Low-grade Parosteal Osteosarcoma Research Articles

Est-ce que l’adhérence aux vaisseaux augmente l’incidence de récidive locale des ostéosarcomes paraostéaux du fémur distal ?

BackgroundProximity of tumor to the neurovascular bundle (NVB) makes achieving a wide margin difficult. In low-grade parosteal osteosarcoma (POS), it is not clear whether adherence of tumor to NVB increases the rate of local recurrence (LR). In this study, we evaluated whether tumor adherence to the NVB increases the risk of LR in low-grade POS of the distal femur? HypothesisWe hypothesized that if the thin neurovascular barrier (the adventitia of the vessels and the epineurium of the nerve) prevents tumor penetration, the rate of LR should be comparable between the lesions with and without a continuous layer of healthy fatty tissue between the tumor and NVB. Materials and methodsIn a retrospective survey, 30 patients with low-grade POS of the posterior aspect of the distal femur were evaluated for the proximity of mass to NVB. Based on the proximal to distal T1 axial MRI sections, the tumors were divided into two groups including the tumors with an uninterrupted (group A) and interrupted (group B) rim of fatty tissue between the mass and NVB. The rate of LR was compared between the two study groups. The concordance of MRI in detecting NVB adherence was checked with pathology specimen. ResultsUsing MRI, we identified 16 cases in group A and 14 cases in group B. The MRI status of fatty rim was concordant with pathology specimen in 96.4 % of cases. The mean follow-up period of the two groups was not statistically different (117±27.6 vs. 105.8±29.4 months, respectively, p=0.29). The other baseline characteristics of the two groups were statistically comparable, as well. The rate of LR was 12.5 % (2 out of 16 patients) in group A and 14.3 % (2 out of 14 patients) in group B (95 % CI: 0.142–9.586, p=0.87). The 10-year recurrence-free survival was 87.5 % for group A and 85.7 % for group B (p=0.9). DiscussionThe absence of a continuous rim of fatty tissue between the tumor and NVB in MRI does not increase the risk of LR in low-grade POS of the distal femur. Level of evidenceIV.

Does the adherence of distal femur parosteal osteosarcoma to the neurovascular bundle increase the rate of local recurrences?

BackgroundProximity of tumor to the neurovascular bundle (NVB) makes achieving a wide margin difficult. In low-grade parosteal osteosarcoma (POS), it is not clear whether adherence of tumor to NVB increases the rate of local recurrence (LR). In this study, we evaluated whether tumor adherence to the NVB increases the risk of LR in low-grade POS of the distal femur? HypothesisWe hypothesized that if the thin neurovascular barrier (the adventitia of the vessels and the epineurium of the nerve) prevents tumor penetration, the rate of LR should be comparable between the lesions with and without a continuous layer of healthy fatty tissue between the tumor and NVB. Materials and MethodsIn a retrospective survey, 30 patients with low-grade POS of the posterior aspect of the distal femur were evaluated for the proximity of mass to NVB. Based on the proximal to distal T1 axial MRI sections, the tumors were divided into two groups including the tumors with an uninterrupted (group A) and interrupted (group B) rim of fatty tissue between the mass and NVB. The rate of LR was compared between the two study groups. The concordance of MRI in detecting NVB adherence was checked with pathology specimen. ResultsUsing MRI, we identified 16 cases in group A and 14 cases in group B. The MRI status of fatty rim was concordant with pathology specimen in 96.4% of cases. The mean follow-up period of the two groups was not statistically different (117±27.6 vs. 105.8±29.4 months, respectively, p=0.29). The other baseline characteristics of the two groups were statistically comparable, as well. The rate of LR was 12.5% (2 out of 16 patients) in group A and 14.3% (2 out of 14 patients) in group B (95% CI: 0.142–9.586, p=0.87). The 10-year recurrence-free survival was 87.5% for group A and 85.7% for group B (p=0.9). DiscussionThe absence of a continuous rim of fatty tissue between the tumor and NVB in MRI does not increase the risk of LR in low-grade POS of the distal femur. Level of evidenceIV.

