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Journal of the Chinese Medical... Dec 2019Parosteal osteosarcoma (POS) is a unique low grade osteosarcoma. Two separate oncogenes, MDM2 and CDK4, are specifically amplified in POS. Its clinical behavior is...
BACKGROUND
Parosteal osteosarcoma (POS) is a unique low grade osteosarcoma. Two separate oncogenes, MDM2 and CDK4, are specifically amplified in POS. Its clinical behavior is usually indolent. In some occasions, it may progress to high grade and become fatal. Malignant transformation with high grade differentiation is the most reliable indicator to predict its aggressiveness and metastatic potential. This study is to discover the relationship between gene amplification and grading.
METHODS
Retrospective analysis of MDM2/CDK4 expression/amplification using immunostaining, multiplex quantitative polymerase chain reaction (MQPCR) and fluorescence in situ hybridization (FISH) were studied on 14 patients with recurrent POS.
RESULTS
Forty tumor specimens in formalin-fixed paraffin-embedded blocks from 14 patients of POS were included in this study. Twenty-seven tumors are low-grade, 13 are high-grade. All POS showed increased expression of both MDM2 and CDK4 proteins, but not those from conventional osteosarcoma. Except some tumors were non-informative (poor DNA quality), the rest of POS had a marked increase of MDM2 and CDK4 genes copies by MQPCR, and confirmed by MDM2 FISH. Moreover, the folds of amplification increase as tumors progress. And, the amplification folds in high-grade POS are consistently higher than those of conventional ones.
CONCLUSION
FISH and MQPCR are both useful assays for estimating oncogene amplification status in bone tumors. Amplification levels of MDM2 and CDK4 are related to tumor grading and progression. Molecular determination of gene amplification status can be a reliable alternative for predicting clinical behavior of POS at small biopsies.
Topics: Adolescent; Adult; Bone Neoplasms; Child; Cyclin-Dependent Kinase 4; Female; Gene Amplification; Humans; In Situ Hybridization, Fluorescence; Male; Multiplex Polymerase Chain Reaction; Neoplasm Grading; Osteosarcoma, Juxtacortical; Proto-Oncogene Proteins c-mdm2; Retrospective Studies; Young Adult
PubMed: 31634336
DOI: 10.1097/JCMA.0000000000000211 -
The Journal of Hand Surgery Jul 2020Osteogenic sarcoma of the hand is very rare and fewer than 50 cases have been reported. There are only 2 previously reported cases of parosteal osteosarcoma involving...
Osteogenic sarcoma of the hand is very rare and fewer than 50 cases have been reported. There are only 2 previously reported cases of parosteal osteosarcoma involving the phalanges. We report a primary parosteal osteosarcoma involving the proximal phalanx of the right middle finger in a 20-year-old woman, which was managed by ray amputation. There was no evidence of local recurrence or systemic metastasis in the postoperative follow-up period of 2 years.
Topics: Adult; Bone Neoplasms; Female; Fingers; Humans; Neoplasm Recurrence, Local; Osteosarcoma; Osteosarcoma, Juxtacortical; Young Adult
PubMed: 31537402
DOI: 10.1016/j.jhsa.2019.08.006 -
A clinical comparison between dedifferentiated low-grade osteosarcoma and conventional osteosarcoma.The Bone & Joint Journal Jun 2019The purpose of this study was to clarify the clinical behaviour, prognosis, and optimum treatment of dedifferentiated low-grade osteosarcoma (DLOS) diagnosed based on... (Comparative Study)
Comparative Study
AIMS
The purpose of this study was to clarify the clinical behaviour, prognosis, and optimum treatment of dedifferentiated low-grade osteosarcoma (DLOS) diagnosed based on molecular pathology.
PATIENTS AND METHODS
We retrospectively reviewed 13 DLOS patients (six men, seven women; median age 32 years (interquartile range (IQR) 27 to 38)) diagnosed using the following criteria: the histological coexistence of low-grade and high-grade osteosarcoma components in the lesion, and positive immunohistochemistry of mouse double minute 2 homolog (MDM2) and cyclin-dependent kinase 4 (CDK4) associated with MDM2 amplification. These patients were then compared with 51 age-matched consecutive conventional osteosarcoma (COS) patients (33 men, 18 women; median age 25 years (IQR 20 to 38)) regarding their clinicopathological features.
