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Undifferentiated Soft Tissue Sarcoma

MedGen UID:
888062
•Concept ID:
C2188153
•
Neoplastic Process
Synonyms: Unclassified Soft Tissue Sarcoma; Undifferentiated Sarcoma; Undifferentiated/Unclassified Sarcoma; Undifferentiated/Unclassified Soft Tissue Sarcoma

Definition

A term that refers to a heterogeneous group of uncommon soft tissue sarcomas that do not show an identifiable line of differentiation using currently available technologies. This is a diagnosis of exclusion and includes undifferentiated pleomorphic sarcoma (also known as malignant fibrous histiocytoma), undifferentiated spindle cell sarcoma, undifferentiated round cell sarcoma, and undifferentiated epithelioid sarcoma. [from NCI]

Professional guidelines

PubMed

The combination of gemcitabine and nab-paclitaxel as a novel effective treatment strategy for undifferentiated soft-tissue sarcoma in a patient-derived orthotopic xenograft (PDOX) nude-mouse model.
Higuchi T, Kawaguchi K, Miyake K, Oshiro H, Zhang Z, Razmjooei S, Wangsiricharoen S, Igarashi K, Yamamoto N, Hayashi K, Kimura H, Miwa S, Nelson SD, Dry SM, Li Y, Chawla SP, Eilber FC, Singh SR, Tsuchiya H, Hoffman RM
Biomed Pharmacother 2019 Mar;111:835-840. Epub 2019 Jan 4 doi: 10.1016/j.biopha.2018.12.110. PMID: 30616082

Recent clinical studies

Etiology

Clinical, histopathological and molecular features of dedifferentiated melanomas: An EORTC Melanoma Group Retrospective Analysis.
Hench J, Mihic-Probst D, Agaimy A, Frank S, Meyer P, Hultschig C, Simi S, Alos L, Balamurugan T, Blokx W, Bosisio F, Cappellesso R, Griewank K, Hadaschik E, van Kempen LC, Kempf W, Lentini M, Mazzucchelli L, Rinaldi G, Rutkowski P, Schadendorf D, Schilling B, Szumera-Cieckiewicz A, van den Oord J, Mandalà M, Massi D; EORTC Melanoma Group
Eur J Cancer 2023 Jul;187:7-14. Epub 2023 Apr 2 doi: 10.1016/j.ejca.2023.03.032. PMID: 37098294
Mesenchymal loss of p53 alters stem cell capacity and models human soft tissue sarcoma traits.
Sorimachi Y, Kobayashi H, Shiozawa Y, Koide S, Nakato R, Shimizu Y, Okamura T, Shirahige K, Iwama A, Goda N, Takubo K, Takubo K
Stem Cell Reports 2023 May 9;18(5):1211-1226. Epub 2023 Apr 13 doi: 10.1016/j.stemcr.2023.03.009. PMID: 37059101Free PMC Article
Establishment and characterization of NCC-UPS4-C1: a novel cell line of undifferentiated pleomorphic sarcoma from a patient with Li-Fraumeni syndrome.
Ono T, Yoshimatsu Y, Noguchi R, Sin Y, Tsuchiya R, Akiyama T, Sugaya J, Fukushima S, Kojima N, Yoshida A, Kawai A, Kondo T
Hum Cell 2022 Mar;35(2):756-766. Epub 2022 Feb 3 doi: 10.1007/s13577-022-00671-y. PMID: 35118583
Individualized doxorubicin sensitivity testing of undifferentiated soft tissue sarcoma (USTS) in a patient-derived orthotopic xenograft (PDOX) model demonstrates large differences between patients.
Kawaguchi K, Igarashi K, Kiyuna T, Miyake K, Miyake M, Murakami T, Chmielowski B, Nelson SD, Russell TA, Dry SM, Li Y, Singh AS, Unno M, Eilber FC, Hoffman RM
Cell Cycle 2018;17(5):627-633. Epub 2018 Mar 29 doi: 10.1080/15384101.2017.1421876. PMID: 29384032Free PMC Article
Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas.
Ruymann FB, Grovas AC
Cancer Invest 2000;18(3):223-41. doi: 10.3109/07357900009031827. PMID: 10754991

