Original articles and abstracts published between january 1991 and january 1997 were selected according to specified criteria and reviewed to provide answers to five interesting questions about the systemic treatment of metastatic osteosarcoma. 1 in patients with metastatic disease at presentation, what is the outcome after intensive multi agent chemotherapy? historically, survival has been poor, but may be improving with the use of ifosfamide containing regimens. 2 can response to new agents be evaluated better in patients who have received no previous chemotherapy? based on limited data, this is probably true. 3 is the response to neo adjuvant chemotherapy, as determined by histopathology, similar for the primary tumor and synchronous pulmonary metastases? with intensive multi agent chemotherapy, good histological response rates are in the range 70 x02013 90% for both groups. 4 what is the outcome, after intensive combined modality treatment with chemotherapy and surgery, in patients relapsing with metastases after previous adjuvant chemotherapy, and what are the important prognostic factors? outcome is highly variable, but 5 year survival ranges between 25 and 50% and a good outcome is more likely if recurrent disease is limited to resectable lung metastases. 5 can a biological agent l mtp pe prolong the time to relapse in patients with resected metastatic osteosarcoma? preliminary data suggest that this is possible, but more studies are required.

This article focuses on high grade intramedullary osteosarcoma often referred to simply as osteosarcoma , including its classic osteoblastic form and its fibroblastic and chondroblastic forms. 1, 2 this disease is thought to arise from primitive mesenchymal bone forming cells, and its histologic hallmark is the production of malignant osteoid. Other cell populations may also be present, as these types of cells may also arise from pluripotential mesenchymal cells, but any area of malignant bone in the lesion establishes the diagnosis as osteosarcoma. Most often, limb sparing limb preserving procedures can be used to treat patients with this disease and, thus, preserve function. Chemotherapy is also required to treat micrometastatic disease, which is present but often not detectable in most patients about 80% at the time of diagnosis.

3 telangiectatic osteosarcoma is a rare variant of osteosarcoma and hence its occurrence, presentation, and prognosis are poorly understood. With advancements in technology and available treatment options, the scenario of its diagnosis, management, and outcome has changed. Chemotherapy with surgery was challenged previously, but has now been proved to be beneficial. We reviewed the available literature and compared results to define the characteristics of the disease, its presentation, radiographic and pathologic features, optimal treatment, and prognosis. keywords: telangiectatic osteosarcoma, osteosarcoma, radiology telangiectatic osteosarcoma to is one of the rare subtypes of osteosarcoma os and accounts for 2% x02013 12% of all tumors in the appendicular skeleton.1 ,2 to is a rare variant of os with distinctive radiographic, gross, and microscopic features and prognostic implications. The common sites for the tumor location are the fastest growing long tubular bones the femur is involved most frequently, followed in frequency by the tibia and humerus x02013 a distribution similar to that of conventional os.2 in these bones, the metaphysis is the usual site of origin.3 x02013 5 other bones such as the sternum, scapula ribs, innominate bone, cranial vault, and mandible are uncommonly involved. To of the spine accounts for 2% of all cases of primary vertebral os.6 this primary high grade os of the bone is commonly seen in children, adolescents, and young adults.

By contrast, primary os in atypical locations such as the axial skeleton or soft tissues occurs in middle to late adulthood. In early days, to was diagnosed only when the mesenchymal tumor was found to be characterized by the direct production of osteoid or bone by malignant cells. Despite the early recognition of to by paget12 in 1853 and its classification as a variant of osteogenic sarcoma in 1922 by ewing,13 there is still disagreement regarding the incidence, clinical behavior, optimal treatment, and prognosis of this entity. The purpose of this review was to systematically study the available literature, compare the results, and define the characteristics of the disease, its presentation, radiographic and pathologic features, optimal treatment, and prognosis.

Fences By August Wilson Essay

Furthermore, the rare occurrence of the disease makes epidemiological studies difficult and the etiologic factors have not been extensively investigated. An 11 year old girl and an 8 year old boy who were cousins presented with tumors in metaphysis of distal tibia and the lamina of thoracic vertebra, respectively. The tumors were aggressive and destructive but the clinical outcome was favorable in both the cases.