RESULTS
The five-year overall survival (OAS) rates in the DLOS and COS patients were 85.7% and 77.1% (p = 0.728), respectively, and the five-year progression-free survival (PFS) rates were 57.7% and 44.9% (p = 0.368), respectively. A total of 12 DLOS patients received chemotherapy largely according to regimens for COS. Among the nine cases with a histological evaluation after chemotherapy, eight showed a poor response, and seven of these had a necrosis rate of < 50%. One DLOS patient developed local recurrence and five developed distant metastases.
CONCLUSION
Based on our study of 13 DLOS cases that were strictly defined by histological and molecular means, DLOS showed a poorer response to a standard chemotherapy regimen than COS, while the clinical outcomes were not markedly different. Cite this article: 2019;101-B:745-752.
Topics: Adult; Biomarkers, Tumor; Bone Neoplasms; Case-Control Studies; Combined Modality Therapy; Female; Humans; Immunohistochemistry; Male; Neoplasm Grading; Osteosarcoma; Retrospective Studies; Survival Rate
PubMed: 31154837
DOI: 10.1302/0301-620X.101B6.BJJ-2018-1207.R1 -
Human Pathology Sep 2019Parosteal osteosarcoma is a low-grade malignant bone tumor that can undergo dedifferentiation. The aim of this study was to analyze clinicopathologic features associated...
Parosteal osteosarcoma is a low-grade malignant bone tumor that can undergo dedifferentiation. The aim of this study was to analyze clinicopathologic features associated with clinical outcome in a large cohort of patients. Patients consecutively treated for parosteal osteosarcoma at Rizzoli Orthopedic Institute from 1900 to 2018 were reviewed and analyzed. Clincopathologic data of 195 patients with parosteal osteosarcoma were analyzed. Age at diagnosis ranged from 9 to 75 years (median 31). Median follow-up time was 150 months (range, 3-720). The most common tumor locations were femur (61.5%), humerus (15.9%) and tibia (12.8%). Wide surgical margins were achieved in 125 (64.1%) patients. Medullary involvement was present in 69 (35.4%) cases. Dedifferentiation occurred in 48 (24.6%) patients. Forty-five patients developed recurrence (23.1%; median time to recurrence of 36 months). At last follow-up, 155 (79.5%) patients were alive and without evidence of disease, 8 (4.1%) were alive with active disease, 23 (11.8%) died from disease, and 9 (4.6%) from unrelated causes. Patients with dedifferentiated parosteal osteosarcoma had worse 5-year (65% versus 96%) and 10-year survival (60% versus 96%) when compared to conventional tumors (P < .001). Wide surgical margins had positive impact on both disease-free (P < .001) and overall survival (P = .036). Medullary involvement, age at presentation and tumor size had no impact on survival. Dedifferentiation is the most important factor that negatively impacts clinical outcome. Surgical aim is to ensure radical removal with wide surgical margins to improve disease-free survival.
Topics: Adolescent; Adult; Aged; Bone Neoplasms; Cell Dedifferentiation; Child; Disease-Free Survival; Female; Humans; Male; Middle Aged; Osteosarcoma, Juxtacortical; Prognosis; Retrospective Studies; Young Adult
PubMed: 31128127
DOI: 10.1016/j.humpath.2019.05.009 -
Cureus Nov 2018We report here a case of periosteal sarcoma in a 10-year-old female, along with quantitative values obtained with bone single photon emission computed...
Usefulness of Quantitative Bone Single Photon Emission Computed Tomography/Computed Tomography for Evaluating Response to Neoadjuvant Chemotherapy in a Patient with Periosteal Osteosarcoma.
