frequent subtype found in 85% of the cases, with a survival rate depending on the histological grade. Among the other less common CHS variants, the dedifferentiated chondrosarcomas (DD-CHS) exhibit a poor prognosis and represent a significant challenge in clinical management, mostly due the co-existence of a high-grade not cartilaginous sarcoma in the context of a low-grade chondrogenic component [3,4]. Despite the occurrence of genetic alterations which have been described in distinct CHS subtypes [5][6][7][8][9], no FDA approved targeted therapies are currently available for CHS [10].…”

Acral Dedifferentiated Chondrosarcoma: Report of a Case Arising in the Proximal Phalanx of the Fourth Finger

“…[2][3][4][5] Dedifferentiated CS (DDCS) is composed of 2 distinct histopathological components: a benign cartilaginous lesion and a low-grade CS abruptly separated from a high-grade nonchondrogenic sarcoma. This component is generally an undifferentiated pleomorphic sarcoma (UPS), a fibrosarcoma, or a high-grade osteosarcoma, 6,7 but dedifferentiation to leiomyosarcoma, angiosarcoma, or rhabdomyosarcoma has been described, albeit rarely. 8 DDCS is more frequently diagnosed between 50 and 60 years (median age 59 years) 9 and in about 75% of cases, it occurs in the appendicular skeleton, especially femur, humerus, and pelvis.…”