In Vitro Diffusion of DYE Through the End-Plates and the Annulus Fibrosus of Human Lumbar Inter-Vertebral Discs

Of the 242 cases of osteosarcoma recorded in the Swedish Cancer Registry for the years 1958 through 1968 only one was found to represent telangiectatic osteosarcoma. Another case was recently diagnosed in our department. The characteristic morphologic features of these neoplasms were anaplastic stroma, high mitotic activity, osteoid-formation, widely anastomosing blood spaces, and alkaline phosphatase activity. The experience gathered indicates that telangiectatic osteosarcoma constitutes a histopathologic variant of genuine osteosarcoma with a serious prognosis, necessitating the same kind of treatment as for the genuine tumour.     

Telangiectatic Osteosarcoma

Of the 242 cases of osteosarcoma recorded in the Swedish Cancer Registry for the years 1958 through 1968 only one was found to represent telangiectatic osteosarcoma. Another case was recently diagnosed in our department. The characteristic morphologic features of these neoplasms were anaplastic stroma, high mitotic activity, osteoid-formation, widely anastomosing blood spaces, and alkaline phosphatase activity. The experience gathered indicates that telangiectatic osteosarcoma constitutes a histopathologic variant of genuine osteosarcoma with a serious prognosis, necessitating the same kind of treatment as for the genuine tumour.     

Osteosarcoma oncogene expression detected by in situ hybridization

Fifteen archival human osteosarcoma specimens were examined by in situ hybridization for the expression of human and mouse transforming growth factor-β (isoforms 1, 2, and 3), c-fos, and metalloproteinase (stromelysin-3 and matrilysin). Osteosarcoma subtypes were confirmed by review of patients' radiographs, histopathology, and age at diagnosis. The outcome and method of treatment were documented. The subtypes of osteosarcoma consisted of nine conventional osteosarcomas and two each of fibroblastic, telangiectatic, and post-radiation osteosarcomas. Each specimen was histologically examined under light microscopy, and then adjacent paraffin sections were assayed with sense and anti-sense RNA probes by in situ hybridization. The probes localized to the neoplastic cells, confirming the methodology of the technique. Human transforming growth factor-β1 had the most uniform binding affinity to the osteosarcomas examined and was more specific in binding than mouse transforming growth factor-β1. Specific mRNA encoding for the transforming growth factor-βs, c-fos, and metalloproteinases are detectable in patterns within osteosarcoma cells, and collectively, their expression parallels the different histopathologic subtypes. The less differentiated subtypes (telangiectatic and post-radiation osteosarcomas) expressed the fewest molecular markers. Osteosarcoma is a heterogeneous tumor. Differential expression of matrilysin in osteosarcoma is the first reported detection of metalloproteinase activity in human skeletal sarcoma.     

Telangiectatic osteosarcoma

Telangiectatic osteosarcoma is a rare variant of osteosarcoma. In the original report from the authors' institution, a poor prognosis was noted. The authors have updated their experience with this entity. The prognosis for patients with telangiectatic osteosarcoma has improved remarkably. The prognosis in the present series seems to be the same as that for conventional osteosarcoma. Adjuvant chemotherapy seems to help in salvaging patients with metastatic disease. However, in this small series, survival of patients without metastasis is apparently not influenced by whether they received chemotherapy.     

Telangiectatic osteosarcoma: unusual behaviour

The telangiectatic variant of osteogenic sarcoma is rare. Its biologic behaviour, treatment and prognosis are controversial. The case of a 15-year-old girl with this tumour is described. Both the location and clinical behaviour of the tumour were unusual. The tumour, which involved the distal ulna, was initially treated by a limited resection of the distal 8 cm of ulna. Sixty-nine months later the tumour recurred locally; there was no evidence of metastases. The forearm was amputated and the patient was then treated aggressively by chemotherapy. She was well 42 months later. This case illustrates the tendency for telangiectatic osteosarcoma to recur locally if it is not radically excised.     