Surface osteosarcoma: Predictors of outcomes.

The subtypes of surface osteosarcomas include well-differentiated, low-grade parosteal osteosarcoma (POS), intermediate-grade periosteal osteosarcoma (PerOS), high-grade surface osteosarcoma (HGSO), and high-grade, dedifferentiated POS (dPOS). We aimed to determine disease progression, defined as local recurrence and metastatic disease, and overall (OS) and disease-specific survival (DSS). We identify outcome predictive factors and report functional results. This retrospective study evaluated patients with primary surface osteosarcoma at our hospital from 1992 to 2019. Fifty-one patients had a median follow-up of 6.1 years (range: 0.1-25.2). Histologic subtypes included 32 POS, 11 PerOS, 4 HGSO, and 3 dPOS. Bone and soft tissue margins were classified using the American Joint Committee on Cancer residual tumor classification (Rx = Not evaluable; R0 = negative margin; R1 = microscopic positive margin; and R2 = macroscopic positive margin) and the modified R classification (mRx = not evaluable; mR0 = negative margin >1 mm; mR1 = negative margin ≤1 mm; mR1-dir: Positive microscopic margin locally; mR2a: Positive macroscopic margin locally; mR2b: positive macroscopic margin distally; and mR2C: positive macroscopic margin locally and distally). Forty-one patients had functional outcomes. Three POS patients developed recurrence: two had R0 margins and one an intralesional resection. Five patients developed lung metastases (POS: 3, dPOS: 2). Four patients died. The only significant disease progression predictor was age. OS at 10 years was 97%. 48 patients had negative bone margins (R0 or mR0 and mR1) and 47 patients had negative soft-tissue margins (R0 or mR0 and mR1). The average MSTS score was 88.43 (range: 34.29-100). We advocate surgery for POS and believe R0 (mR0 and mR1 resections) or planned R1 (mR1-dir) to preserve function are acceptable. We favor chemotherapy and surgery for PerOS, though a chemotherapeutic response is highly variable. High-grade tumors are the most infrequent subtype, but HGSO and dPOS seem to portend a poorer prognosis. Good function can be obtained.

Protuberant fibro-osseous lesion of the temporal bone: report of four cases and review of the literature

‘Bullough lesions’, also referred to as protuberant fibro-osseous lesions (PFOL), are rare temporal bone lesions initially described in 1999. Since only 12 cases have been reported, several key issues, such as their origin and recommended management strategies, remain unresolved. This article reports the largest cohort included in the literature to date, comprising four patients with PFOL. PFOL appears to be characterized by female and right-side predominance. These lesions were consistently located regarding the mastoid, generally diagnosed in early adulthood, without functional symptoms, and were always fibro-osseous. Invasive/malignant features were not found on imaging or histology. The main differential diagnosis was malignant low-grade parosteal osteosarcoma. Clinical examination and computed tomography images provided strong elements supporting the diagnosis of PFOL. Biopsy allowed molecular biology investigations (MDM2 and CDK4 amplification), in order to rule out low-grade parosteal osteosarcoma.

The value of chest and skeletal staging in parosteal osteosarcoma: two-centre experience and literature review.

