Dedifferentiated chondrsarcoma: a clinicopathologic analysis of 25 cases

Abstract

Background To investigate the clinical, imaging and pathological features of dedifferentiated chondrosarcoma for better diagnosis. Methods Patients who had been confirmed to have dedifferentiated chondrosarcoma were enrolled in this study and analyzed in the clinical, imaging and pathological data. Results Twenty-five patients had pathologically confirmed dedifferentiated chondrosarcoma including 15 males and 10 females with an age range of 24–74 (median 58, interquartile range 49–65). Ten patients had the tumor at the femur, four at the ilium, two at the humerus, two at the tibia, two at cotyle, and one at each of the following locations: scapula, sacrum, rib, pubic branch, and calcaneus. Twenty-one patients had local pain and a soft tissue mass while the other four patients had only local pain without a soft tissue mass. Four patients had pathological fractures. Imaging showed extensive bone destruction with calcification inside the lesion and possible pathological fractures. On gross observation of the specimen, the chondrosarcoma components were usually located inside the bone, and the dedifferentiated sarcoma components were mainly located outside the bone. Microscopy showed the dedifferentiated tumor had two components: well-differentiated chondrosarcoma and poorly differentiated non-chondral sarcoma including malignant fibrous histiocytoma in eleven cases, osteosarcoma in ten cases, fibrosarcoma in two, liomyosarcoma in one, and lipoblastoma in the remaining one.. Followed up from 3 moths to 60 months (mean 15.6), eight patients died with a survival time of 10–23 months (mean 16), and the other 17 patients survived with the survival duration from three to 60 months (15). Conclusion Dedifferentiated chondrosarcoma is a fatal disease with multiple components, and most of the cases have dual morphological and imaging features of chondrosarcoma and non-chondrosarcoma. The imaging presentations are primarily of common central chondrosarcoma, combined with cortical destruction, soft tissue mass, and pathological fractures.

Small cell carcinoma of esophagus: Case report

“…Dedifferentiated chondrosarcoma is a rare highly aggressive tumor which manifests in 6 t h to 7 t h decade of life with no gender predilection. [1][2][3] However, when the chondrosarcoma is secondary to an enchondroma, the presentation is earlier, as seen in this case. 9 Pain, swelling, increase in size of an enchondroma or a recurrence after excision should alert the clinician to look out for a malignant transformation.…”

“…Other components which can be seen are malignant fibrous histiocytoma, leiomyosarcoma, angiosarcoma, rhabdomyosarcoma, lipoblastoma and very rarely an epithelioid sarcoma. 1,9 The common sites involved by DDCS are femur and pelvis while humerus, ribs, spine tibia and upper extremities are rarer sites. 4 In a dedifferentiated chondrosarcoma, the cartilaginous component is usually seen within the bone and the dedifferentiated component exists outside in the soft tissue.…”

“…CT scan helps to appreciate fine calcification in the lesion, while MRI helps to assess the spread of tumor which is very essential to plan a surgery to get clean resection margins. 1,6 Hence both these modalities should be employed for the radiological work up. Preferably, the biopsy should be image guided and multipoint to ascertain that different tumor areas are sampled.…”

“…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.…”

Clinical features and treatment outcomes of dedifferentiated and grade 3 chondrosarcoma: A multi‐institutional study

“…Liu et al 32 reported the prognosis in 23 patients with DDCS. Cao et al 39 investigated the clinical, imaging, and pathological features in 25 cases of DDCS. Gong et al 40 analyzed the clinical, histopathological, and immunohistochemical features of 57 cases of DDCS.…”