Dedifferentiated chondrosarcoma

We reviewed 74 cases of dedifferentiated central and peripheral chondrosarcoma. Histologically these tumours consist of an underlying cartilaginous component (either benign or malignant) juxtaposed to a highgrade non-cartilaginous component, with a typically abrupt transition between the two tissue types. The noncartilaginous component may constitute a very small or a very large proportion of the tumour, so diagnosis often requires histological evaluation of the entire tumour. The diagnosis is often suspected on the basis of the clinical course and careful evaluation of the radiographie characteristics. Central dedifferentiated chondrosarcoma can be classified radiographically into three types. In type 1 (36 cases in our review) the radiographie features are the same as those of a central chondrosarcoma, with the addition of a region with very aggressive radiographie features. Type 2 lesions (20 cases) resemble the underlying benign enchondroma but also have destructive changes and/or a large soft tissue mass. Type 3 lesions (8 cases) are not distinctive radiographically and present as a very high grade destructive lesion of bone. These cases are diagnosed following biopsy or tumour resection. The prognosis of these tumours is extremely poor, with 13% overall 5-year survival in this series. Improved survival was found in those cases where diagnosis was prompt and surgical treatment with a wide or radical margin was attained. No benefit was found from the use of adjuvant chemotherapy or radiotherapy. Thus, early recognition of the characteristic radiographie features, adequate histological sampling, and wide or radical surgical margins are necessary for satisfactory management of this highly malignant variant of chondrosarcoma.     

Chondrosarcoma as a complicating factor in Paget’s disease of bone

 A 65-year-old male patient with an 8-year history of poliostotic Paget’s disease complained of shoulder pain that started 6 months prior to admission. An extensive lytic area was identified in the right proximal humerus along with Paget’s disease. There was cortical destruction and a soft tissue mass. Following an incisional biopsy, a diagnosis of grade 2 chondrosarcoma associated with Paget’s disease was made. The histologic identification of chondrosarcoma associated with Paget’s disease is rare. However, the presence of a calcified matrix in a destructive lesion associated with Paget’s disease should alert the radiologist and the pathologist to the possibility of a chondromatous differentiation taking place in the sarcoma associated with Paget’s disease. The histologic evaluation of the lesion will form the basis for the diagnosis.     

Endolaryngeal resection of laryngeal chondrosarcoma

Chondrosarcoma is a rare tumor. In this study, we present a case with laryngeal chondrosarcoma that was treated by an endolaryngeal approach using an operation microscope. The patient remains disease-free to date, with no evidence of new or recurrent disease more than 3 years after the endolaryngeal surgery. The computerized tomography and endoscopic examinations did not reveal any recurrent disease, indicating the success of the endolaryngeal approach.     

Chondrosarcoma of the nasal septum

Chondrosarcoma of the head and neck region are relatively uncommon, arising rarely in the naval septum. The reported cases of nasal septal chondrosarcomas are extensive lesions with involvement of paranasal sinuses, orbit or skull base at the lime of diagnosis. Those limited to the nasal cavity is extremely rare and to date there has been one case report in English language literature. We present a case of chondrosarcoma of the nasal septum with involvement of the nasal cavity alone and no evidence of bony erosion. Initial multiple biopsies showed mature chondromatous areas with no atypia. The patient had wide excision of the tumour. The final biopsy of the excised specimen revealed foci of well-differentiated chondrosarcoma. Wide surgical excision with adequate margins should be considered as the treatment of choice in lesion of nasal septum even if initial biopsies are negative for malignancy. Hence this case report.     

Femur chondrosarcoma misdiagnosed as acute knee arthritis and osteomyelitis—Further developing a hitherto unreported complication of tumor embolic ischemic ileal perforation after arthroscopic lavage

The differentiation between osteomyelitis and bone tumor may be difficult due to their overlapping clinical and radiological features. A 25-year-old lady presented with left knee pain and joint effusion associated with redness and hotness. A sub-optimally taken plain radiograph showed mixed osteolytic and osteoblastic lesion in the left lower femur with surrounding soft tissue swelling. Since the clinical diagnosis was acute osteomyelitis and arthritis, arthroscopic lavage was performed as a diagnostic and therapeutic procedure. The removed loose bodies and fibrinous tissue showed pathological features suspicious of chondrosarcoma. Subsequent MRI revealed an infiltrative tumor eroding through the cortex and joint cartilage. En bloc excision of the left lower femur, upper tibia including the knee joint and patella was performed, and the final diagnosis was grade 2 chondrosarcoma. The patient developed bilateral pulmonary metastasis 33 months after operation. Five months later, she suffered from a hitherto undescribed complication of ischemic perforation of the terminal ileum secondary to tumor embolic arterial obstruction with no macroscopic intestinal or peritoneal tumor deposit. The patient developed multiple brain metastases and died 43 months after initial presentation.Our case illustrates that malignant bone tumor as a differential diagnosis of acute osteomyelitis and arthritis merits recognition and exclusion before arthroscopic lavage, which may enhance tumor dissemination and in our patient results in embolic ischemic ileal perforation.     

Distinctive Head and Neck Bone and Soft Tissue Neoplasms

Benign and malignant primary bone and soft tissue lesions of the head and neck are rare. The uncommon nature of these tumors, combined with the complex anatomy of the head and neck, pose diagnostic challenges to pathologists. This article describes the pertinent clinical, radiographic, and pathologic features of selected bone and soft tissue tumors involving the head and neck region, including angiofibroma, glomangiopericytoma, rhabdomyosarcoma, biphenotypic sinonasal sarcoma, chordoma, chondrosarcoma, and osteosarcoma. Emphasis is placed on key diagnostic pitfalls, differential diagnosis, and the importance of correlating clinical and radiographic information, particularly for tumors involving bone.     

对比分析颅底软骨肉瘤与脊索瘤CT和MRI征象

目的 对比分析颅底软骨肉瘤与脊索瘤CT和MRI征象。方法 回顾性分析经病理证实的7例颅底软骨肉瘤和18例脊索瘤患者的CT和MRI资料。结果 颅底软骨肉瘤与脊索瘤好发部位相似,以斜坡中线区多见,少数位于前、后颅窝底;其CT和MRI有共性征象：均伴颅底膨胀性或溶骨性骨质破坏,T2WI以明显高信号为主,增强后多呈"花瓣样"或"蜂房样"明显不均匀强化。5例软骨肉瘤可见明显粗大钙化,密度较高且边缘锐利,4例DWI呈低信号;16例脊索瘤无明显钙化或见细小条状残留骨嵴,仅2例DWI呈低信号。结论 颅底软骨肉瘤与脊索瘤的CT和MRI征象有一定共性,肿瘤内钙化特点及DWI信号强度对鉴别诊断有一定价值。