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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 -
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