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Soft Tissue Tumor of Uncertain Differentiation

MedGen UID:
233192
•Concept ID:
C1334776
•
Neoplastic Process
Synonym: Soft Tissue Neoplasm of Uncertain Differentiation

Definition

A benign,intermediate, or malignant soft tissue neoplasm in which the line of differentiation is uncertain. Representative examples include neoplasm with perivascular epithelioid cell differentiation, alveolar soft part sarcoma, desmoplastic small round cell tumor, epithelioid sarcoma, extraskeletal myxoid chondrosarcoma, and synovial sarcoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSoft Tissue Tumor of Uncertain Differentiation

Professional guidelines

PubMed

Head and neck sarcomas in adulthood: current trends and evolving management concepts.
Kalavrezos N, Sinha D
Br J Oral Maxillofac Surg 2020 Oct;58(8):890-897. Epub 2020 Jul 4 doi: 10.1016/j.bjoms.2020.05.015. PMID: 32636095
Ossifying fibromyxoid tumor: morphology, genetics, and differential diagnosis.
Schneider N, Fisher C, Thway K
Ann Diagn Pathol 2016 Feb;20:52-8. Epub 2015 Dec 2 doi: 10.1016/j.anndiagpath.2015.11.002. PMID: 26732302
Dendritic cell and histiocytic neoplasms: biology, diagnosis, and treatment.
Dalia S, Shao H, Sagatys E, Cualing H, Sokol L
Cancer Control 2014 Oct;21(4):290-300. doi: 10.1177/107327481402100405. PMID: 25310210

Recent clinical studies

Etiology

Mesenchymal lesions of the vulva.
Chapel DB, Cipriani NA, Bennett JA
Semin Diagn Pathol 2021 Jan;38(1):85-98. Epub 2020 Sep 6 doi: 10.1053/j.semdp.2020.09.003. PMID: 32958293
Soft Tissue Tumors of Uncertain Histogenesis: A Review for Dermatopathologists.
Buehler D, Weisman P
Clin Lab Med 2017 Sep;37(3):647-671. doi: 10.1016/j.cll.2017.06.005. PMID: 28802505
Soft-Tissue Sarcomas of the Abdomen and Pelvis: Radiologic-Pathologic Features, Part 2-Uncommon Sarcomas.
Levy AD, Manning MA, Miettinen MM
Radiographics 2017 May-Jun;37(3):797-812. doi: 10.1148/rg.2017160201. PMID: 28493803Free PMC Article
Smooth muscle tumors of soft tissue and non-uterine viscera: biology and prognosis.
Miettinen M
Mod Pathol 2014 Jan;27 Suppl 1(Suppl 1):S17-29. doi: 10.1038/modpathol.2013.178. PMID: 24384850Free PMC Article
Soft tissue tumors of uncertain origin.
Alaggio R, Coffin CM, Vargas SO
Pediatr Dev Pathol 2012;15(1 Suppl):267-305. doi: 10.2350/11-08-1079-PB.1. PMID: 22420732

Diagnosis

Updates on WHO classification for small round cell tumors: Ewing sarcoma vs. everything else.
Dehner CA, Lazar AJ, Chrisinger JSA
Hum Pathol 2024 May;147:101-113. Epub 2024 Jan 26 doi: 10.1016/j.humpath.2024.01.007. PMID: 38280658
Pearls and Pitfalls in the Imaging of Soft-Tissue Masses in Children.
Navarro OM
Semin Ultrasound CT MR 2020 Oct;41(5):498-512. Epub 2020 May 28 doi: 10.1053/j.sult.2020.05.014. PMID: 32980096
Soft Tissue Tumors of Uncertain Histogenesis: A Review for Dermatopathologists.
Buehler D, Weisman P
Clin Lab Med 2017 Sep;37(3):647-671. doi: 10.1016/j.cll.2017.06.005. PMID: 28802505
Myoepithelial tumor of soft tissue and bone: A current perspective.
Verma A, Rekhi B
Histol Histopathol 2017 Sep;32(9):861-877. Epub 2017 Feb 1 doi: 10.14670/HH-11-879. PMID: 28145559
Inflammatory Myofibroblastic Tumors: Current Update.
Surabhi VR, Chua S, Patel RP, Takahashi N, Lalwani N, Prasad SR
Radiol Clin North Am 2016 May;54(3):553-63. Epub 2016 Mar 12 doi: 10.1016/j.rcl.2015.12.005. PMID: 27153788

