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Poorly Differentiated Fibrosarcoma

MedGen UID:
235486
•Concept ID:
C1335442
•
Neoplastic Process

Definition

A usually aggressive malignant neoplasm arising from the deep soft tissue. It is characterized by the presence of poorly-differentiated spindle-shaped fibroblasts, collagenous stroma formation and increased mitotic activity. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPoorly Differentiated Fibrosarcoma

Professional guidelines

PubMed

Mohs micrographic surgery in the treatment of rare aggressive cutaneous tumors: the Geisinger experience.
Thomas CJ, Wood GC, Marks VJ
Dermatol Surg 2007 Mar;33(3):333-9. doi: 10.1111/j.1524-4725.2007.33069.x. PMID: 17338692
Cytogenetic and molecular genetic analyses of liposarcoma and its soft tissue simulators: recognition of new variants and differential diagnosis.
Meis-Kindblom JM, Sjögren H, Kindblom LG, Peydró-Mellquist A, Röijer E, Aman P, Stenman G
Virchows Arch 2001 Aug;439(2):141-51. doi: 10.1007/s004280100423. PMID: 11561754

Recent clinical studies

Etiology

Review of Potential Significance of Mutations of ADAMTS20, NF1 and PKHD1 Detected Using Next Generation Sequencing (NGS) in Dermal Fibrosarcoma Arising in Dermatofibrosarcoma Protuberans.
Kowalik A, Wincewicz A, Zięba S, Kopczynski J, Koda M, Sulkowski S, Kańczuga-Koda L, Góźdź S
Folia Med (Plovdiv) 2020 Mar 31;62(1):17-22. doi: 10.3897/folmed.62.e48003. PMID: 32337892

Diagnosis

Experimental pulmonary sarcoma metastases in athymic nude mice.
Chao TC, Greager JA
J Surg Oncol 1997 Jun;65(2):123-6. doi: 10.1002/(sici)1096-9098(199706)65:2<123::aid-jso9>3.0.co;2-9. PMID: 9209524
Resection of primary hepatic malignant fibrous histiocytoma, fibrosarcoma, and leiomyosarcoma.
Pinson CW, Lopez RR, Ivancev K, Ireland K, Sawyers JL
South Med J 1994 Mar;87(3):384-91. doi: 10.1097/00007611-199403000-00017. PMID: 7510907

Clinical prediction guides

Review of Potential Significance of Mutations of ADAMTS20, NF1 and PKHD1 Detected Using Next Generation Sequencing (NGS) in Dermal Fibrosarcoma Arising in Dermatofibrosarcoma Protuberans.
Kowalik A, Wincewicz A, Zięba S, Kopczynski J, Koda M, Sulkowski S, Kańczuga-Koda L, Góźdź S
Folia Med (Plovdiv) 2020 Mar 31;62(1):17-22. doi: 10.3897/folmed.62.e48003. PMID: 32337892

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