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Undifferentiated Spindle Cell Sarcoma

MedGen UID:
887831
•Concept ID:
C4048234
•
Neoplastic Process
Synonym: Undifferentiated spindle cell sarcoma
SNOMED CT: Undifferentiated spindle cell sarcoma (1290806003); Undifferentiated spindle cell sarcoma (1290807007)

Definition

An undifferentiated soft tissue sarcoma characterized by the presence of a malignant spindle cell infiltrate with amphophilic or palely eosinophilic cytoplasm. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUndifferentiated Spindle Cell Sarcoma

Professional guidelines

PubMed

Spermatocytic tumor of testis: A case series of 26 cases elucidating unusual patterns with diagnostic and treatment dilemmas.
Rabade K, Panjwani PK, Menon S, Prakash G, Pal M, Bakshi G, Desai S
J Cancer Res Ther 2022 Dec;18(Supplement):S449-S454. doi: 10.4103/jcrt.JCRT_1727_20. PMID: 36511002

Recent clinical studies

Etiology

Malignant peripheral nerve sheath tumor (MPNST) and MPNST-like entities are defined by a specific DNA methylation profile in pediatric and juvenile population.
Patrizi S, Miele E, Falcone L, Vallese S, Rossi S, Barresi S, Giovannoni I, Pedace L, Nardini C, Masier I, Abballe L, Cacchione A, Russo I, Di Giannatale A, Di Ruscio V, Salgado CM, Mastronuzzi A, Ciolfi A, Tartaglia M, Milano GM, Locatelli F, Alaggio R
Clin Epigenetics 2024 Jan 4;16(1):9. doi: 10.1186/s13148-023-01621-7. PMID: 38178234Free PMC Article
ARID1A deficient undifferentiated spindle cell and rhabdoid sarcoma of the prostate: report of a unique case with emphasis on diagnostic implications.
Xu W, Dong H, Ru G, Zhao M
Diagn Pathol 2022 Feb 6;17(1):22. doi: 10.1186/s13000-022-01198-4. PMID: 35125107Free PMC Article
Temozolomide regresses a doxorubicin-resistant undifferentiated spindle-cell sarcoma patient-derived orthotopic xenograft (PDOX): precision-oncology nude-mouse model matching the patient with effective therapy.
Igarashi K, Kawaguchi K, Kiyuna T, Miyake K, Miyake M, Li Y, Nelson SD, Dry SM, Singh AS, Elliott IA, Russell TA, Eckardt MA, Yamamoto N, Hayashi K, Kimura H, Miwa S, Tsuchiya H, Eilber FC, Hoffman RM
J Cell Biochem 2018 Aug;119(8):6598-6603. Epub 2018 May 8 doi: 10.1002/jcb.26792. PMID: 29737543
High-grade myxoinflammatory fibroblastic sarcoma: a report of 23 cases.
Michal M, Kazakov DV, Hadravský L, Kinkor Z, Kuroda N, Michal M
Ann Diagn Pathol 2015 Jun;19(3):157-63. Epub 2015 Apr 6 doi: 10.1016/j.anndiagpath.2015.03.012. PMID: 25886867
Identification of an MLL4-GPS2 fusion as an oncogenic driver of undifferentiated spindle cell sarcoma in a child.
O'Meara E, Stack D, Phelan S, McDonagh N, Kelly L, Sciot R, Debiec-Rychter M, Morris T, Cochrane D, Sorensen P, O'Sullivan MJ
Genes Chromosomes Cancer 2014 Dec;53(12):991-8. Epub 2014 Aug 19 doi: 10.1002/gcc.22208. PMID: 25139254