We report here a case of periosteal sarcoma in a 10-year-old female, along with quantitative values obtained with bone single photon emission computed tomography/computed tomography (SPECT/CT), which were useful to evaluate treatment response to preoperative chemotherapy. Pretreatment radiograph images of the lower leg showed cortical thickening eroded by a broad-based soft-tissue mass without the involvement of the underlying cortex, while computed tomography (CT) revealed a small juxtacortical mass with thick calcification and periosteal reaction. In magnetic resonance imaging (MRI), the mass showed hypointensity in the inner part and isointensity in the outer part in T1-weighted images, while the inner part showed hypointensity and the outer part hyperintensity in T2-weighted images. Bone SPECT/CT results indicated the focal and intense uptake of the mass. Following neoadjuvant chemotherapy (NAC), radiograph and MRI results revealed a slight increase in size, with growing calcification. Although visual inspection of the bone SPECT/CT findings showed nearly the same amount of focal uptake, quantitative parameters determined with those findings were decreased, with maximum standardized uptake value (SUV), peak SUV, mean SUV, metabolic bone volume (MBV), and total bone uptake (TBU) reduced by -20.7%, -22.0%, -12.6%, -33.5%, and -41.9%, respectively. The excision biopsy at the surgery showed a pathological grade 1 (non-complete response) after NAC, including a more than 20% of cell necrosis part. The quantitative bone SPECT/CT was considered to reflect treatment response in this case.
PubMed: 30723654
DOI: 10.7759/cureus.3655 -
Journal of Medical Case Reports Jan 2019Masses which develop on the surface of the rib bones are rare. The differential diagnosis includes benign and malignant lesions.
INTRODUCTION
Masses which develop on the surface of the rib bones are rare. The differential diagnosis includes benign and malignant lesions.
CASE PRESENTATION
A 23-year-old European woman presented at an out-patient clinic with a 9-month history of a painless swelling on the right posterolateral side of her chest wall. The case reported here is of a very rarely seen parosteal osteosarcoma of the rib that was treated with wide resection and chest wall reconstruction. There was no evidence of local recurrence or distal metastasis after a 1-year follow-up.
CONCLUSION
Parosteal osteosarcoma is a locally aggressive malignant tumor, and resection with a wide margin is the most appropriate treatment. Correct diagnosis of parosteal osteosarcoma is challenging for an orthopedic surgeon. Although rare, in the differential diagnosis of lesions located on the ribs, parosteal osteosarcoma should be considered and a systematic diagnostic approach should be taken.
Topics: Bone Neoplasms; Bone Plates; Female; Humans; Osteosarcoma, Juxtacortical; Plastic Surgery Procedures; Ribs; Surgical Mesh; Thoracic Wall; Tomography, X-Ray Computed; Treatment Outcome; Young Adult
PubMed: 30670083
DOI: 10.1186/s13256-018-1958-7 -
Medicine Jan 2019Parosteal osteosarcoma (PO) is relatively rare, accounting for about 1% of primary malignant bone tumors and 4% of all osteosarcomas. Onset is generally at approximately...
RATIONALE
Parosteal osteosarcoma (PO) is relatively rare, accounting for about 1% of primary malignant bone tumors and 4% of all osteosarcomas. Onset is generally at approximately 20 to 40 years of age; the distal posterior surface of femur is the most common site, accounting for more than 60% cases. However, PO in middle-aged or elderly patients is extremely rare and PO occurring in the fibula is even rarer. To our knowledge, this is the first case of PO occurring in the fibula of a middle-aged adult.
PATIENT CONCERNS
We present a case of a 47-year-old female who had a lateral mass on her left knee for 3 months with no obvious cause.
DIAGNOSES
The patient was initially diagnosed with a proximal left fibula tumor via x-ray scan at a local hospital. Subsequently, osteochondroma was highly suspected through computed tomography (CT) and magnetic resonance imaging (MRI) obtained at our hospital. Finally, the postoperative pathological report confirmed PO.
INTERVENTIONS
She underwent a complete resection of the left fibula tumor and received postoperative adjuvant chemotherapy.
OUTCOMES
The patient recovered well and was discharged on the 10th day after admission. Additionally, there was no evidence of recurrence at the 4-year follow-up after surgery.
LESSONS
In middle-aged or elderly patients, early diagnosis of PO in the fibula is difficult and misdiagnosis or missed diagnosis is common. In clinical practice, PO is uncommon in middle-aged or elderly patients, occurs more rarely in the peroneal bone, and should be carefully identified. CT and MRI are essential for diagnosis, with pathological examination being effective for confirmation. Because of the limited reports of PO of the fibula in middle-aged or elderly adults, the present case raises awareness of this rare condition.
Topics: Bone Neoplasms; Diagnosis, Differential; Female; Fibula; Humans; Middle Aged; Osteosarcoma
PubMed: 30633158
DOI: 10.1097/MD.0000000000013857 -
Acta Ortopedica Mexicana 2018Dedifferentiated parosteal osteosarcoma is a variant in which a high grade osteosarcoma coexists with a parosteal osteosarcoma. We report the case of a 20-year-old...
