Juxtacortical Osteosarcoma Research Articles

Juxtacortical Osteosarcoma-Imaging Spectrum

Juxtacortical Osteosarcoma (OS) presents with three sub-types. These include parosteal OS, periosteal OS, and Highgrade surface OS. Imaging diagnosis of sub-types of Juxtacortical osteosarcoma is difficult. However, an analysis of 14 cases of these sub-types presents a spectrum of findings in each entity. The analysis of the Site, Transitional zone, Age, Matrix mineralization, Periosteal reaction, and Soft tissue component (STAMPS) are essential in making the final diagnosis and differential diagnosis.

The preliminary report of intercalary diaphyseal endoprosthetic reconstruction for the proximal femur malignant bone tumor

Objective To evaluate the clinical effectiveness of the treatment for the proximal femur malignant bone tumor by conducting intercalary diaphyseal endoprosthetic reconstruction. Methods Form June 2011 to July 2014, 7 patients who had proximal femur malignant bone tumor were treated with intercalary diaphyseal endoprosthetic reconstruction. There were 3 males and 4 females, 61.8 years old on the average (ranging from 28 to 76 years). The tumors involved: 4 case of the limbs primary malignant tumor, 1 case of osteosarcoma, 1 case of juxtacortical osteosarcoma, 1 case of malignant lymphoma with pathological fracture, and 1 case of malignant fibrous histiocytoma with pathological fracture, as well as 3 cases of the Metastatic breast cancer of the proximal femur. All patients were evaluated by plain radiography, CT and MRI before operation. After these 7 patients were examined with MRI and the level of osteotomy was determined, prosthesis fitting is ready. The exposures of all the tumors were via the femur lateralis surgical approach. According to the general principles of tumor surgery, expose the tumor segment and soft tissue tumors. The segment of involved bone was then removed, with a surrounding cuff of normal tissue overlying the tumor. The level of osteotomy was determined as the result of preoperative imaging measurement. The proximal and distal femur intramedullary canals are reamed. The prosthesis is assembled and installed after the cement used. Results The duration of surgery was from 45 min to 120 min (90 min on average). The blood loss was from 200 ml to 800 ml (400 ml on average). All patients were followed-up for 6 months to 2 years (14 months on average). 1 case with osteosarcoma died due to pulmonary metastases 18 months after surgery. The remaining 3 cases with the primary malignant bone tumors did not recur or transfer during the follow-up period. 1 case with the metastases was comprehensive treated in another hospital, and survival with tumors. The remaining 2 cases with the metastases did not recur or transfer during the follow-up period. Conclusion The clinical results demonstrated that the intercalary diaphyseal endoprosthetic reconstruction was an ideal treatment for the proximal femur malignant bone tumor. Key words: Femur; Neoplasms; Prostheses and implants