To determine the value of chest and skeletal staging in patients with parosteal osteosarcoma (POS), and the incidence of late local and chest recurrence. Retrospective review of patients across two institutions with histological confirmation of POS diagnosed between January 2007 and March 2020. Data collected included age, sex, skeletal location, results of chest CT and whole-body bone scintigraphy (WB-BSc) or whole-body MRI (WB-MRI) obtained at initial diagnosis. The histological tumour grade based on surgical resection specimens was classified as low-grade POS (LG-POS) and dedifferentiated POS (DD-POS). Findings of chest CT and skeletal staging were correlated with tumour grade. Follow-up chest CT and MRI studies were reviewed to determine the rate of late lung metastases and local recurrence. There were 27 males and 44 females, mean age 33years (range 12-79years). The femur (n = 43) and tibia (n = 14) were the most commonly involved bones. From surgical resection histology, 42 (59.2%) were LG-POS and 29 (40.8%) were DD-POS. WB-BSc/WB-MRI showed no skeletal metastases, while 1 case of DD-POS presented with bilateral calcified lung metastases. At follow-up, 7 patients (9.9%) developed lung metastases (mean of 18.9months, range 10-48months) from initial presentation, of which all were DD-POS. All but 1 patient who developed subsequent lung metastases had a local recurrence. Skeletal staging is unlikely to be of value in POS. Staging chest CT is very unlikely to demonstrate lung metastases in LG-POS and could be limited to DD-POS, particularly at the time of local recurrence.

Parosteal low-grade osteosarcoma with focal differentiation

This case study deals with the case of a 16-year-old male patient with a low-grade parosteal osteosarcoma of the distal femur with focal differentiation. Case history, disease course, and surgery as well as the pathological workup with final diagnosis are presented. Relevant radiologic and pathologic differential diagnoses und diagnostic pitfalls are explained in detail and discussed. Additionally, postoperative treatment options are illustrated.

Paraosteal osteochondromatosis proliferation (Nora’s lesion) of the great toe

Parosteal osteochondromatous proliferation also called Nora’s lesion is a rare tumor which occurs on the proximal/distal phalanx, metacarpals, metatarsals of hand (more commonly) and feet. These lesions show 50% recurrence following excision. It has atypical histo-pathological features that must be differentiated from chondrosarcoma, low grade parosteal osteosarcoma and conventional osteosarcoma. Among benign lesions florid reactive periostitis, myositis ossificans, periosteal chondroma and osteochondroma should be considered.

Rare Repeated Recurrence of Parosteal Osteosarcoma Adjacent to a Prosthesis after 15 Years: A Case Report

Late relapsing parosteal osteosarcoma has been reported very rarely; however, to the best of our knowledge, long-term repeated recurrence of this tumor has not been reported yet. Herein, we present a case of late relapsing low grade parosteal osteosarcoma after 15 years in popliteal cavity after advising tumor excision, removal of the organ involved, and the placement of prosthesis.

Parosteal osteosarcoma mimicking osteochondroma: A radio-histologic approach on two cases

ObjectiveParosteal osteosarcoma is a well-differentiated variant of osteosarcoma that affects the surface of the bone. The imaging pattern is very typical. We report two cases mimicking an osteochondroma, radiologically and histologically and propose an explanation.MaterialThe review of 86 parosteal osteosarcomas of bone revealed this atypical pattern only once. A consultation case was received in the same time, and added to ours. Patients were 28 years old and 56 years old females. Imaging studies included two radiographs, two CTscans, one MRI examination and one bone scan and the results were compared to histology.ResultsOn imaging, both lesions presented as ossified lobulated masses attached with a broad base to the underlying cortex. No radiolucent cleft separated the masses and the host bone and cortex continuity between the mass and the femur was seen, with medullary communication. The marrow of the mass had a different density and intensity compared to normal marrow. So, there were features of an osteochondroma (cortex and medullary continuity) and of a parosteal osteosarcoma (ossified marrow). Pathological assessment on the final specimen confirmed the presence of low-grade parosteal osteosarcomas, after an erroneous diagnosis of osteochondroma on the initial biopsy.ConclusionsParosteal osteosarcoma can be rarely confused with osteochondroma. A radiologic-pathologic correlation is essential. Cortex continuity is the most misleading imaging feature that may occur in parosteal osteosarcomas. A knowledge of this misleading pattern will help diagnose the lesion from the beginning.

