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Osteosarcoma arising in bone Paget disease

MedGen UID:
569679
•Concept ID:
C0334546
•
Neoplastic Process
Synonyms: osteosarcoma arising in bone Paget disease; Osteosarcoma Arising in Bone Paget's Disease; osteosarcoma arising in bone Paget's disease; Osteosarcoma Arising in Osseous Paget's Disease; osteosarcoma arising in osseous Paget's disease; Osteosarcoma Arising in Osteitis Deformans; osteosarcoma arising in osteitis deformans; Osteosarcoma Arising in Paget Disease of Bone; osteosarcoma arising in Paget disease of bone; Osteosarcoma Arising in Paget's Disease of Bone; osteosarcoma arising in Paget's disease of bone; Osteosarcoma in Paget disease of bone; Osteosarcoma in Paget's disease of bone; Paget Osteosarcoma; Paget osteosarcoma; Paget's Osteosarcoma; Paget's osteosarcoma
SNOMED CT: Osteosarcoma in Paget disease of bone (33681003); Osteosarcoma in Paget's disease of bone (33681003)
 
Monarch Initiative: MONDO:0004275

Definition

A sarcomatous transformation of pre-existing Paget disease of the bone. Osteosarcomas arising from Paget disease of the bone are high grade lesions and usually have a poor prognosis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOsteosarcoma arising in bone Paget disease

Professional guidelines

PubMed

Paget Disease of Bone for Primary Care.
Rianon NJ, des Bordes JK
Am Fam Physician 2020 Aug 15;102(4):224-228. PMID: 32803929
Clinical features and pharmacologic treatment of Paget's disease.
Hamdy RC
Endocrinol Metab Clin North Am 1995 Jun;24(2):421-36. PMID: 7656897

Recent clinical studies

Etiology

Paget Disease of Bone for Primary Care.
Rianon NJ, des Bordes JK
Am Fam Physician 2020 Aug 15;102(4):224-228. PMID: 32803929
The role of surgery and adjuvants to survival in Pagetic osteosarcoma.
Ruggieri P, Calabrò T, Montalti M, Mercuri M
Clin Orthop Relat Res 2010 Nov;468(11):2962-8. doi: 10.1007/s11999-010-1473-7. PMID: 20652460Free PMC Article
Sarcomas arising in Paget disease of bone: a clinicopathologic analysis of 70 cases.
Deyrup AT, Montag AG, Inwards CY, Xu Z, Swee RG, Krishnan Unni K
Arch Pathol Lab Med 2007 Jun;131(6):942-6. doi: 10.5858/2007-131-942-SAIPDO. PMID: 17550323
Osteosarcoma over the age of forty.
Grimer RJ, Cannon SR, Taminiau AM, Bielack S, Kempf-Bielack B, Windhager R, Dominkus M, Saeter G, Bauer H, Meller I, Szendroi M, Folleras G, San-Julian M, van der Eijken J
Eur J Cancer 2003 Jan;39(2):157-63. doi: 10.1016/s0959-8049(02)00478-1. PMID: 12509946
Neoplasms arising in Paget's disease of bone: a study of 82 cases.
Haibach H, Farrell C, Dittrich FJ
Am J Clin Pathol 1985 May;83(5):594-600. doi: 10.1093/ajcp/83.5.594. PMID: 3857854

Diagnosis

An update on imaging of Paget's sarcoma.
Tilden W, Saifuddin A
Skeletal Radiol 2021 Jul;50(7):1275-1290. Epub 2021 Jan 2 doi: 10.1007/s00256-020-03682-8. PMID: 33386903
Paget Disease of Bone for Primary Care.
Rianon NJ, des Bordes JK
Am Fam Physician 2020 Aug 15;102(4):224-228. PMID: 32803929
The Loss of Profilin 1 Causes Early Onset Paget's Disease of Bone.
Scotto di Carlo F, Pazzaglia L, Esposito T, Gianfrancesco F
J Bone Miner Res 2020 Aug;35(8):1387-1398. Epub 2020 Feb 19 doi: 10.1002/jbmr.3964. PMID: 31991009
Osteosarcoma in Paget's disease of bone.
Hansen MF, Seton M, Merchant A
J Bone Miner Res 2006 Dec;21 Suppl 2:P58-63. doi: 10.1359/jbmr.06s211. PMID: 17229010
Osteosarcoma of bone and its important recognizable varieties.
Dahlin DC, Unni KK
Am J Surg Pathol 1977 Mar;1(1):61-72. doi: 10.1097/00000478-197701010-00007. PMID: 203202

