Articular invasion by malignant bone tumours around the knee is one of the most important criterions to determine prior surgery. MR imaging is the most accurate exam in staging bone sarcomas. Although, past studies showed that when MRI shows evidence of intra articular involvement by tumour, the incidence of false positive diagnosis and subsequent excessively radical surgery is as high as 50%. The aim of this study is to determine growth pattern of bone sarcomas into the joint in order to assess which are the limits of the joint compartment. We reviewed retrospectively 18 cases of primary intra medullary sarcomas with epiphyseal extension located around the knee. The tumour was located in the distal femur in 11 cases and in the proximal tibia in 7 cases. In tumours located in the distal femur, two distinct modes of extension towards synovium and joint space were identified. The most common pattern was tumour growth along the anterior and intra articular part of the distal femur. This pattern was observed in 10 cases. The tumour displaced anteriorly soft tissues and remained extra synovial in 6 cases. Only in 4 cases, tumour contaminated the joint space. The extension was in all cases marginally close to the cartilage of the trochlea in the transitional zone between cartilage and synovial membrane. The second pattern was extension through the inter condylar notch which was observed in three cases. Growth was around the osseous-tendinous junction of the cruciate ligaments and never within the ligament. In tumors in the proximal tibia, although tumour was close to the osteochondral junction, cartilage was not breached anyway. Tumour got around the cartilage. Extension of the tumour to the articular joint was marginally under the posterior capsule insertions making contact with the edge of the articular cartilage. This pattern was observed in two cases. We didn’t observe an erosion of cartilage layer, in the limits of the sections done. Our study, demonstrated that cartilage and synovial membrane, since they are not breached, represent reliable margins for intra articular resections. We identified in the current study, one mode of tumour extension towards synovium and joint space. In all cases, extension was in junctional zones between cartilage and synovial membrane or cartilage and articular capsule. The articular cartilage was the most resistant barrier, having no vascular perforations and probably an intrinsic resistance to tumour.
Adamantinoma is a primary low-grade, malignant bone tumor that is predominantly located in the mid-portion of the tibia. The aetiology of the tumor is still a matter of debate. Histologically, classic adamantinoma is a biphasic tumor characterized by epithelial and osteofibrous components that may be intermingled with each other in various proportions and differentiating patterns. We report here a particular morphologic variant of adamantinoma characterised by the loss of classic epithelial differentiation. A 17 year-old teenager presented with a long history of a tumor in the left leg. Explorations revealed an osteolytic lesion of the middle shaft of the left tibia with a huge mass invading soft tissues. An intra medullar bone lesion with benign appearance was observed in the right tibia. CT of the chest revealed lung metastases. Histologically, we observed in the left side a pattern of spinde-celled high grade sarcoma without any evident differentiation. Spindle-shaped tumor cells express cytokeratins and vimentin. Ultrasturctural study showed tonofibrils and desmosomes and was helpful to recognize the epithelial nature of the tumour. The diagnosis of dedifferentiated adamantinoma was retained. In the right tibia biopsy revealed fibrous dysplasia. Amputation above the knee was performed followed by chemotherapy. One year later, the patient is still alive with multiple lung metastases. In our study, we discussed the clinico-pathologic features of this particular pattern of adamantinoma as well as its association with fiobrous dysplasia in this case. A review of literature was done.
Clear cell meningioma is a rare subtype of meningiomas graded II according to the World Health Organisation classification. In spite of its benign appearance, clear cell meningioma has an aggressive behaviour and it is characterized by its inordinately tendency to metastasize. The purpose of this study is to discuss the clinico-pathological features of this subtype of meningiomas as well as the metastatic pathways. We wish to report a rare case of a clear cell meningioma metastasizing to the sacrum 17 years after the removal of the primary tumour. A 26 year-old man was referred to our centre for low back pain related to a lytic lesion of the sacrum. He had a history of a tumour of the forth cervical vertebra that was removed when he was 9 year-old. CT scan revealed an osteolysis of the entire sacrum invading the intrapelvic organs and the sacro-iliac joints. Open biopsy revealed a clear cell meningioma. That was the same pattern of the tumour removed 17 years earlier. Chest CT showed lung metastases. The patient was managed conservatively by palliative radiation therapy. One year later, he experienced improvement of pain and walk. The mass was stable. Clear ell meningioma is an aggressive tumour with a potential to spread via cerebro-spinal fluid and haematologically. Patients with such a tumour should be closely followed for a long time.