Diagnosis

Malignant peripheral nerve sheath tumor (MPNST) and MPNST-like entities are defined by a specific DNA methylation profile in pediatric and juvenile population.
Patrizi S, Miele E, Falcone L, Vallese S, Rossi S, Barresi S, Giovannoni I, Pedace L, Nardini C, Masier I, Abballe L, Cacchione A, Russo I, Di Giannatale A, Di Ruscio V, Salgado CM, Mastronuzzi A, Ciolfi A, Tartaglia M, Milano GM, Locatelli F, Alaggio R
Clin Epigenetics 2024 Jan 4;16(1):9. doi: 10.1186/s13148-023-01621-7. PMID: 38178234Free PMC Article
ARID1A deficient undifferentiated spindle cell and rhabdoid sarcoma of the prostate: report of a unique case with emphasis on diagnostic implications.
Xu W, Dong H, Ru G, Zhao M
Diagn Pathol 2022 Feb 6;17(1):22. doi: 10.1186/s13000-022-01198-4. PMID: 35125107Free PMC Article
BCOR-CCNB3 fusions are frequent in undifferentiated sarcomas of male children.
Peters TL, Kumar V, Polikepahad S, Lin FY, Sarabia SF, Liang Y, Wang WL, Lazar AJ, Doddapaneni H, Chao H, Muzny DM, Wheeler DA, Okcu MF, Plon SE, Hicks MJ, López-Terrada D, Parsons DW, Roy A
Mod Pathol 2015 Apr;28(4):575-86. Epub 2014 Oct 31 doi: 10.1038/modpathol.2014.139. PMID: 25360585Free PMC Article
Metastatic undifferentiated spindle cell sarcoma of the thyroid gland evaluated by 18F-FDG PET/CT.
Treglia G, Bongiovanni M, Paone G, Ceriani L, Giovanella L
Clin Nucl Med 2015 Mar;40(3):e208-10. doi: 10.1097/RLU.0000000000000552. PMID: 25140545
Primary undifferentiated spindle-cell sarcoma of sella turcica: successful treatment with adjuvant temozolomide.
Sareen P, Chhabra L, Trivedi N
BMJ Case Rep 2013 May 27;2013 doi: 10.1136/bcr-2013-009934. PMID: 23715844Free PMC Article

Therapy

Undifferentiated sarcoma of the pericardium after radiation therapy for Hodgkin's lymphoma.
Fomin VV, Kogan EA, Chichkova NV, Komarov RN, Fominykh EV, Shchedrina IS, Morosova NS, Karseladze AI
Ter Arkh 2018 Aug 27;90(8):69-73. doi: 10.26442/terarkh201890869-73. PMID: 30701941
Temozolomide regresses a doxorubicin-resistant undifferentiated spindle-cell sarcoma patient-derived orthotopic xenograft (PDOX): precision-oncology nude-mouse model matching the patient with effective therapy.
Igarashi K, Kawaguchi K, Kiyuna T, Miyake K, Miyake M, Li Y, Nelson SD, Dry SM, Singh AS, Elliott IA, Russell TA, Eckardt MA, Yamamoto N, Hayashi K, Kimura H, Miwa S, Tsuchiya H, Eilber FC, Hoffman RM
J Cell Biochem 2018 Aug;119(8):6598-6603. Epub 2018 May 8 doi: 10.1002/jcb.26792. PMID: 29737543
Recombinant methioninase in combination with doxorubicin (DOX) overcomes first-line DOX resistance in a patient-derived orthotopic xenograft nude-mouse model of undifferentiated spindle-cell sarcoma.
Igarashi K, Kawaguchi K, Li S, Han Q, Tan Y, Murakami T, Kiyuna T, Miyake K, Miyake M, Singh AS, Eckardt MA, Nelson SD, Russell TA, Dry SM, Li Y, Yamamoto N, Hayashi K, Kimura H, Miwa S, Tsuchiya H, Singh SR, Eilber FC, Hoffman RM
Cancer Lett 2018 Mar 28;417:168-173. Epub 2018 Jan 3 doi: 10.1016/j.canlet.2017.12.028. PMID: 29306021
High Efficacy of Pazopanib on an Undifferentiated Spindle-Cell Sarcoma Resistant to First-Line Therapy Is Identified With a Patient-Derived Orthotopic Xenograft (PDOX) Nude Mouse Model.
Igarashi K, Kawaguchi K, Murakami T, Kiyuna T, Miyake K, Singh AS, Nelson SD, Dry SM, Li Y, Yamamoto N, Hayashi K, Kimura H, Miwa S, Tsuchiya H, Eilber FC, Hoffman RM
J Cell Biochem 2017 Sep;118(9):2739-2743. Epub 2017 Apr 25 doi: 10.1002/jcb.25923. PMID: 28176365
Primary undifferentiated spindle-cell sarcoma of sella turcica: successful treatment with adjuvant temozolomide.
Sareen P, Chhabra L, Trivedi N
BMJ Case Rep 2013 May 27;2013 doi: 10.1136/bcr-2013-009934. PMID: 23715844Free PMC Article