Diagnosis

Clinical, histopathological and molecular features of dedifferentiated melanomas: An EORTC Melanoma Group Retrospective Analysis.
Hench J, Mihic-Probst D, Agaimy A, Frank S, Meyer P, Hultschig C, Simi S, Alos L, Balamurugan T, Blokx W, Bosisio F, Cappellesso R, Griewank K, Hadaschik E, van Kempen LC, Kempf W, Lentini M, Mazzucchelli L, Rinaldi G, Rutkowski P, Schadendorf D, Schilling B, Szumera-Cieckiewicz A, van den Oord J, Mandalà M, Massi D; EORTC Melanoma Group
Eur J Cancer 2023 Jul;187:7-14. Epub 2023 Apr 2 doi: 10.1016/j.ejca.2023.03.032. PMID: 37098294
Primary renal sarcoma with SS18::POU5F1 gene fusion.
Argani P, Matoso A, Gross JM, Zhang Y, SoRelle JA, Gagan J, Antonescu CR, Palsgrove D
Genes Chromosomes Cancer 2022 Sep;61(9):572-577. Epub 2022 May 19 doi: 10.1002/gcc.23053. PMID: 35521683Free PMC Article
Unravelling undifferentiated soft tissue sarcomas: insights from genomics.
Hames-Fathi S, Nottley SWG, Pillay N
Histopathology 2022 Jan;80(1):109-121. doi: 10.1111/his.14446. PMID: 34958500
Challenging epithelioid mesenchymal neoplasms: mimics and traps.
Cacciatore M, Dei Tos AP
Pathology 2014 Feb;46(2):126-34. doi: 10.1097/PAT.0000000000000063. PMID: 24384587
Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas.
Ruymann FB, Grovas AC
Cancer Invest 2000;18(3):223-41. doi: 10.3109/07357900009031827. PMID: 10754991

Therapy

Combination of oral recombinant methioninase and decitabine arrests a chemotherapy-resistant undifferentiated soft-tissue sarcoma patient-derived orthotopic xenograft mouse model.
Higuchi T, Han Q, Miyake K, Oshiro H, Sugisawa N, Tan Y, Yamamoto N, Hayashi K, Kimura H, Miwa S, Igarashi K, Bouvet M, Singh SR, Tsuchiya H, Hoffman RM
Biochem Biophys Res Commun 2020 Feb 26;523(1):135-139. Epub 2019 Dec 12 doi: 10.1016/j.bbrc.2019.12.024. PMID: 31839218
The combination of olaratumab with gemcitabine and docetaxel arrests a chemotherapy-resistant undifferentiated soft-tissue sarcoma in a patient-derived orthotopic xenograft mouse model.
Higuchi T, Miyake K, Sugisawa N, Oshiro H, Zhang Z, Razmjooei S, Yamamoto N, Hayashi K, Kimura H, Miwa S, Igarashi K, Bouvet M, Singh SR, Tsuchiya H, Hoffman RM
Cancer Chemother Pharmacol 2019 Jun;83(6):1075-1082. Epub 2019 Mar 30 doi: 10.1007/s00280-019-03824-3. PMID: 30929053
The combination of gemcitabine and nab-paclitaxel as a novel effective treatment strategy for undifferentiated soft-tissue sarcoma in a patient-derived orthotopic xenograft (PDOX) nude-mouse model.
Higuchi T, Kawaguchi K, Miyake K, Oshiro H, Zhang Z, Razmjooei S, Wangsiricharoen S, Igarashi K, Yamamoto N, Hayashi K, Kimura H, Miwa S, Nelson SD, Dry SM, Li Y, Chawla SP, Eilber FC, Singh SR, Tsuchiya H, Hoffman RM
Biomed Pharmacother 2019 Mar;111:835-840. Epub 2019 Jan 4 doi: 10.1016/j.biopha.2018.12.110. PMID: 30616082
Individualized doxorubicin sensitivity testing of undifferentiated soft tissue sarcoma (USTS) in a patient-derived orthotopic xenograft (PDOX) model demonstrates large differences between patients.
Kawaguchi K, Igarashi K, Kiyuna T, Miyake K, Miyake M, Murakami T, Chmielowski B, Nelson SD, Russell TA, Dry SM, Li Y, Singh AS, Unno M, Eilber FC, Hoffman RM
Cell Cycle 2018;17(5):627-633. Epub 2018 Mar 29 doi: 10.1080/15384101.2017.1421876. PMID: 29384032Free PMC Article
Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas.
Ruymann FB, Grovas AC
Cancer Invest 2000;18(3):223-41. doi: 10.3109/07357900009031827. PMID: 10754991

