Bizarre parosteal osteochondromatous proliferation genetic. Parosteal osteosarcoma shows cartilaginous cap that can be confused with osteosarcoma ct or mri scan of the affected region microscopic and pathological evaluation of tissue biopsy the specimen is examined under a microscope by a pathologist, to arrive at a definitive diagnosis. It is generally a lowgrade highly differentiated tumor but has the potential to turn into a highgrade poorly differentiated tumor, even though the incidence of such conversion is very low. It typically presents in early adulthood and middle age with a. Paul chihhsueh chen, md, phd parosteal osteosarcoma is a rare malignant bone tumor arising from the bone cortical surface. Osteoma is a benign, slowly growing, asymptomatic, osteogenic neoplasm. Unlike conventional osteosarcomas, it involves an older age group typically in the 3 rd and 4 th decades of life and shows a slight female predilection 1, 3, 4. Only 12 cases of intraoral parosteal osteosarcoma have been reported in the english language literature. Bizarre parosteal osteochondromatous proliferations bpop, also known as a nora lesions, are benign exophytic osteochondral lesions which have an appearance similar to an osteochondroma and are typically seen in the hands and feet. Parosteal osteosarcomas, being low grade lesions, can be treated by upfront. Mets are less common as compared to conventional os, about 210 % in grades 12 parosteal os, but rising to about 6070 %. A rare case report swati gupta 1, shilpa parikh 2, sumit goel 1 1 department of oral medicine and radiology, subharti dental college, swami vivekanand subharti university, meerut, uttar pradesh, india 2 department of oral medicine and radiology, government dental college and hospital, ahmedabad, gujarat, india. Nov 14, 2019 parosteal osteosarcoma usually occurs in older age with a peak in the third and fourth decades of life, with no sex predilection. Good prognosis better than high grade osteosarcoma, poorer than parosteal osteosarcoma or juxtacortical chondrosarcoma, with high local recurrence rate and 15% metastatic rate radiology images afip images.
My 42yearold daughterinlaw has been diagnosed with parosteal osteosarcoma, low grade. The changes tell the cell to start making new bone when it isnt needed. We report a third such case, with a 32month disease free survival. Periosteal osteosarcoma, as described in chapter 11, is distinctly different from parosteal osteosarcoma radiographically and histologically. Of the 147 conventional parosteal osteosarcoma, 119 87. Parosteal osteosarcomas have an excellent prognosis 8095% longterm survival.
The result is a mass tumor of poorly formed bone cells that can invade and destroy healthy body tissue. Osteosarcoma os is a common primary malignant tumor of bone that produces osteoid matrix. Most common type of juxtacorticalsurface osteosarcoma. All 17 highgrade lesions evaluated with mr imaging were of predominantly high signal intensity on t2weighted images.
Parosteal osteosarcoma usually occurs in older age with a peak in the third and fourth decades of life, with no sex predilection. According to the world health organization, os of bone is classified into eight subtypes with distinct biologic behaviors and clinical outcomes. It typically presents in early adulthood and middle age with a peak incidence in the third decade. Presentations ppt, key, pdf logging in or signing up. Patients with dedifferentiated parosteal osteosarcoma had worse 5year 65% versus 96% and 10year survival 60% versus 96% when compared to conventional tumors p free p parosteal osteosarcoma is a low grade, well differentiated fibroblastic tumor that produces boneosteoid immature woven bone. John hunter is a professor in the department of radiology musculoskeletal section at uc davis school of medicine. Ahuja and coauthors and, more recently, ritschl and coauthors have tended to consider all surface osteosarcomas to be parosteal and have distinguished them by the histologic grade. Osteoma of a bone other than the skull and facial bones is extremely rare. In the mayo clinic study 5 of 226 parosteal os, 37 16% of the 226 tumors underwent dedifferentiation. The classic or socalled conventional osteosarcoma develops in the medullary cavity of the metaphysis of long bones. A cells dna contains the instructions that tell a cell what to do. Find out how osteosarcoma is tested for, diagnosed.