Dedifferentiated parosteal osteosarcoma is a variant in which a high grade osteosarcoma coexists with a parosteal osteosarcoma. We report the case of a 20-year-old female patient who presented with six months of evolution of pain and functional limitation of the right forearm, with no apparent cause; radiographs were performed, observing a tumoral lesion in the diaphysis of the right ulna. Physical examination showed pain upon palpation in the diaphysis of the ulna and limitation of prone-supination. Axial computed tomography of the thorax revealed metastatic disease in the upper lobe of the left lung. An incisional biopsy was performed on the right ulna, with a report of dedifferentiated parosteal osteosarcoma. Therefore, the patient was managed with neoadjuvant chemotherapy with cisplatin and doxorubicin until completing three cycles. Surgical treatment consisted of intercalary resection of the diaphysis of the right ulna, plus reconstruction of the microvascularized autologous graft of the right fibular diaphysis and graft stabilization with 3.5 mm dynamic compression plate (DCP) and one-third tubular plate. In the same procedure, pulmonary metastasectomy was performed by thoracoscopy. Post-surgical histopathological report with 100% necrosis. Currently, the patient is asymptomatic, with no evidence of tumor activity. Dedifferentiated parosteal osteosarcoma is a rare pathology, but should be suspected as a differential diagnosis in the presence of a parosteal osteosarcoma; it should be taken into account that this disease can metastasize due to its dedifferentiated pattern. It is important to plan a surgical treatment that allows an adequate functional reconstruction, always taking into account the oncological principle.
Topics: Adult; Bone Neoplasms; Female; Humans; Osteosarcoma; Osteosarcoma, Juxtacortical; Tomography, X-Ray Computed; Ulna; Young Adult
PubMed: 30521709
DOI: No ID Found -
Radiology Case Reports Feb 2019Parosteal osteosarcoma originates on the surface of long bones. The juxtacortical variety is one of the most common ones and accounts for about 5% of all osteosarcomas....
Parosteal osteosarcoma originates on the surface of long bones. The juxtacortical variety is one of the most common ones and accounts for about 5% of all osteosarcomas. We report the case of a 34-year-old female patient with a rare variant of parosteal osteosarcoma. Because of the less aggressive biological behavior, it is important to know it, in order to recognize and differentiate it from benign osseous lesions.
PubMed: 30425774
DOI: 10.1016/j.radcr.2018.10.028 -
Oncology Letters Nov 2018Only few systematic and comprehensive studies have focused on osteosarcoma in children and adolescents. In the present study, 3,085 patients with osteosarcoma were...
Only few systematic and comprehensive studies have focused on osteosarcoma in children and adolescents. In the present study, 3,085 patients with osteosarcoma were identified in the Surveillance, Epidemiology and End Results Program database. The patients were <25 years of age and diagnosed between 1973 to 2012. A retrospective study was performed to investigate the factors associated with tumor incidence, metastasis, treatment and survival. The results indicated that the incidence of osteosarcoma was higher in male patients compared with female patients. In addition, the incidence rate of osteosarcoma was higher among male and female patients between the ages of 10 and 19. Osteosarcoma located in the chest and pelvic bones was associated with metastatic disease; however, metastasis in two histological types, parosteal and periosteal, was infrequent. Survival analysis revealed the following were associated with poor outcomes: Sex, patients diagnosed between 1973 and 1982, distant metastasis, treatment without surgery or with radiation, a tumor with a poorly differentiated or undifferentiated grade, tumor size ≥100 mm, and a tumor in the pelvic bones. Patient's whose histologic type was parosteal osteosarcoma and whose tumor was located in one of the limbs, or who underwent local or radical excision, exhibited a good survival outcome. Survival outcomes were ranked according to the type of surgery, from best to worst, as follows: Local excision, radical excision, amputation and no surgery. In summary, the incidence of osteosarcoma is higher in male patients compared with female patients. Furthermore, individuals between the ages of 10 and 19 have a higher risk of osteosarcoma. Osteosarcoma located in the chest and pelvic bones has a high risk of metastasis. Limb-salvage surgery may be the optimal treatment approach for non-metastatic osteosarcoma.
PubMed: 30405789
DOI: 10.3892/ol.2018.9453