Prognosis

Mesenchymal loss of p53 alters stem cell capacity and models human soft tissue sarcoma traits.
Sorimachi Y, Kobayashi H, Shiozawa Y, Koide S, Nakato R, Shimizu Y, Okamura T, Shirahige K, Iwama A, Goda N, Takubo K, Takubo K
Stem Cell Reports 2023 May 9;18(5):1211-1226. Epub 2023 Apr 13 doi: 10.1016/j.stemcr.2023.03.009. PMID: 37059101Free PMC Article
Glypican 3 expression in tumors with loss of SMARCB1/INI1 protein expression.
Kohashi K, Nakatsura T, Kinoshita Y, Yamamoto H, Yamada Y, Tajiri T, Taguchi T, Iwamoto Y, Oda Y
Hum Pathol 2013 Apr;44(4):526-33. Epub 2012 Oct 16 doi: 10.1016/j.humpath.2012.06.014. PMID: 23084579
Molecular characterization of an EWSR1-POU5F1 fusion associated with a t(6;22) in an undifferentiated soft tissue sarcoma.
Deng FM, Galvan K, de la Roza G, Zhang S, Souid AK, Stein CK
Cancer Genet 2011 Aug;204(8):423-9. doi: 10.1016/j.cancergen.2011.05.006. PMID: 21962892
Pediatric undifferentiated sarcoma of the soft tissues: a clinicopathologic study.
Somers GR, Gupta AA, Doria AS, Ho M, Pereira C, Shago M, Thorner PS, Zielenska M
Pediatr Dev Pathol 2006 Mar-Apr;9(2):132-42. Epub 2006 Jun 16 doi: 10.2350/08-05-0098.1. PMID: 16822084
Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas.
Ruymann FB, Grovas AC
Cancer Invest 2000;18(3):223-41. doi: 10.3109/07357900009031827. PMID: 10754991

Clinical prediction guides

Clinical, histopathological and molecular features of dedifferentiated melanomas: An EORTC Melanoma Group Retrospective Analysis.
Hench J, Mihic-Probst D, Agaimy A, Frank S, Meyer P, Hultschig C, Simi S, Alos L, Balamurugan T, Blokx W, Bosisio F, Cappellesso R, Griewank K, Hadaschik E, van Kempen LC, Kempf W, Lentini M, Mazzucchelli L, Rinaldi G, Rutkowski P, Schadendorf D, Schilling B, Szumera-Cieckiewicz A, van den Oord J, Mandalà M, Massi D; EORTC Melanoma Group
Eur J Cancer 2023 Jul;187:7-14. Epub 2023 Apr 2 doi: 10.1016/j.ejca.2023.03.032. PMID: 37098294
Forearm reconstruction by induced-membrane technique after sarcoma resection in children: technique and functional outcome in three cases.
Rougereau G, Marty-Diloy T, Pietton R, Koneazny C, Fitoussi F, Vialle R, Mary P, Langlais T
Hand Surg Rehabil 2021 Dec;40(6):799-803. Epub 2021 Jun 23 doi: 10.1016/j.hansur.2021.06.007. PMID: 34171528
Challenging epithelioid mesenchymal neoplasms: mimics and traps.
Cacciatore M, Dei Tos AP
Pathology 2014 Feb;46(2):126-34. doi: 10.1097/PAT.0000000000000063. PMID: 24384587
Cyclophosphamide dose escalation in combination with vincristine and actinomycin-D (VAC) in gross residual sarcoma. A pilot study without hematopoietic growth factor support evaluating toxicity and response.
Ruymann FB, Vietti T, Gehan E, Wiener E, Wharam M, Newton WA Jr, Maurer H
J Pediatr Hematol Oncol 1995 Nov;17(4):331-7. doi: 10.1097/00043426-199511000-00009. PMID: 7583389
Soft-tissue sarcoma of undetermined histogenesis: an ultrastructural study.
Dardick I, Ho SP, McCaughey WT
Arch Pathol Lab Med 1981 Apr;105(4):214-7. PMID: 6260060

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