Orthopaedic Case of the Month: Painful Lower-leg Mass in a 76-year-old Man

Orthopaedic Case of the Month: Painful Lower-leg Mass in a 76-year-old Man

The safety evaluation of biopsy in malignant bone tumors

Objective To investigate the incidence and extent of biopsy tract contamination in malignant bone tumors by either core needle biopsy or open biopsy and detect the safe extent in resection of biopsy tract. Methods Forty-eight cases were performed core needle biopsy, including 37 osteosarcomas, 5 malignant fibrous histiocytomas, 1 juxtacortical osteosarcoma, 1 low grade central osteosarcoma, 1 periosteal osteosarcoma, 1 primary malignant melanoma of bone and 2 chondrosarcomas. There were 37 males and 11 females with a mean age of 23.3 years (range, 10-64 years). The mean time between core needle biopsy and definitive surgery was 1.3 months (range, 0-2 months). All the patients were performed limb salvage surgery.Twenty-six cases were performed open biopsy, including 20 osteosareomas, 1 Ewing's sarcoma, 2 chondrosarcomas, 1 mesenchymal chondrosarcoma, 1 malignant fibrous histiocytoma, 1 lymphoma. There were 21males and 5 females with a mean age of 21.9 years (range, 8-59 years). The mean time between open biopsy and definitive surgery was 2.3 months (range, 1-4 months). The tumor and tissue around the biopsy tract at least 2 cm were resected. The pathological examination was performed in specimens via the biopsy tract, including the normal soft tissue outside the tumor, deep fascia, subcutaneous tissue and skin. The incidence and extent of biopsy tract contamination were evaluated with pathological examination. Results Forty-four cases were followed up. The mean follow-up time was 17.6 months (range, 4-39 months). In core needle biopsy group, four of forty-eight cases were found malignant tumor cells seeding in biopsy tract, the positive rate was 8.3%. In open biopsy group, all the cases were followed up with the mean time of 12.9 months (range, 2-29 months), and two of twenty-six cases were found malignant tumor cells seeding in biopsy tract,the positive rate was 7.7%. Conclusion Biopsy of malignant bone tumors has the risk of biopsy tract contamination. The tumor cell seeding exists in both core needle biopsy and open biopsy. The biopsy tract should be performed en bloc resection with the tumor. Key words: Bone Neoplasms; Biopsy; Neoplasm seeding; Neoplasm recurrence, local

Periosteal Osteosarcoma of the Mandible: Case Report and Review of the Literature

Osteosarcoma (OS) is the most common primary malignancy of bone, with a reported incidence of 1:100,000 per year.1 These tumors typically originate in the extremities and the pelvis, with only 2.7–6.6% of OSs were located in the jaws.2,3 Although conventional OS arises intramedullary, some OSs occur on the outer surface of bone, this is called surface or juxtacortical OS and exhibits distinct radiographic, histologic features and biological behavior that is obviously different from conventional OS. OSs arising on the surface of bone can be classified into three types according to the nature of the tumor: parosteal, periosteal, and high-grade surface OS.4 Parosteal OS is a low-grade OS, with an incidence of about 4% of all OSs. Periosteal OS is an intermediate-grade chondroblastic OS, accounting for less than 2% of all OSs. High-grade surface OS is the most rare subtype, accounting for less than 1%. Periosteal type tends to have a better prognosis than that of conventional OS or high-grade surface OS, but poorer than that of parosteal OS and it is capable of local recurrence and distant metastasis.5 Periosteal OS occurs extremely rarely in the jaws. To our knowledge, including this report, only ten cases have been reported. We report a case of periosteal OS located at the mental region of the mandible and review the literature with respect to clinical, radiological and histopathological criteria, we also discuss the differential diagnosis.

Juxtacortical osteosarcoma of mandible

This article presents a case of juxtacortical (paraosteal) osteosarcoma in a 43-year-old female, which is rare malignant mesenchymal tumor. As per the literature, it accounts for less than 4% of all osteosarcomas. Due to its rare variety, this tumor can cause diagnostic dilemma and clinicians should be aware of it. Due to its clinical picture, it can be confused with peripheral fibro-osseous lesions. Success rate of treatment are good if the tumor is detected early and resected with wide margins giving a negligible chance for recurrence.

Paraosteal Osteosarcoma of Mandible: A Case Report

Osteosarcoma is the true malignant neoplasm of bone in which the malignant osteoblasts differentiate from the sarcomatous stroma and produce tumorous osteoid, tumorous bone and sometimes even tumorous cartilage. An uncommon separate class of osteosarcomas termed juxtacortical osteosarcoma, which includes intermediate grade periosteal and low-grade paraosteal variants represents less than 4% of all osteosarcomas and 1 % of all bone tumours. Juxtacortical osteosarcoma (incidence of 0.07 per 100,000 populations) occurs much less commonly than central medullary osteosarcoma. They have less aggressive biological behaviour as compared to the conventional type. So it is important to recognize these subtypes of osteosarcomas to plan treatment appropriately and avoid overtreatment. Juxtacortical osteosarcoma of the jaws appears to have a slightly more favourable prognosis than in long bones, although a larger series of cases needs to be evaluated before a definitive conclusion concerning the behaviour of juxtacortical osteosarcoma of the jaws can be made.