Parosteal osteosarcoma is a lowgrade, malignant, boneforming tumor that arises on the surface of bone. Nov 14, 2019 parosteal osteosarcoma is slowgrowing and late to metastasize. Radiation therapy for osteosarcoma american cancer society. A case of parosteal osteosarcoma with a rare complication. Mets may be seen in grade 2 and mainly when the underlying bone is invaded. A poorly defined softtissue component distinct from the ossific matrix is the most distinctive feature of highgrade parosteal osteosarcoma and may be an optimal site for biopsy. A case of parosteal osteosarcoma of the tarsus of a 23yearold woman is reported. Bizarre parosteal osteochondromatous proliferation. Osteosarcoma begins when a healthy bone cell develops changes in its dna. The majority are conventional osteosarcomas, and the relative benign parosteal osteosarcoma was only found once in the jaws, whereas relative benign periosteal osteosarcoma was not diagnosed. Pdf parosteal osteosarcomas are rare, lowgrade juxtacortical variant of.
Patients usually report a slowgrowing mass for years. It has a slight female predominance and most frequently occurs in the third decade of life, 2 which is a decade older than the peak age of the conventional counterpart. Oct 02, 2017 bizarre parosteal osteochondromatous proliferation bpop, also known as noras lesion, is an uncommon, benign bone tumor that grows on the surface of the bone. Compared with conventional osteosarcoma, parosteal osteosarcoma differs in prevalence with regard to sex and age. Approximately 8% to 25% of the cases reported in literature developed dedifferentiation, which was reported to had increase. Lesion arising within the medullary space of the bone most common type.
Xray shows lobulated bony outgrowth from proximal femoral shaft just distal to greater trochanter and introduction bizarre parosteal osteochondromatous proliferation bpop also known as noras disease is a rare condition. It commonly presents as a slowgrowing painless lump in the posterior aspect of the distal femur. Parosteal osteosarcoma is a lowgrade, malignant bone tumor that usually arises on the metaphyseal surface of long bones. It was attached to the bone but free from overlying structures. Parosteal osteosarcoma is a low grade, well differentiated fibroblastic tumor that produces boneosteoid immature woven bone. Learn about the risk factors for osteosarcoma and if there are things that might help lower risk. A poorly defined softtissue component distinct from the ossific matrix is the most distinctive feature of highgrade parosteal. Parosteal osteosarcoma information including symptoms, causes, diseases, symptoms, treatments, and other medical and health issues. The full text of this article is available in pdf format. Of all parosteal osteosarcomas 77% are located in the lower extremity. We report two cases mimicking an osteochondroma, radiologically and histologically and propose an explanation. Issues relevant to the management of rib parosteal osteosarcomas are discussed. Long term diseasefree survival is possible after resection of the local.
Patients with dedifferentiated parosteal osteosarcoma had worse 5year 65% versus 96% and 10year survival 60% versus 96% when compared to conventional tumors p free p free survival. Get an overview of osteosarcoma and the latest key statistics in the us. Parosteal osteosarcoma is a subtype of osteosarcoma and arises from the outer layer of the periosteum. Parosteal osteosarcoma atlas of musculoskeletal oncology.
It is the most common type of juxtacortical or surface osteosarcoma and accounts for 5% of all osteosarcomas. Dedifferentiation was significantly associated with an increased risk of metastases 47% at 5 years compared with 0% for the. Parosteal osteosarcoma proximal humerus age 28 cancer. Bizarre parosteal osteochondromatous proliferation arises. However, fibrous or cartilaginous tissue may coexist or even predominate.
Timothy rapp, chief of orthopedic oncology at nyu medical center. Parosteal osteosarcoma radiology reference article. Femoral parosteal osteosarcoma 18 years after its discovery. Whereas the intramedullary variant affects the underlying. It arises from the surface of the bone, namely the outer layer of the periosteum. The lung mass was found to be a metastasis from parosteal osteosarcoma and the biopsy of the forearm mass revealed a myositis ossificans. Parosteal osteosarcoma from a rib the annals of thoracic.