Ovarian sarcoma with histologic features of telangiectatic osteosarcoma of the bone

A primary osteosarcoma occurred in the left ovary of a 47-year-old Japanese woman. The preoperative diagnosis, based on computerized tomography, was cystic teratoma. The excised tumor was composed of large multilocular cysts containing blood and associated with an area of solid tissue. Histologically, the tumor was a "pure" osteosarcoma that showed prominent cellular anaplasia and blood-filled spaces lined with tumor cells. The lesion resembled a telangiectatic osteosarcoma of bone. Serum alkaline phosphatase levels reflected progression of the disease. Despite aggressive adjuvant chemotherapy, the patient died 8 months later of a local recurrence and intra-abdominal spread of the tumor.     

Fat embolism syndrome complicating intraarterial chemotherapy with cis-platinum

A 19-year-old man with telangiectatic osteosarcoma of the left proximal femur was started on a course of neoadjuvant chemotherapy consisting of intraarterial administration of cis-platinum. Within 72 hours of receiving the first intraarterial dose, the patient developed signs and symptoms of fat embolism syndrome (FES). A physical examination revealed cyanosis, tachycardia, and seizure activity. Laboratory studies demonstrated a pO2 of less than 65 mmHg, lipuria, and a drop in hematocrit of three percentage points. There was no clinical or roentgenographic evidence of pathologic fracture. Tumor necrosis secondary to intraarterial cis-platinum therapy in this patient with osteosarcoma may have caused a sudden release of free fatty acids and embolization of fat macroglobules that precipitated this episode of FES. FES in association with the intraarterial administration of cis-platinum seems not to have been previously reported.     

Telangiectatic osteosarcoma of the spine: a case report

Telangiectatic osteosarcoma (TOS) of the spine is rare accounting for only 0.08% of all primary osteosarcomas. Though a well described radio-pathological entity it is not often thought of as a cause of paraplegia. We describe the clinical, radiological and pathological features and discuss the treatment options of telangiectatic osteosarcoma of the dorsal spine presenting in a young man. The diagnostic pitfalls are discussed emphasising the fact that the diagnosis of TOS of the spine requires not only a multi modal approach of appropriate radiological and pathological tests but also an awareness of this condition.

     

Telangiectatic osteosarcoma of the extremity: Neoadjuvant chemotherapy in 24 cases

Between April 1990 and December 1994, we treated 24 patients with telangiectatic osteosarcoma (TO) of the extremities with neoadjuvant chemotherapy using 2 protocols. Surgery consisted of limb salvage in 21 patients and amputation or rotation plasty in 3. The histologic response to chemotherapy was good (90% or more tumor necrosis) in 23 patients, of whom 12 had total necrosis. With a mean follow-up of 74 (60-96) months, 20 patients remained continuously free of disease and 4 relapsed with lung metastases. There were no local recurrences. Comparing these results to the ones achieved in 269 contemporary patients with conventional osteosarcoma of the extremities using the same protocols for chemotherapy, we found a significantly better histologic response to chemotherapy (96% vs 68% of good histologic response; p = 0.004) and disease-free survival (83% vs 55%; p = 0.01) in the TO group. We conclude that TO, once considered a lethal tumor, seems to be even more sensitive to chemotherapy than conventional osteosarcoma, and that most of these patients may be cured without amputation.     

Telangiectatic osteosarcoma of the extremity: neoadjuvant chemotherapy in 24 cases

Between April 1990 and December 1994, we treated 24 patients with telangiectatic osteosarcoma (TO) of the extremities with neoadjuvant chemotherapy using 2 protocols. Surgery consisted of limb salvage in 21 patients and amputation or rotation plasty in 3. The histologic response to chemotherapy was good (90% or more tumor necrosis) in 23 patients, of whom 12 had total necrosis. With a mean follow-up of 74 (60-96) months, 20 patients remained continuously free of disease and 4 relapsed with lung metastases. There were no local recurrences. Comparing these results to the ones achieved in 269 contemporary patients with conventional osteosarcoma of the extremities using the same protocols for chemotherapy, we found a significantly better histologic response to chemotherapy (96% vs 68% of good histologic response; p = 0.004) and disease-free survival (83% vs 55%; p = 0.01) in the TO group. We conclude that TO, once considered a lethal tumor, seems to be even more sensitive to chemotherapy than conventional osteosarcoma, and that most of these patients may be cured without amputation.     

