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Intimal sarcoma

MedGen UID:
317791
•Concept ID:
C1708550
•
Neoplastic Process
Synonyms: Intimal Sarcoma; intimal sarcoma; INTS
SNOMED CT: Intimal sarcoma (703661007)
 
Monarch Initiative: MONDO:0006255

Definition

A malignant neoplasm arising from the large blood vessels. It is characterized by the presence of tumor cells that grow within the lumen of the blood vessels. The intraluminal tumor growth may result in vascular obstruction and spread of tumor emboli to peripheral organs. The prognosis is usually poor. [from NCI]

Professional guidelines

PubMed

Differential diagnosis of heart tumours.
Matějková A, Fabián O
Cesk Patol 2020 Winter;56(1):18-25. PMID: 32393042
A comprehensive review on the diagnosis and management of intimal sarcoma of the pulmonary artery.
Assi T, Kattan J, Rassy E, Moussa T, Nassereddine H, Honore C, Adam J, Terrier P, Dumont S, Mir O, Le Cesne A
Crit Rev Oncol Hematol 2020 Mar;147:102889. Epub 2020 Jan 30 doi: 10.1016/j.critrevonc.2020.102889. PMID: 32035299
Pulmonary artery intimal sarcoma. Problems in the differential diagnosis.
Attinà D, Niro F, Tchouanté P, Mineo G, Russo V, Palazzini M, Galiè N, Fanti S, Lovato L, Zompatori M
Radiol Med 2013 Dec;118(8):1259-68. Epub 2013 Jun 25 doi: 10.1007/s11547-013-0943-x. PMID: 23801391

Recent clinical studies

Etiology

Primary cardiac sarcomas: A clinicopathologic study in a single institution with 25 years of experience with an emphasis on MDM2 expression and adjuvant therapy for prognosis.
Cho H, Song IH, Jo U, Jeong JS, Koo HJ, Yang DH, Jung SH, Song JS, Cho KJ
Cancer Med 2023 Aug;12(16):16815-16828. Epub 2023 Jul 3 doi: 10.1002/cam4.6303. PMID: 37395142Free PMC Article
Natural history and treatment efficacy in an ambispective case series of NTRK-rearranged mesenchymal tumors.
Dufresne A, Pissaloux D, Ngo C, Penel N, Le Cesne A, Macagno N, Vanacker H, Hénon C, Jean-Denis M, Rughoo K, Tirode F, Blay JY, Brahmi M
ESMO Open 2023 Apr;8(2):101202. Epub 2023 Apr 11 doi: 10.1016/j.esmoop.2023.101202. PMID: 37054503Free PMC Article
Primary sarcoma of the heart: case report and literature review.
Moeri-Schimmel R, Pras E, Desar I, Krol S, Braam P
J Cardiothorac Surg 2020 May 19;15(1):104. doi: 10.1186/s13019-020-01157-4. PMID: 32430055Free PMC Article
Pulmonary Artery Intimal Sarcoma versus Pulmonary Artery Thromboembolism: CT and Clinical Findings.
Kim C, Kim MY, Kang JW, Song JS, Lee KY, Kim SS
Korean J Radiol 2018 Jul-Aug;19(4):792-802. Epub 2018 Jun 14 doi: 10.3348/kjr.2018.19.4.792. PMID: 29962886Free PMC Article
Single-Center Experience with Intimal Sarcoma, an Ultra-Orphan, Commonly Fatal Mesenchymal Malignancy.
Van Dievel J, Sciot R, Delcroix M, Vandeweyer RO, Debiec-Rychter M, Dewaele B, Cornillie J, Van Cann T, Meyns B, Schöffski P
Oncol Res Treat 2017;40(6):353-359. Epub 2017 May 12 doi: 10.1159/000476036. PMID: 28501860