Therapy

Novel MEAF6-SUZ12 fusion in ossifying fibromyxoid tumor with unusual features.
Killian K, Leckey BD, Naous R, McGough RL, Surrey LF, John I
Genes Chromosomes Cancer 2021 Sep;60(9):631-634. Epub 2021 Apr 21 doi: 10.1002/gcc.22951. PMID: 33840146
Bleeding "sarcomatosis" as a rare presentation of vascular graft-related angiosarcoma: case report and review of the literature.
Presacco S, Agnes AL, Magalini S, Carbone A, Martini M, Agnes A
BMC Surg 2020 Nov 20;20(1):291. doi: 10.1186/s12893-020-00966-7. PMID: 33218319Free PMC Article
Dermatofibrosarcoma protuberans - the use of neoadjuvant imatinib for treatment of an uncommon breast malignancy: a case report.
McGee MW, Boukhar SA, Monga V, Weigel R, Phadke SD
J Med Case Rep 2019 Dec 19;13(1):374. doi: 10.1186/s13256-019-2316-0. PMID: 31852518Free PMC Article
Severe osteolysis and soft tissue mass around total hip arthroplasty: description of four cases and review of the literature with respect to clinico-radiographic and pathologic differential diagnosis.
Fabbri N, Rustemi E, Masetti C, Kreshak J, Gambarotti M, Vanel D, Toni A, Mercuri M
Eur J Radiol 2011 Jan;77(1):43-50. Epub 2010 Oct 8 doi: 10.1016/j.ejrad.2010.08.015. PMID: 20934822
Retroperitoneal soft tissue sarcomas: a review of the literature.
Van Dam PA, Lowe DG, McKenzie-Gray B, Shepherd JH
Obstet Gynecol Surv 1990 Oct;45(10):670-82. doi: 10.1097/00006254-199010000-00004. PMID: 2234702

Prognosis

Updates on WHO classification for small round cell tumors: Ewing sarcoma vs. everything else.
Dehner CA, Lazar AJ, Chrisinger JSA
Hum Pathol 2024 May;147:101-113. Epub 2024 Jan 26 doi: 10.1016/j.humpath.2024.01.007. PMID: 38280658
Ossifying fibromyxoid tumor: morphology, genetics, and differential diagnosis.
Schneider N, Fisher C, Thway K
Ann Diagn Pathol 2016 Feb;20:52-8. Epub 2015 Dec 2 doi: 10.1016/j.anndiagpath.2015.11.002. PMID: 26732302
Vascular tumors of bone.
Hart JL, Edgar MA, Gardner JM
Semin Diagn Pathol 2014 Jan;31(1):30-8. Epub 2014 Jan 7 doi: 10.1053/j.semdp.2014.01.003. PMID: 24680180
Smooth muscle tumors of soft tissue and non-uterine viscera: biology and prognosis.
Miettinen M
Mod Pathol 2014 Jan;27 Suppl 1(Suppl 1):S17-29. doi: 10.1038/modpathol.2013.178. PMID: 24384850Free PMC Article
Soft tissue tumors of uncertain origin.
Alaggio R, Coffin CM, Vargas SO
Pediatr Dev Pathol 2012;15(1 Suppl):267-305. doi: 10.2350/11-08-1079-PB.1. PMID: 22420732

Clinical prediction guides

Updates on WHO classification for small round cell tumors: Ewing sarcoma vs. everything else.
Dehner CA, Lazar AJ, Chrisinger JSA
Hum Pathol 2024 May;147:101-113. Epub 2024 Jan 26 doi: 10.1016/j.humpath.2024.01.007. PMID: 38280658
The management of deep-seated, lowgrade lipomatous lesions.
Al-Ani Z, Fernando M, Wilkinson V, Kotnis N
Br J Radiol 2018 Jun;91(1086):20170725. Epub 2018 Jan 17 doi: 10.1259/bjr.20170725. PMID: 29303371
Solitary, multiple, benign, atypical, or malignant: the "Granular Cell Tumor" puzzle.
Machado I, Cruz J, Lavernia J, Llombart-Bosch A
Virchows Arch 2016 May;468(5):527-38. Epub 2015 Dec 5 doi: 10.1007/s00428-015-1877-6. PMID: 26637199
Hemangioblastoma of pelvic cavity: report of a case and review of literature.
Shi H, Li H, Zhen T, Zhang F, Han A
Int J Clin Exp Pathol 2014;7(10):7054-8. Epub 2014 Sep 15 PMID: 25400795Free PMC Article
Molecular pathology of soft tissue and bone tumors. A review.
Slominski A, Wortsman J, Carlson A, Mihm M, Nickoloff B, McClatchey K
Arch Pathol Lab Med 1999 Dec;123(12):1246-59. doi: 10.5858/1999-123-1246-MPOSTA. PMID: 10583931

Recent systematic reviews

NTRK Fusions Detection in Paediatric Sarcomas to Expand the Morphological Spectrum and Clinical Relevance of Selected Entities.
Nozzoli F, Lazar AJ, Castiglione F, Campanacci DA, Beltrami G, De Logu F, Caporalini C, Massi D, Roviello G
Pathol Oncol Res 2022;28:1610237. Epub 2022 Feb 28 doi: 10.3389/pore.2022.1610237. PMID: 35295613Free PMC Article

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