Juxtacortical Osteogenic Sarcoma of the Jaws: Case Report and Review of the Literature

Osteogenic sarcoma is the most common malignant tumor of bone cells, occurring in 1 of every 100,000 people.1 Approximately 7% of osteogenic sarcomas occur in the head and neck, and patients present in the third and fourth decades of age.1 Lesions of the mandible and maxilla are usually noted as bony, hard swellings of the buccal and lingual cortices and are often associated with separation of the teeth.1 Some lesions are exophytic hard nodules on the attached gingiva, appearing as soft tissue epulides.

50 Years Ago in CORR: Parosteal Osteogenic Sarcoma: Treatment by Block Resection Everett J. Gordon MD CORR 1959;14:171-178

As with most diseases, the concepts and terminology of bone sarcomas have changed over time. In the early part of the 20th century after the ready availability of biopsy and radiographs, doctors recognized only a few types of recognized primary malignant bone tumors. In his 1937 textbook, McMurray listed but three major bone tumors: osteogenic sarcoma, “Ewing’s Tumour,” and multiple myelomata [7]; Campbell, in 1939, listed five: chondromyxosarcoma, osteoblastic, chondroblastic, chondrosarcoma, and osteolytic [1]. While these numbers in common textbooks should not reflect the state of knowledge of malignant tumors, they do reflect the relative paucity of recognized types or subtypes of tumors. Osteogenic sarcomas, for example, were subsequently subtyped based primarily on differing prognoses. Among these subtypes were the various “juxtacortical” or “surface osteosarcomas,” including parosteal and periosteal tumors. The implication of these terms is that the tumors lie outside the medullary cavity, when in fact a small percent do not. Gordon, in 1959 in CORR [4], ascribed the original description of parosteal tumors to Geschickter and Copeland in 1951 [2]. The tumor was juxtacortical and had a histologic appearance similar to that of myositis ossificans and was considered to have benign and malignant forms, and with propensity to recur after resection or to metastasize. In their 13 cases, seven recurred after local resection and four of those patients died with metastases. They recommended amputation with recurrences owing to the rather grave prognosis. Clearly, the tumors were either inadequately resected or extended into the medullary cavity, and were associated with a worse prognosis. By 1959, Gordon noted a number of names ascribed to these sorts of tumors: periosteal osteogenic sarcoma, parosteal osteoma, juxtacortical osteogenic sarcoma, and parosteal osteogenic sarcoma. He also noted the difficulty of distinguishing this tumor from others: osteogenic sarcoma, exostosis, and osteochondroma (Fig. 1). He believed these lesions should not be biopsied owing to the potential for seeding, and rather recommended “local resection” (which we would likely consider marginal resection today). Gordon recommended en bloc resection for small lesions, but amputation “in most instances of parosteal osteogenic sarcoma.” Fig. 1 August, 1955, preoperative roentgenogram. Cortical mass, metaphysis, lower femur, no medullary involvement. (Reprinted with permission and ©Lippincott Williams & Wilkins, from Gordon EJ. Parosteal osteogenic sarcoma: Treatment by block ... While classic osteosarcoma constitutes the large majority of lesions, variants account for perhaps 20–25% with the following frequencies: telangiectatic 3.5–11%, parosteal 3–4%, periosteal 1–2%, gnathic 6–9%, and small cell 1% [5]. The high grade surface lesions are infrequent variants [6, 9]. With the exception of these latter lesions, surface sarcomas generally have a better prognosis than the intramedullary lesions and the parosteal sarcoma being better than the periosteal sarcoma. The subtypes reflect changes in thinking about these tumors. Contemporary imaging and histologic grading are crucial for determining how surface osteosarcomas should be treated: low grade parosteal tumors limited to the surface may be treated with wide or segmental resection, while high grade tumors or those extending into the intramedullary cavity should be treated with segmental resection and chemotherapy. Genetic characterization appears to relate to malignant potential: a subgroup of parosteal tumors have ring chromosomes with amplified material from chromosome 12 (12q13-15) and high grade osteosarcomas more frequently have amplified genetic sequences on the short arm of chromosome 12 than the low grade [3]. Furthermore, MDM2 and CDK4 genes are frequently co-amplified in two separate amplicons in parosteal OS and less frequently in classical high-grade OS [8]. Differential gene expression may imply different mechanisms or etiologies for the development of osteosarcoma variants. Genetically characterizing malignant potential will undoubtedly supplement imaging and help clinicians select more patient-specific treatment in the near future. In contrast to the aggressive recommendation of Gordon in 1959, most surgeons today rely on high quality imaging to select treatment and to avoid amputations when less radical surgery will suffice.