Protuberant Fibro-osseous Lesion of the Temporal Bone: “Bullough Lesion”

Primary tumors of the calvarium are infrequent, and with the exception of osteoma, lesions confined to the surface of the skull are very rare. The differential diagnosis includes benign and malignant matrix forming tumors, other mesenchymal tumors, and reactive lesions. Fibro-osseous lesions are characteristically centered within bone and surface fibro-osseous lesions always prompt consideration of parosteal osteosarcoma, which is rare but well documented in the calvarium. We present 2 cases of a distinctive lesion of the temporal bone intimately related to the occipito-mastoid suture and typically presenting as a retroauricular soft tissue mass with calcific densities, confined to the soft tissues on the outer table of the skull without intraosseous involvement. The lesion is characterized histologically by rounded and ovoid zones of ossification within a bland fibrous stroma. The first 2 cases were documented in 1999 as "Protuberant fibroosseous lesion of the temporal bone.:" We present a further 2 cases, 1 of 2 years duration and the other with a 10-year history. This distinctive entity, which must be distinguished from other fibro-osseous lesions, including subtle low-grade parosteal osteosarcoma, seems to behave in a benign fashion and thus far recurrence is not documented. Local excision seems adequate. The pathologic features in the original report were documented by Prof Peter Bullough. As these cases were recognized by him alone we propose calling this entity "Bullough lesion" or, better still, "Bullough's Bump!"

Parosteal osteosarcoma dedifferentiating into telangiectatic osteosarcoma: importance of lytic changes and fluid cavities at imaging

Dear Sir: The scientific article entitled “Parosteal osteosarcoma dedifferentiating into telangiectatic osteosarcoma: importance of lytic changes and fluid cavities at imaging.” by Azura et al. published in the July issue of Skeletal Radiology [1] raises a few questions that need more clarification than the information in the paper provides. While this paper intends to demonstrate that telangiectatic osteosarcoma is the dedifferentiated component in low-grade parosteal osteosarcoma, the microscopic photographs do not adequately demonstrate that either of these components is present. There is some suggestion of these features in the imaging, but the premise of the paper and the confirmation of the imaging impression rest upon their histological correlation. Sincerely,

Parosteal Osteosarcoma of the Orbit

A 60-year-old woman was referred to Bristol Eye Hospital because of a progressive, painless, right proptosis. CT revealed a well-defined, hyperdense lesion adjacent to the lateral orbital wall. A marginal excision was performed. The mass was stony-hard, lobulated, and encapsulated. Histopathologic findings were consistent with a low-grade parosteal osteosarcoma. Parosteal osteosarcoma is a rare osteogenic tumor that usually affects the long bones. It represents a malignant, though well differentiated, tumor that has a relatively good prognosis after a wide excision. However, local recurrences are not rare and have been associated with dedifferentiation. The latter affects the prognosis adversely and, thus, regular follow-ups are strongly suggested after the initial tumor excision. In the present case, no further treatment was administered and the patient was reported disease free 26 months after surgery.

Parosteal osteosarcoma: Case report and review of the literature

The majority of osteosarcoma cases of the head and neck are high-grade lesions. We present a case and discuss the diagnostic and therapeutic implications of a rare low-grade parosteal osteosarcoma of the maxilla. A 32-year-old man presenting to the Head and Neck Surgical Oncology clinic with a 1-year history of a firm palatal mass. Evaluation clinically and radiographically raised the suspicion of an osteosarcoma. A partial maxillectomy revealed a parosteal osteosarcoma with negative margins. No adjuvant therapy was recommended, and the patient remains without evidence of local recurrence after 3 years. Parosteal osteosarcomas of the head and neck region are rare, low-grade variants of osteosarcoma, but have the potential to recur with simple local excision. Clinical and radiographic features are diagnostically helpful. Definitive diagnosis comes from histopathology, and wide local resection should be employed as the optimal treatment.

Chronic Knee Pain in a 21-Year-Old Woman

Chronic Knee Pain in a 21-Year-Old Woman

Characterization of bone and soft-tissue tumors with in vivo 1H MR spectroscopy: initial results.