Telangiectatic osteosarcoma of the extremity: Neoadjuvant chemotherapy in 24 cases

Between April 1990 and December 1994, we treated 24 patients with telangiectatic osteosarcoma (TO) of the extremities with neoadjuvant chemotherapy using 2 protocols. Surgery consisted of limb salvage in 21 patients and amputation or rotation plasty in 3. The histologic response to chemotherapy was good (90% or more tumor necrosis) in 23 patients, of whom 12 had total necrosis. With a mean follow-up of 74 (60-96) months, 20 patients remained continuously free of disease and 4 relapsed with lung metastases. There were no local recurrences. Comparing these results to the ones achieved in 269 contemporary patients with conventional osteosarcoma of the extremities using the same protocols for chemotherapy, we found a significantly better histologic response to chemotherapy (96% vs 68% of good histologic response; p = 0.004) and disease-free survival (83% vs 55%; p = 0.01) in the TO group. We conclude that TO, once considered a lethal tumor, seems to be even more sensitive to chemotherapy than conventional osteosarcoma, and that most of these patients may be cured without amputation.     

Histologic response of high-grade nonmetastatic osteosarcoma of the extremity to chemotherapy,

In 510 patients with osteosarcoma of the extremity treated at the authors' institute between March 1983 and June 1995 with different regimens of neoadjuvant chemotherapy, factors that influenced the histologic response were investigated. The rate of total necrosis was not related to the patients' gender, age, site, size of tumor, serum of alkaline phosphatase values, or route of cisplatin administration. The histologic response significantly and independently correlated with the number of drugs administered before surgery and with the histologic subtype of the tumor. According to the number of drugs used, the percentage of total necrosis was 31% for a four-drug regimen, 18% for a three-drug regimen, and only 1.5% for a two-drug regimen. According to the histologic type, the rates of total necrosis were 41% for telangiectatic tumors, 36% for fibroblastic tumors, 15% for osteoblastic tumors, and 3% for chondroblastic tumors. The authors concluded that in neoadjuvant therapy of osteosarcoma, the histologic response to preoperative treatment, which correlates with prognosis, depends on the effectiveness of the chemotherapy regimen and on some features intrinsically inherent to the tumor. These data should be considered when selecting the type of treatment (adjuvant or neoadjuvant) and the combinations of drugs to be used in preoperative treatment of patients with osteosarcoma.     

Aneurysmal bone cyst of the acetabulum: a case report and long term follow-up

Aneurysmal bone cysts (ABCs) are benign, non-neoplastic, expansile, osteolytic lesions of unknown etiology. ABCs are most commonly located in the metaphysis of long bones. Differential diagnoses include unicameral bone cyst, chondromyxoid fibroma, giant cell tumour, osteoblastoma and telangiectatic osteosarcoma. We describe a case of a 14-year-old girl who presented to the accident and emergency department with an 18-h history of right-sided medial thigh pain. Plain radiology of the pelvis demonstrated a well-circumscribed lytic lesion with sclerotic margins in the medial part of the right acetabulum. Curettage and primary autogenous bone grafting was performed. The diagnosis of this condition requires a high index of suspicion. This report demonstrates that with adequate surgical treatment, a long-term, excellent outcome is achievable.     

Tibial Chondroblastic Osteosarcoma—Case Report

Osteosarcoma is a bone tumour that can occur in any bone. It most commonly occurs in the long bones of the extremities near the metaphyseal growth plates. The most common sites are femur (42%), tibia (19%), and humerus (10%). Other locations include the skull or jaw (8%) and pelvis (8%). A number of variants of osteosarcoma include conventional types (i.e., osteoblastic, chondroblastic, fibroblastic types) and telangiectatic, multifocal, parosteal, and periosteal types. We present a 10-year-old boy with Tibial Chondroblastic Osteosarcoma. The clinical features, diagnosis and management are discussed.     