Diagnosis

PRAME immunohistochemistry in soft tissue tumors and mimics: a study of 350 cases highlighting its imperfect specificity but potentially useful diagnostic applications.
Cammareri C, Beltzung F, Michal M, Vanhersecke L, Coindre JM, Velasco V, Le Loarer F, Vergier B, Perret R
Virchows Arch 2023 Aug;483(2):145-156. Epub 2023 Jul 21 doi: 10.1007/s00428-023-03606-6. PMID: 37477762
GLI1 Immunohistochemistry Distinguishes Mesenchymal Neoplasms With GLI1 Alterations From Morphologic Mimics.
Parrack PH, Mariño-Enríquez A, Fletcher CDM, Hornick JL, Papke DJ Jr
Am J Surg Pathol 2023 Apr 1;47(4):453-460. Epub 2023 Jan 23 doi: 10.1097/PAS.0000000000002018. PMID: 36693363
Intimal Sarcoma of the Great Vessels.
Ropp AM, Burke AP, Kligerman SJ, Leb JS, Frazier AA
Radiographics 2021 Mar-Apr;41(2):361-379. doi: 10.1148/rg.2021200184. PMID: 33646906
A comprehensive review on the diagnosis and management of intimal sarcoma of the pulmonary artery.
Assi T, Kattan J, Rassy E, Moussa T, Nassereddine H, Honore C, Adam J, Terrier P, Dumont S, Mir O, Le Cesne A
Crit Rev Oncol Hematol 2020 Mar;147:102889. Epub 2020 Jan 30 doi: 10.1016/j.critrevonc.2020.102889. PMID: 32035299
Predictors of survival in malignant aortic tumors.
Vacirca A, Faggioli G, Pini R, Freyrie A, Indelicato G, Fenelli C, Bacchi Reggiani ML, Vasuri F, Pasquinelli G, Stella A, Gargiulo M
J Vasc Surg 2020 May;71(5):1771-1780. Epub 2019 Dec 17 doi: 10.1016/j.jvs.2019.09.030. PMID: 31862201

Therapy

Clinical Activity and Exploratory Resistance Mechanism of Milademetan, an MDM2 Inhibitor, in Intimal Sarcoma with MDM2 Amplification: An Open-Label Phase Ib/II Study.
Koyama T, Shimizu T, Kojima Y, Sudo K, Okuma HS, Shimoi T, Ichikawa H, Kohsaka S, Sadachi R, Hirakawa A, Yoshida A, Ando RM, Ueno T, Yanagaki M, Matsui N, Nakamura K, Yamamoto N, Yonemori K
Cancer Discov 2023 Aug 4;13(8):1814-1825. doi: 10.1158/2159-8290.CD-23-0419. PMID: 37369013
MDM2 for the practicing pathologist: a primer.
Menon AA, Deshpande V, Suster D
J Clin Pathol 2023 May;76(5):285-290. Epub 2023 Mar 10 doi: 10.1136/jcp-2022-208687. PMID: 36898827
Percutaneous intravenous catheter forceps biopsy in right atrial mass: two case reports and literature review.
Chang L, Gong C, Lu H, Liu Y, Kang L, Chen J, Wang L, Xu B
BMC Cardiovasc Disord 2022 Feb 20;22(1):63. doi: 10.1186/s12872-022-02507-x. PMID: 35184743Free PMC Article
Systemic treatments in MDM2 positive intimal sarcoma: A multicentre experience with anthracycline, gemcitabine, and pazopanib within the World Sarcoma Network.
Frezza AM, Assi T, Lo Vullo S, Ben-Ami E, Dufresne A, Yonemori K, Noguchi E, Siontis B, Ferraro R, Teterycz P, Duffaud F, Ravi V, Vincenzi B, Gelderblom H, Pantaleo MA, Baldi GG, Desar I, Fedenko A, Maki RG, Jones RL, Benjamin RS, Blay JY, Kawai A, Gounder M, Gronchi A, Le Cesne A, Mir O, Czarnecka AM, Schuetze S, Wagner AJ, Adam J, Barisella M, Sbaraglia M, Hornick JL, Meurgey A, Mariani L, Casali PG, Thornton K, Stacchiotti S
Cancer 2020 Jan 1;126(1):98-104. Epub 2019 Sep 19 doi: 10.1002/cncr.32508. PMID: 31536651Free PMC Article
Pulmonary artery intimal sarcoma. Problems in the differential diagnosis.
Attinà D, Niro F, Tchouanté P, Mineo G, Russo V, Palazzini M, Galiè N, Fanti S, Lovato L, Zompatori M
Radiol Med 2013 Dec;118(8):1259-68. Epub 2013 Jun 25 doi: 10.1007/s11547-013-0943-x. PMID: 23801391