To determine if in vivo detection of choline by using hydrogen 1 (1H) magnetic resonance (MR) spectroscopy with dynamic contrast material-enhanced MR imaging can help differentiate between benign and malignant musculoskeletal tumors. MR imaging was performed in 36 consecutive patients with bone and soft-tissue tumors larger than 1.5 cm in diameter. Examinations were performed at 1.5 T with a surface coil appropriate for the location of the lesions. Single-voxel 1H MR spectroscopy was performed by using a point-resolved spectroscopic sequence with echo times of 40, 135, and 270 msec. The volume of interest within lesions was positioned on the areas of early enhancement (<8 seconds after arterial enhancement) according to the findings of dynamic contrast-enhanced MR imaging with subtraction. The criterion for determining whether choline was present in a lesion was a clearly identifiable peak at 3.2 ppm in at least two of the three spectra acquired at echo times. MR spectroscopic results and histopathologic findings were determined in blinded fashion and compared with kappa statistics. P <.001 was considered to indicate a significant difference. Choline was detected in 18 of 19 patients with malignant tumors and in three of 17 patients with benign lesions. The three benign lesions included one perineurioma, one giant cell tumor, and one abscess. Choline was not detected in 14 patients with benign lesions nor in one patient with a densely ossifying low-grade parosteal osteosarcoma. In vivo 1H MR spectroscopy characterized bone and soft-tissue tumors, resulting in a sensitivity of 95%, specificity of 82%, and accuracy of 89% (P <.001). Choline can be reliably detected in large malignant bone and soft-tissue tumors by using a multiecho point-resolved spectroscopic protocol. 1H MR spectroscopy can help differentiate malignant from benign musculoskeletal tumors by revealing the presence or absence of water-soluble choline metabolites.

Hemicortical allograft reconstruction after resection of low-grade malignant bone tumours.

Low-grade surface tumours of bone may theoretically be treated by hemicortical resection, retaining part of the circumference of the cortex. An inlay allograft may be used to reconstruct the defect. Since 1988 we have performed 22 hemicortical procedures in selected patients with low-grade parosteal osteosarcoma (6), peripheral chondrosarcoma (6) and adamantinoma (10). Restricted medullary involvement was not a contraindication for this procedure. There was no evidence of local recurrence or distant metastasis at a mean follow-up of 64 months (27 to 135). Wide resection margins were obtained in 19 patients. All allografts incorporated completely and there were no fractures or infections. Fractures of the remaining hemicortex occurred in six patients and were managed successfully by casts or by osteosynthesis. The functional results were excellent or good in all except one patient. Hemicortical procedures for selected cases of low-grade surface tumours give excellent oncological and functional outcomes. There was complete remodelling and fewer complications when compared with larger intercalary procedures. The surgery is technically demanding but gives good clinical results.

Hemicortical allograft reconstruction after resection of low-grade malignant bone tumours

Low-grade surface tumours of bone may theoretically be treated by hemicortical resection, retaining part of the circumference of the cortex. An inlay allograft may be used to reconstruct the defect. Since 1988 we have performed 22 hemicortical procedures in selected patients with low-grade parosteal osteosarcoma (6), peripheral chondrosarcoma (6) and adamantinoma (10). Restricted medullary involvement was not a contraindication for this procedure. There was no evidence of local recurrence or distant metastasis at a mean follow-up of 64 months (27 to 135). Wide resection margins were obtained in 19 patients. All allografts incorporated completely and there were no fractures or infections. Fractures of the remaining hemicortex occurred in six patients and were managed successfully by casts or by osteosynthesis. The functional results were excellent or good in all except one patient. Hemicortical procedures for selected cases of low-grade surface tumours give excellent oncological and functional outcomes. There was complete remodelling and fewer complications when compared with larger intercalary procedures. The surgery is technically demanding but gives good clinical results.

Immunohistochemical detection of c-fos and c-jun expression in osseous and cartilaginous tumours of the skeleton.