Pathology of osteosarcoma.

Osteosarcoma of bone is a tumor composed of malignant cells that produce osteoid. Some tumors show predominant chondroid or fibromatoid ground substance. All, however, are highly malignant and about 80 per cent produce death with metastases. The roentgenogram affords important evidence for the correct diagnosis of many of them. Differential diagnosis should include consideration of those sarcomas with many benign giant cells and the group of "telangiectatic" osteosarcomas that may contain only small diagnostic areas. Malignant fibrous histiocytoma is now considered as a possible diagnosis for some malignant bone tumors, but the exact criteria for the diagnosis of this condition are still somewhat obscure. Newer modalities of adjunctive treatment, such as resection of pulmonary metastases, chemotherapy, and immunotherapy, give promise of improving the prognosis for osteosarcoma.     

Osteosarcoma: A Multifactorial Clinical and Histopathological Study with Special Regard to Therapy and Survival

A multifactorial analysis was performed on all 153 unequivocal cases of genuine osteosarcoma recorded in the Swedish Cancer Registry for the years 1958 through 1968. Cases of so-called parosteal osteosarcoma, soft-tissue osteosarcoma and osteosarcoma secondary to Paget's disease of bone were not included. The osteosarcomas were subclassifiecl as follows: osteoblastic (69 per cent), chondroblastic (19 per cent) and fibroblastic (12 per cent). The overall 5-year survival rate was 22 per cent; 55 per cent for those who had undergone amputation above the joint proximal to the involved skeletal part, 22 per cent for those amputated on the involved skeletal part, 11 per cent for those treated with local extirpation of the tumor, and 1 per cent in cases in which the lesion was not radically removed. Tumors of the femur, humerus and scapula were as malignant as axial tumors. The former carried a 5-year survival rate of 13 per cent, regardless of whether the patients had been treated with exarticulation or amputation on the involved skeletal part. Patients with axial tumors showed a 5-year survival rate of 15 per cent. These survival data suggest that proximal amputation alone might suffice for lesions situated distally to the knee and elbow joints, while tumors in the humerus and femur should be treated with amputation combined with multicytostatic treatment or immunotherapy and axial tumors with local resection and multicytostatic or immunologic treatment.     

Osteosarcoma. A multifactorial clinical and histopathological study with special regard to therapy and survival.

A multifactorial analysis was performed on all 153 unequivocal cases of genuine osteosarcoma recorded in the Swedish Cancer Registry for the years 1958 through 1968. Cases of so-called parosteal osteosarcoma, soft-tissue osteosarcoma and osteosarcoma secondary to Paget's disease of bone were not included. The osteosarcomas were subclassified as follows: osteoblastic (69 per cent), chondroblastic (19 per cent) and fibroblastic (12 per cent). The overall 5-year survival rate was 22 per cent; 55 per cent for those who had undergone amputation above the joint proximal to the involved skeletal part, 22 per cent for those amputated on the involved skeletal part, 11 per cent for those treated with local extirpation of the tumor, and 1 per cent in cases in which the lesion was not radically removed. Tumors of the femur, humerus and scapula were as malignant as axial tumors. The former carried a 5-year survival rate of 13 per cent, regardless of whether the patients had been treated with exarticulation or amputation on the involved skeletal part. Patients with axial tumors showed a 5-year survival rate of 15 per cent. These survival data suggest that proximal amputation alone might suffice for lesions situated distally to the knee and elbow joints, while tumors in the humerus and femur should be treated with amputation combined with multicytostatic treatment or immunotherapy and axial tumors with local resection and multicytostatic or immunologic treatment.     