Osteosarcoma os is a malignant spindle cell sarcoma in which the malignant cells produce osteoid or bone in the background of a sarcomatous stroma. We report the case of a parosteal osteosarcoma of the distal ulna, treated with wide resection without reconstruction. Parosteal osteosarcoma is slowgrowing and late to metastasize. Seven of their patients underwent intralesional excision due to misdiagnosis and all of them developed local recurrence. Only six cases of parosteal osteosarcoma have previously been reported to arise from tubular bones in the hand. On imaging, bpops are shown to be continuous with the underlying cortex, but usually without continuation of the medulla. Parosteal osteosarcoma is a lowgrade malignant tumor which is capable of dedifferentiation. Parosteal osteosarcoma is a very rare bone tumor with a predilection for the posterior aspect of the distal femoral metaphysis. Parosteal osteosarcoma is a rare malignancy of the bone that usually arises in the long bones.
This is the second case of parosteal osteosarcoma of the tarsus reported in the english literature. Osteosarcoma of the head and neck is relatively rare as it constitutes only around 8. An extremely rare case of parosteal osteoma is reported. May 25, 2016 periosteal osteosarcoma, as described in chapter 11, is distinctly different from parosteal osteosarcoma radiographically and histologically. Bizarre parosteal osteochondromatous proliferation bpop, also known as noras lesion, is an uncommon, benign bone tumor that grows on the surface of the bone. Radiographs and ct demonstrate a parosteal osteosarcoma of the distal femur these images are from dr. Dedifferentiated parosteal osteosarcoma dpos is defined as a highgrade surface osteosarcoma which rarely occurs as either a primary or secondary event of conventional lowgrade parosteal osteosarcoma cpos. The majority of osteosarcoma cases of the head and neck are high. The patient developed lung metastasis and a mass in the interosseus membrane of the forearm proximally to the osteotomy. Parosteal osteosarcoma, juxtacortical osteosarcoma. Several incidences of dedifferentiation in cpos were reported by earlier studies 1824% 1,2,3. We present a case and discuss the diagnostic and therapeutic implications of a rare low. It most commonly occurs in young women over the metaphyseal region, especially the long bones near the knee joint. Before treatments start, the radiation team takes careful measurements with imaging tests such as mri scans to determine the correct angles for aiming the radiation.
However, at other times, it can be painful and lead to bone deformity. Parosteal osteosarcoma article about parosteal osteosarcoma. Parosteal osteosarcoma proximal humerus age 28 hi my name is cheryl. It accounts for about 4% of all osteosarcomas and, although rare, it is the most common type of osteosarcoma of the bone surface. Local recurrence is the rule after intralesional surgery. I have recently found out that i have a bone cyst on my tibia, the pain has been going on sence oct. Periosteal osteosarcoma cesari 2011 cancer wiley online. Rib parosteal osteosarcomas are even rarer, with only 2 cases in the literature. Periosteal osteosarcoma po is a rare primary malignant bone tumor.
A 51yearold woman presented with a large mass in the left supraclavicular fossa. These include florid reactive periostitis, 7 bizarre parosteal osteochondromatous proliferation or noras lesion, 5 myositis ossificans, 6 and ossified hematoma. This is the type of radiation therapy most often used to treat osteosarcoma. Abstract parosteal osteosarcoma is a rare malignancy of the bone that. Parosteal osteosarcoma pos is a slowgrowing tumor which originates from the outer layer of the periosteum and represents 65% of surface osteosarcomas and in our database accounts approximately for 4, 8% of all osteosarcomas. Dedifferentiated parosteal osteosarcoma of the maxilla. Parosteal osteosarcoma is a welldifferentiated variant of osteosarcoma that affects the surface of the bone. Conventional osteosarcoma with periosteal spread high grade surface osteosarcomas. The vast majority involve the metaphysis of the femur, humerus, or tibia. Parosteal definition of parosteal by medical dictionary. The only patient who did not undergo surgery died of disease after 9 months. It focuses highenergy beams on the tumor from a machine outside the body to kill the cancer cells. Parosteal osteosarcoma definition of parosteal osteosarcoma.
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