Small Cell Osteosarcoma of mandible: A rare case report and review of literature.

Osteosarcoma is the most common primary malignant tumor of bone but only 5% of these tumours occur in the jaws. Of all the varieties of osteosarcoma, the juxtacortical type is rare and comprises only less than 4% of all osteosarcomas. Juxtacortical osteosarcoma is further subdivided into parosteal (low grade) and periosteal (high grade). Osteosarcomas can be categorized histologically mainly into chondroblastic, osteoblastic, fibroblastic and numerous others. One of the rare histological variant is small cell osteosarcoma which consists of sheets of round cells that produce an osteoid matrix. The aim of this article is to present a rare case of periosteal osteosarcoma of mandible which on histopathological examination showed characteristics of small cell tumour.

Femoral parosteal osteosarcoma 18 years after its discovery: A case report

Osteosarcomas are a heterogeneous group of tumors with diverse anatomical, clinical, and progressional characteristics. Parosteal osteosarcoma, or juxtacortical osteosarcoma, is a rare form of osteosarcoma that develops at the bone surface, but has a better prognosis than other conventional osteosarcomas. We report the observation of a 22-year-old female patient whose initial presentation was an enormous tumefaction of the knee that had been progressing for 10 years. The biopsy concluded in PO of the lower third of the femur. Staging was negative. The tumor had reached an enormous size and required amputation of the left lower extremity. A custom external prosthesis was manufactured to get her back to walking. Eight years after surgery, no local recurrence or metastasis has been detected. Parosteal osteosarcoma is a rare form of osteosarcoma with very slow progression (in spite of the particularly dramatic presentation in our observation), with an excellent prognosis and very rare metastasis.

Parosteal Osteoclastic Osteosarcoma in the Left Tarsal Joint of A Cat

Summary A parosteal (juxtacortical) osteosarcoma case was diagnosed on the lateral aspect of the left tarsal joint of an 11-year-old male Siamese-crossbred cat. A radiolucent line of demarcation between the tumor and the underlying cortical bone was present. The immobile mass was of soft to firm consistency with cystic areas on the cut surface. Histologically, the tumor tissue consisted generally of large, ovoid or fusiform cells with vesicular nuclei and partially of epithelioid like mesenchymal elements and abundant osteoclastic giant cells. Cellular pleomorphism and mitotic activity were found. The tumor cells were observed to have produced partially hyalinized collagen like osteoid matrix and constituted few crude osteochondroid areas. Partial aneurismal cysts like vascular structures in association with numerous giant cells resembling osteoclasts were present in the tumoral mass with marked necrosis. Based on the radiological and histomorphological features, the tumor with numerous osteoclasts and aneurysmal cystic changes was classified as parosteal osteoclastic osteosarcoma.