The histologic features showed anaplastic tumor cells with osteoid and proliferation of blood cavities.14 mirra et al also described three well documented cases of pure, extra skeletal to of the soft tissues, with a x0201c pure x0201d telangiectatic gross and histologic pattern.10 the distinctive gross features of the tumors were a predominance of large, blood filled spaces. On histologic examination, large blood filled cavities in association with cyst like walls containing anaplastic spindle cells and definite osteoid production were noted in two of three cases. These cases suggest the presence of genetic factor in the development of to.10 to most frequently affects the femur, followed in frequency by the tibia and humerus however, the occurrence of to at other sites such as the patella, vertebrae, cranial vault, and mandible have also been reported.

Chowdhury et al15 reported a case of a 15 year old girl presenting with complaints of wrist pain and restricted movements of the wrist joint. The patient underwent resection of the distal 8 cm of the ulna after the confirmatory diagnosis of to. The forearm was amputated and the patient was aggressively treated with chemotherapy. A follow up was done for 42 months after her second operation, and no recurrence or metastasis was observed.15 the first case of the telangiectatic variant of os in the jaw bones was reported in a 15 year old chinese patient by chan et al.16 this patient had a 4 month history of progressive left cheek swelling and the diagnosis was confirmed by biopsy of the resected tumor. Initial excision and subsequent left hemimandibulectomy failed to eradicate the tumor.

Radiotherapy and then chemotherapy were given, but the patient showed no improvement. Right proptosis developed and the patient ran a downhill course, dying 16 months after presentation. Simon and irwin17 described a case of to in the anterior region of sacrum in a 31 year old male patient. The tumor was originally thought to be a chordoma and treated surgically, but on pathologic examination was diagnosed as to. The patient presented with decreased scrotal sensation and difficulty in urination and bowel movement postsurgery, which was subsequently resolved. He did not receive any adjuvant treatment and, after a follow up of 5 years, was found to have no recurrence or metastasis.17 another unusual case of to has been published by shehadeh et al,18 wherein a 22 year old male patient was referred with a history of anterior knee pain for 6 months, which had been originally diagnosed as chondromalacia patellae based on history. Review of anteroposterior and lateral radiographs of the knee demonstrated a diffuse destructive process involving the majority of the patella.

A second, noncontiguous lesion skip lesion involving the proximal tibia was also observed, with signal characteristics similar to that of the patellar lesion on magnetic resonance imaging mri. Based on the histologic findings and imaging studies, the patient was classified as having an enneking stage 3 american joint committee on cancer stage 4 metastatic to with both skip metastases and lung metastases. He received pre and postoperative chemotherapy and a palliative limb sparing surgery was done. The patient recovered and resumed activity but long term follow up was unavailable.18 amritanand et al19 described a rare case of to of the thoracic spine leading to paraplegia. The patient complained of back pain for 3 months and subsequently developed weakness and numbness of both lower limbs, which over a period of 1 week progressed to complete paraplegia with loss of bowel and bladder function.

Histopathological assessment of the specimen showed organizing fibrous tissue, giant cell reaction, and osteoid production. Three possible diagnoses were considered x02013 aneurysmal bone cyst abc , giant cell tumor gct , or a to. Open biopsy was done and the tissues were sent for a histopathological examination, which revealed a tumor showing multiple, aneurysmally dilated, large blood filled septate spaces with spindle cells and osteoclast type multinucleate giant cells. Scattered, markedly pleomorphic tumor cells with hyperchromatic, mitotically active nuclei were present in foci, lace like osteoid admixed with tumor cells and reactive new bone was seen, and a diagnosis of to was finally made. The patient was advised palliative chemotherapy and radiotherapy.19 another case of spine to with the involvement of cervical vertebrae has been reported by turel et al.2 the patient presented with a 6 month history of neck pain and torticollis. A cervical spine radiograph showed an expansile bony lesion involving the anterior and posterior elements of the c 5 vertebra with anterolisthesis of c 5 over c 6. The histopathological sample obtained at the first surgery corpectomy showed sclerotic cancellous bone with foci of hemorrhage, osteonecrosis, and spindle cell proliferation typical of an abc.

The patient underwent preoperative glue embolization followed by a staged subtotal c 5 spondylectomy and posterior fusion for a c 5 vertebral body lytic expansile lesion. Histopathological examination after the second surgery showed foci of malignant osteoid formation and a diagnosis of to was made. The surgery was followed by adjuvant radiation and chemotherapy with a favorable outcome at the 1 year follow up.2 a rare case of case of to involving multiple bones has been reported by park and joo.20 the patient complained of mild pain and swelling in her foot, occurring over a period of 4 months. A plain x ray of the foot showed a pure osteolytic lesion with involvement of the navicular, cuboid and 1, 2, and 3 cuneiform bones.