Therapy

Paget Disease of Bone for Primary Care.
Rianon NJ, des Bordes JK
Am Fam Physician 2020 Aug 15;102(4):224-228. PMID: 32803929
Osteoblastic osteosarcoma in monostotic Paget's disease.
Calabrò T, Mavrogenis AF, Ruggieri P
Musculoskelet Surg 2011 Apr;95(1):37-40. Epub 2011 Mar 16 doi: 10.1007/s12306-011-0100-4. PMID: 21409504
The role of surgery and adjuvants to survival in Pagetic osteosarcoma.
Ruggieri P, Calabrò T, Montalti M, Mercuri M
Clin Orthop Relat Res 2010 Nov;468(11):2962-8. doi: 10.1007/s11999-010-1473-7. PMID: 20652460Free PMC Article
Changing metastatic patterns of osteosarcoma.
Giuliano AE, Feig S, Eilber FR
Cancer 1984 Nov 15;54(10):2160-4. doi: 10.1002/1097-0142(19841115)54:10<2160::aid-cncr2820541016>3.0.co;2-p. PMID: 6593114

Prognosis

An update on imaging of Paget's sarcoma.
Tilden W, Saifuddin A
Skeletal Radiol 2021 Jul;50(7):1275-1290. Epub 2021 Jan 2 doi: 10.1007/s00256-020-03682-8. PMID: 33386903
Paget Disease of Bone for Primary Care.
Rianon NJ, des Bordes JK
Am Fam Physician 2020 Aug 15;102(4):224-228. PMID: 32803929
The Loss of Profilin 1 Causes Early Onset Paget's Disease of Bone.
Scotto di Carlo F, Pazzaglia L, Esposito T, Gianfrancesco F
J Bone Miner Res 2020 Aug;35(8):1387-1398. Epub 2020 Feb 19 doi: 10.1002/jbmr.3964. PMID: 31991009
Osteosarcoma of bone.
Unni KK
J Orthop Sci 1998;3(5):287-94. doi: 10.1007/s007760050055. PMID: 9732564
Osteosarcoma of bone and its important recognizable varieties.
Dahlin DC, Unni KK
Am J Surg Pathol 1977 Mar;1(1):61-72. doi: 10.1097/00000478-197701010-00007. PMID: 203202

Clinical prediction guides

Study of malignant fibrous histiocytoma: clinical, statistic and histopatological interrelation.
Pobirci DD, Bogdan F, Pobirci O, Petcu CA, Roşca E
Rom J Morphol Embryol 2011;52(1 Suppl):385-8. PMID: 21424079
Osteoblastic osteosarcoma in monostotic Paget's disease.
Calabrò T, Mavrogenis AF, Ruggieri P
Musculoskelet Surg 2011 Apr;95(1):37-40. Epub 2011 Mar 16 doi: 10.1007/s12306-011-0100-4. PMID: 21409504
Late sarcoma development after curettage and bone grafting of benign bone tumors.
Picci P, Sieberova G, Alberghini M, Balladelli A, Vanel D, Hogendoorn PC, Mercuri M
Eur J Radiol 2011 Jan;77(1):19-25. Epub 2010 Sep 15 doi: 10.1016/j.ejrad.2010.06.053. PMID: 20828957
Simultaneous multifocal sarcomatous degeneration in Paget's disease of bone. A hypothesis.
Choquette D, Haraoui B, Altman RD, Pelletier JP
Clin Orthop Relat Res 1983 Oct;(179):308-11. doi: 10.1097/00003086-198310000-00046. PMID: 6577998

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