A Comparison of Intramedullary and Juxtacortical Low-grade Osteogenic Sarcoma

While low-grade juxtacortical and low-grade intramedullary osteogenic sarcomas are histologically indistinguishable, they have been studied as separate entities. We retrospectively reviewed the clinical, radiographic, histologic features and treatment of 59 patients treated surgically to compare the rate of local recurrence, grade progression, and survival between low-grade intramedullary and low-grade juxtacortical osteogenic sarcoma. Forty-five (76%) patients were treated for low-grade juxtacortical osteogenic sarcoma and 14 (24%) were treated for low-grade intramedullary osteogenic sarcoma. Local recurrence rates of 7% were similar for both groups studied. The rate of distant metastases was also similar for both groups. . The rate of dedifferentiation for the entire group was 29%. Dedifferentiated lesions were treated with adjuvant chemotherapy in 16 of 17 cases. Recurrence preceded dedifferentiation in four cases. Five-year survival was over 90% in both groups. Low-grade intramedullary and low-grade juxtacortical osteogenic sarcoma were clinically indistinguishable with identical rates of local recurrence, distant metastases, dedifferentiation, and survival. Level III, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.

Selected Papers Presented at the 2007 Meeting of the Musculoskeletal Tumor Society: Editorial Comment

The meritorious papers selected from the 2007 MSTS meeting comprise a wide range of topics. They underscore the fascinating breadth of orthopaedic oncology. Although the articles are diverse in content, they reflect important areas of research where data are lacking. One of the challenges in clinical care of patients with metastatic bone disease is how to determine whether a destructive lesion should be stabilized. Assessing the risk of impending fracture is imprecise, and efforts have been made in the past 5 years to utilize more precise imaging techniques to outline indications for treatment. The Mirels classification is widely used as a guideline to stabilize impending fractures, but it was originally validated only for the femur. Evans et al. in this symposium have utilized the Mirels scoring tool to evaluate pathologic lesions in the humerus. From the same institution, Mann et al. are tackling the issue from a more translational angle by developing a murine model of osteolysis. By using noninvasive imaging and finite element modeling, they have developed an algorithm by which to estimate bone strength in the setting of osteolytic lesions. We applaud the institutional efforts to study this problem from both basic science and clinical perspectives. The survival of patients with osteosarcoma has not changed substantially in the past 25 years and this is another area where both clinical and basic research efforts are needed. The article by Hugate et al. provides the latest update of a series of 53 patients with osteosarcoma or malignant fibrous histiocytoma of bone treated with intraarterial cisplatinum combined with more traditional intravenous chemotherapy. These patients had an 82% 10-year survival and there was a suggestion of improved local tumor necrosis and ease of surgical resection when compared historically with series of patients treated by standard intravenous protocols. From a translational perspective, Sorenson et al. have developed a murine model of metastatic osteosarcoma. They are evaluating treatment using Salmonella typhimurium with or without interleukin-2 in order to cause tumor cell death and harness the power of the local immune system, respectively. This translational work is vital if we hope to improve the survival of patients with osteosarcoma over the next 25 years. The remaining articles within this collection are unrelated but will improve patient care and contribute to the education of the next generation of orthopaedic oncologists. Sawamura et al. evaluated a large series of patients treated with rotationplasty for bone sarcomas. They critically investigated the complications associated with this unusual procedure and provided insight into which patients should be indicated for this treatment. Institutions with a long history of orthopaedic oncology have the opportunity to evaluate rare tumors or procedures and this is highly encouraged. Damron et al. have retrospectively reviewed a rare surgical reconstructive tool, the intercalary humeral spacer. With a new design, this spacer provides excellent stability in uniquely selected patients with humeral diaphyseal lesions. Schwab et al. have reviewed the extensive records at Memorial Sloan-Kettering Cancer Center in order to better distinguish the clinical behavior of two histologically similar tumors: low grade juxtacortical osteosarcoma and low grade intramedullary osteosarcoma. They reported indistinguishable rates of recurrence and metastasis. Finally, Frassica et al. critically reviewed the pathology questions on the Orthopaedic In Training Examination for the past five years. They evaluated the specific content and question types in order to identify how to better teach residents prior to the examination. We compliment the authors on their efforts to bring this information to the orthopaedic community. The data advance the field of orthopaedic oncology and hopefully stimulate others to investigate related research areas. These authors submitted completed manuscripts shortly after the MSTS meeting, and we wish to encourage such timely submissions in the future.