The products of c-fos and c-jun proto-oncogenes form the heterodimeric complex AP-1 (activator protein 1), which play an important part in the control of bone cell proliferation and differentiation and in the development of bone tumours. We examined the expression of c-fos and c-jun in a series of 52 primary skeletal neoplasms, using an immunohistochemical method on formalin-fixed, paraffin-embedded sections. The expression of c-fos and c-jun was restricted to bone-forming lesions, while cartilaginous tumours were devoid of immunoreactivity. In benign osteoblastic lesions moderate c-fos and c-jun expression was found in 2 cases (18.1%). The highest levels of c-fos and c-jun expression were detected in high-grade central osteosarcomas (7 of 15 cases with moderate/diffuse expression) while 1 telangiectatic osteosarcoma, 2 low-grade central osteosarcomas, 1 low-grade periosteal osteosarcoma and 7 low-grade parosteal osteosarcomas were either negative or had low expression. The high-grade component of a dedifferentiated parosteal osteosarcoma showed diffuse immunoreactivity for both oncoproteins. Comparison of c-fos and c-jun expression by histological grade showed that high-grade osteosarcomas had a significantly higher expression of both oncoproteins than did low-grade osteosarcomas (P = 0.01, Fisher's exact test). Thus, c-fos and c-jun overexpression may be implicated in the development of high-grade osteosarcomas, but they appear to have little or no relevance for the development of low-grade osteosarcomas and cartilaginous skeletal neoplasms.

Cystic Periosteal Osteosarcoma in the Tibia of a Dog

SummaryPeriosteal osteosarcoma, a rare neoplasm in dogs, was diagnosed in an eleven- year-old Golden Retriever. In this case, the tumour formed a single, large, fluid filled cyst, that originated from the periosteum of the tibia. Periosteal osteosarcomas are intermediate grade tumours and behave less aggressively than central osteosarcomas, however, they are more malignant than the low grade parosteal osteosarcoma. Cure for periosteal osteosarcoma in people may be achieved in 70-80% of cases by local excision. In this case euthanasia was elected one year post-amputation due to emaciation and lethargy associated with metastatic disease.A cystic periosteal osteosarcoma was diagnosed in an eleven year old Golden Retriever. The tumour originated from the tibia and formed a single large fluid filled cyst. Periosteal osteosarcomas are rare, moderately aggressive tumours that may be differentiated from parosteal, surface, and central osteosarcomas. In this case euthanasia was elected one year post-amputation due to metastatic disease.

DNA analysis in the differential diagnosis of osteosarcoma.

The DNA content of osteosarcomas, and of giant cell tumors, osteoblastomas, aneurysmal bone cysts, and fibrous dysplasias was determined by cytophotometry. Of 158 tumors, 141 were histologically noncontroversial, whereas 17 posed diagnostic difficulties. In the noncontroversial group, all 41 benign tumors had a diploid (normal) DNA content. Ninety-two of 96 high-grade osteosarcomas were hyperploid (increased DNA content). The four analyzed low-grade parosteal osteosarcomas were diploid. Among 17 diagnostically controversial cases, nine were primarily diagnosed and treated as benign. Three of these patients, nevertheless, exhibited a malignant clinical course and two had local recurrence. All five proved to have hyperploid tumors. The four nonrecurrent lesions were diploid. Of eight patients primarily evaluated as malignant, one died and two developed local recurrence. These three patients had hyperploid tumors. Among the five nonrecurrent lesions, two were hyperploid and three diploid. In the diagnostically controversial group, recurrence or death was consistently related to hyperploidy. The present study shows that the vast majority of high-grade osteosarcomas are hyperploid. Benign bone tumors, which may be mixed up histologically with osteosarcoma, are diploid. Routine DNA analysis of primary bone tumors, as an adjunct to histopathologic assessment, can be employed to obtain diagnostic confirmation. In cases presenting histopathologic difficulties, ploidy determination may provide decisive diagnostic information.