Aneurysmal bone cyst

Aneurysmal bone cysts are rare skeletal tumors that most commonly occur in the first two decades of life. They primarily develop about the knee but may arise in any portion of the axial or appendicular skeleton. Pathogenesis of these tumors remains controversial and may be vascular, traumatic, or genetic. Radiographic features include a dilated, radiolucent lesion typically located within the metaphyseal portion of the bone, with fluid-fluid levels visible on MRI. Histologic features include blood-filled lakes interposed between fibrous stromata. Differential diagnosis includes conditions such as telangiectatic osteosarcoma and giant cell tumor. The mainstay of treatment is curettage and bone graft, with or without adjuvant treatment. Other management options include cryotherapy, sclerotherapy, radionuclide ablation, and en bloc resection. The recurrence rate is low after appropriate treatment; however, more than one procedure may be required to completely eradicate the lesion.     

Diaphyseal aneurysmal bone cysts (ABCs) of long bones in extremities: Analysis of surgical management and comparison with metaphyseal lesions

IntroductionAneurysmal bone cysts (ABCs) are benign lesions of long bone metaphysis affecting mostly medullary region in younger age below 20 years of life. These may be originated rarely either in the cortex or in the superficial regions of diaphysis. The study highlighted the differences of diaphyseal lesions from the usual metaphyseal ones in view of their clinical, radiological and biological behavior and also discussed their management in brief.Material and methodsWe reviewed a total of 84 aneurysmal bone cysts over the past fourteen years (2004–2017) and evaluated their surgical outcomes retrospectively. Total ten lesions were diaphyseal cortical in location (group I), while 74 lesions were conventional metaphyseal type (group II).ResultsWe observed that cortical ABCs were occurred commonly in diaphysis of femur, humerus, tibia and radius while presentation was at an older age than metaphyseal type. Radiographically these were eccentric lesion and more prone for pathological fractures than conventional type. These are differentiated radiographically from other benign lesions but also mimicking malignant conditions including low grade surface osteosarcoma and telangiectatic osteosarcoma while resemble similar to these on histopathological examination (HPE).ConclusionBiological behavior of cortical lesions does not differ significantly than conventional type but these are more prone for pathological fractures so these eccentric cortical ABC lesions should be treated with adequate internal fixation along with curettage and bone grafting. Although incidence of cortical or surface variety of ABCs is rare but surgeons might evaluate its severity in view of aggressive benign or malignant lesions of diaphysis.     

Prevention of postoperative progression of pulmonary metastases in osteosarcoma by antiangiogenic therapy using endostatin

Background We have previously offered data suggesting a positive linkage of postoperative up-regulation of systemic angiogenic activity and postoperative progression of pulmonary metastasis in osteosarcoma. The finding that the significant down-regulation of endostatin was critical in angiogenic elevation after primary tumor removal suggests that endostatin is a candidate for antiangiogenic therapy for osteosarcoma. Methods In the current study, we evaluated the effect of antiangiogenic therapy using endostatin on postoperative progression of pulmonary metastasis from osteosarcoma. Mouse osteosarcoma cell line LM 8 cells were inoculated in subcutaneous layer of nude mice. Two weeks after tumor inoculation, the primary tumor was removed surgically, and antiangiogenic therapy using adenovirus encoding endostatin expression vector (Ad5CMV-mEnd) was performed. Two weeks after the antiangiogenic treatment, pulmonary metastasis was evaluated by counting the number of metastatic nodules. The evaluation of systemic angiogenic activity was performed using Matrigel plug assay. Results Two weeks after the viral injection, mice were sacrificed, and the macroscopic pulmonary metastases were counted. Notably, the number of pulmonary metastases was smaller in the mice injected with Ad5CMV-mEnd than in controls, accompanied by significant suppression of systemic angiogenic activity. In addition, the sizes of the pulmonary metastases of the mice injected with Ad5CMV-mEnd were smaller than in the control group. Conclusions Our results indicate that antiangiogenic therapy using endostatin has the potential to prevent postoperative progression of pulmonary metastasis from osteosarcoma. Although this therapeutic strategy cannot provide a cure for osteosarcoma, it should enable osteosarcoma patients to coexist with dormant pulmonary metastasis and lead to improvement of their prognosis.