Periosteal Osteosarcoma in the Hand of a Pediatric Patient: A Case Report

We report a case of periosteal osteosarcoma, an uncommon juxtacortical osteosarcoma variant, in the metacarpal of a 5-year-old girl treated with wide excision and chemotherapy with no recurrence at 25 months.

CASE REPORT: Multifocal Synchronous Low-grade Intramedullary and Juxtacortical Osteogenic Sarcoma

We report the case of a 41-year-old woman who presented with ipsilateral synchronous low-grade juxtacortical osteogenic sarcoma of the distal femur and low-grade intramedullary osteogenic sarcoma of the proximal tibia. The diagnosis and treatment and a review of the literature also are provided.

Parosteal osteosarcoma of the mandible

Parosteal Osteosarcoma, a variant of juxtacortical osteosarcoma, is seldom seen in the jaws. Here, a case of parosteal osteosarcoma of mandible in a 15-year-old female patient is presented, which occurred at the angle of mandible on the right side.The clinicopathologic features that distinguish this lesion from periosteal lesion are discussed. The contribution of computed tomography (CT) for the diagnosis is emphasized.

Chronic Knee Pain in a 21-Year-Old Woman

Chronic Knee Pain in a 21-Year-Old Woman

An Unusual Case of Juxtacortical Osteosarcoma Presenting on Tc-99m MDP Bone Scan With Central Photopenia

A 26-year-old man presented with a 2-year history of a slowly enlarging, nontender left lower-extremity mass. He denied any history of trauma. Initial plain films showed a subtle, well-circumscribed, calcific mass adherent in the left anteromedial proximal tibia. Subsequent computed tomography and bone scans were performed. Both studies demonstrated a mass with increased peripheral calcification. This pattern of distribution in an exophytic lesion has been reported to be highly suggestive of heterotopic ossification. Biopsy provided a diagnosis of juxtacortical osteosarcoma. This case demonstrates a disparity between the scintigraphic findings and histologic diagnosis in a patient with suspected heterotopic ossification. A photopenic center in an exophytic lesion on bone scan might not always indicate a benign lesion.

Telomerase activity in juxtacortical and conventional high-grade osteosarcomas: correlation with grade, proliferative activity and clinical response to chemotherapy

Seven samples from seven patients with juxtacortical osteosarcomas, and 27 samples from 19 patients with conventional high-grade osteosarcomas were investigated for a possible correlation between telomerase activity and clinicopathological features such as age, sex, and response to chemotherapy. Of seven juxtacortical osteosarcomas, telomerase activity was weakly positive in three parosteal osteosarcomas, and highly positive in one parosteal osteosarcoma. In contrast, of 27 conventional high-grade osteosarcomas, telomerase activity was weakly positive in eight tumors and highly positive in three. Of all samples, 44.1% of the osteosarcomas showed telomerase activity (57.1% of juxtacortical and 40.7% of conventional osteosarcomas). The majority of poor responders to chemotherapy showed no telomerase activity (nine of 11), whereas five of seven good responders showed strong or weak telomerase activity. There was a significant correlation between telomerase activity and the response to chemotherapy ( P<0.05). Telomerase activity was not correlated with MIB-1 proliferation index, age at the time of surgery, or sex. These findings suggest that telomerase activation occurs early in the oncogenesis of osteoblastic tumors without having an effect on the progression of these tumors. In malignant osteoblastic tumors, the biological significance of telomerase activation is different from that described for most epithelial cancers.