Prognosis

Malignant peripheral nerve sheath tumor (MPNST) and MPNST-like entities are defined by a specific DNA methylation profile in pediatric and juvenile population.
Patrizi S, Miele E, Falcone L, Vallese S, Rossi S, Barresi S, Giovannoni I, Pedace L, Nardini C, Masier I, Abballe L, Cacchione A, Russo I, Di Giannatale A, Di Ruscio V, Salgado CM, Mastronuzzi A, Ciolfi A, Tartaglia M, Milano GM, Locatelli F, Alaggio R
Clin Epigenetics 2024 Jan 4;16(1):9. doi: 10.1186/s13148-023-01621-7. PMID: 38178234Free PMC Article
ARID1A deficient undifferentiated spindle cell and rhabdoid sarcoma of the prostate: report of a unique case with emphasis on diagnostic implications.
Xu W, Dong H, Ru G, Zhao M
Diagn Pathol 2022 Feb 6;17(1):22. doi: 10.1186/s13000-022-01198-4. PMID: 35125107Free PMC Article
The novel finding of an FGFR1::TACC1 fusion in an undifferentiated spindle cell sarcoma of soft tissue with aggressive clinical course.
Yau DT, Lacambra MD, Chow C, To KF
Genes Chromosomes Cancer 2022 Apr;61(4):206-211. doi: 10.1002/gcc.23024. PMID: 35064610
Undifferentiated sarcoma of the pericardium after radiation therapy for Hodgkin's lymphoma.
Fomin VV, Kogan EA, Chichkova NV, Komarov RN, Fominykh EV, Shchedrina IS, Morosova NS, Karseladze AI
Ter Arkh 2018 Aug 27;90(8):69-73. doi: 10.26442/terarkh201890869-73. PMID: 30701941
High-grade myxoinflammatory fibroblastic sarcoma: a report of 23 cases.
Michal M, Kazakov DV, Hadravský L, Kinkor Z, Kuroda N, Michal M
Ann Diagn Pathol 2015 Jun;19(3):157-63. Epub 2015 Apr 6 doi: 10.1016/j.anndiagpath.2015.03.012. PMID: 25886867

Clinical prediction guides

Rapid but nondurable response of a BRAF exon 15 double-mutated spindle cell sarcoma to a combination of BRAF and MEK inhibitors.
Sinichenkova K, Sidorov I, Kriventsova N, Konovalov D, Abasov R, Usman N, Karachunskiy A, Novichkova G, Litvinov D, Druy A
Oncotarget 2024 Jul 17;15:493-500. doi: 10.18632/oncotarget.28606. PMID: 39018206Free PMC Article
ARID1A deficient undifferentiated spindle cell and rhabdoid sarcoma of the prostate: report of a unique case with emphasis on diagnostic implications.
Xu W, Dong H, Ru G, Zhao M
Diagn Pathol 2022 Feb 6;17(1):22. doi: 10.1186/s13000-022-01198-4. PMID: 35125107Free PMC Article
The novel finding of an FGFR1::TACC1 fusion in an undifferentiated spindle cell sarcoma of soft tissue with aggressive clinical course.
Yau DT, Lacambra MD, Chow C, To KF
Genes Chromosomes Cancer 2022 Apr;61(4):206-211. doi: 10.1002/gcc.23024. PMID: 35064610
Primary Sarcomas of the Larynx: A Single Institutional Experience with Ten Cases.
Mantilla JG, Xu H, Ricciotti RW
Head Neck Pathol 2020 Sep;14(3):707-714. Epub 2019 Dec 7 doi: 10.1007/s12105-019-01106-1. PMID: 31813100Free PMC Article
Identifying actionable variants using next generation sequencing in patients with a historical diagnosis of undifferentiated pleomorphic sarcoma.
Lewin J, Garg S, Lau BY, Dickson BC, Traub F, Gokgoz N, Griffin AM, Ferguson PC, Andrulis IL, Sim HW, Kamel-Reid S, Stockley TL, Siu LL, Wunder JS, Razak ARA
Int J Cancer 2018 Jan 1;142(1):57-65. Epub 2017 Oct 9 doi: 10.1002/ijc.31039. PMID: 28891048

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