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Undifferentiated high grade pleomorphic sarcoma of bone

MedGen UID:
149264
Concept ID:
C0740479
Neoplastic Process
Synonyms: malignant bone fibrous histiocytoma; malignant fibrous histiocytoma of bone; malignant fibrous histiocytoma of the bone; Undifferentiated High Grade Pleomorphic Sarcoma; undifferentiated high grade pleomorphic sarcoma; Undifferentiated High Grade Pleomorphic Sarcoma of Bone; undifferentiated high grade pleomorphic sarcoma of bone; Undifferentiated High-Grade Pleomorphic Sarcoma; undifferentiated high-grade pleomorphic sarcoma
 
Monarch Initiative: MONDO:0002618

Definition

A rare, high-grade pleomorphic malignant neoplasm arising from the bone. It usually presents with pain which may or may not be associated with swelling in the affected area. It is characterized by the presence of spindle-shaped cells, polygonal or epithelioid cells, multinucleated giant cells, and inflammatory cells. The neoplastic cells exhibit nuclear pleomorphism and high mitotic activity. It metastasizes frequently, most often in the lungs. [from NCI]

Professional guidelines

PubMed

Tlemsani C, Larousserie F, De Percin S, Audard V, Hadjadj D, Chen J, Biau D, Anract P, Terris B, Goldwasser F, Pasmant E, Boudou-Rouquette P
Int J Mol Sci 2023 Jan 10;24(2) doi: 10.3390/ijms24021361. PMID: 36674874Free PMC Article
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142Free PMC Article
Han S, Yin X, Xu W, Wang Y, Han W
J Craniofac Surg 2020 Mar/Apr;31(2):e189-e192. doi: 10.1097/SCS.0000000000006162. PMID: 31934973

Recent clinical studies

Diagnosis

Al-Hassi HO, Ali NM, Cooke H, De Silva S, Brini AT, Babu P, Sumathi V, Morris MR, Niada S
Genes (Basel) 2024 Feb 14;15(2) doi: 10.3390/genes15020242. PMID: 38397231Free PMC Article

Clinical prediction guides

Al-Hassi HO, Ali NM, Cooke H, De Silva S, Brini AT, Babu P, Sumathi V, Morris MR, Niada S
Genes (Basel) 2024 Feb 14;15(2) doi: 10.3390/genes15020242. PMID: 38397231Free PMC Article

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