Prognosis

Intimal Sarcoma with MDM2/CDK4 Amplification and p16 Overexpression: A Review of Histological Features in Primary Tumor and Xenograft, with Immunophenotype and Molecular Profiling.
Giner F, Machado I, Rubio-Martínez LA, López-Guerrero JA, Claramunt-Alonso R, Navarro S, Ferrández A, Mayordomo-Aranda E, Llombart-Bosch A
Int J Mol Sci 2023 Apr 19;24(8) doi: 10.3390/ijms24087535. PMID: 37108696Free PMC Article
Natural history and treatment efficacy in an ambispective case series of NTRK-rearranged mesenchymal tumors.
Dufresne A, Pissaloux D, Ngo C, Penel N, Le Cesne A, Macagno N, Vanacker H, Hénon C, Jean-Denis M, Rughoo K, Tirode F, Blay JY, Brahmi M
ESMO Open 2023 Apr;8(2):101202. Epub 2023 Apr 11 doi: 10.1016/j.esmoop.2023.101202. PMID: 37054503Free PMC Article
Intimal Sarcoma of the Great Vessels.
Ropp AM, Burke AP, Kligerman SJ, Leb JS, Frazier AA
Radiographics 2021 Mar-Apr;41(2):361-379. doi: 10.1148/rg.2021200184. PMID: 33646906
Predictors of survival in malignant aortic tumors.
Vacirca A, Faggioli G, Pini R, Freyrie A, Indelicato G, Fenelli C, Bacchi Reggiani ML, Vasuri F, Pasquinelli G, Stella A, Gargiulo M
J Vasc Surg 2020 May;71(5):1771-1780. Epub 2019 Dec 17 doi: 10.1016/j.jvs.2019.09.030. PMID: 31862201
Abdominal and pelvic tumors with musculoskeletal histology.
Dyrstad SW, Fowler AM, Jokerst CE, Baker JC, Hillen TJ, Menias C
Curr Probl Diagn Radiol 2014 Mar-Apr;43(2):68-79. doi: 10.1067/j.cpradiol.2013.11.001. PMID: 24629660

Clinical prediction guides

Clinical Activity and Exploratory Resistance Mechanism of Milademetan, an MDM2 Inhibitor, in Intimal Sarcoma with MDM2 Amplification: An Open-Label Phase Ib/II Study.
Koyama T, Shimizu T, Kojima Y, Sudo K, Okuma HS, Shimoi T, Ichikawa H, Kohsaka S, Sadachi R, Hirakawa A, Yoshida A, Ando RM, Ueno T, Yanagaki M, Matsui N, Nakamura K, Yamamoto N, Yonemori K
Cancer Discov 2023 Aug 4;13(8):1814-1825. doi: 10.1158/2159-8290.CD-23-0419. PMID: 37369013
Predictors of survival in malignant aortic tumors.
Vacirca A, Faggioli G, Pini R, Freyrie A, Indelicato G, Fenelli C, Bacchi Reggiani ML, Vasuri F, Pasquinelli G, Stella A, Gargiulo M
J Vasc Surg 2020 May;71(5):1771-1780. Epub 2019 Dec 17 doi: 10.1016/j.jvs.2019.09.030. PMID: 31862201
MDM2 dual-color in situ hybridization (DISH) aids the diagnosis of intimal sarcomas.
Jimbo N, Komatsu M, Itoh T, Hirose T
Cardiovasc Pathol 2019 Nov-Dec;43:107142. Epub 2019 Jul 16 doi: 10.1016/j.carpath.2019.07.001. PMID: 31442826
Intimal sarcoma of the pulmonary trunk showing broad intimal extension and focal chondrosarcomatous differentiation: an autopsy case.
Miyai K, Takeo H, Matsukuma S
Cardiovasc Pathol 2017 Nov-Dec;31:5-8. Epub 2017 Jul 8 doi: 10.1016/j.carpath.2017.07.002. PMID: 28797682
Intimal sarcoma is the most frequent primary cardiac sarcoma: clinicopathologic and molecular retrospective analysis of 100 primary cardiac sarcomas.
Neuville A, Collin F, Bruneval P, Parrens M, Thivolet F, Gomez-Brouchet A, Terrier P, de Montpreville VT, Le Gall F, Hostein I, Lagarde P, Chibon F, Coindre JM
Am J Surg Pathol 2014 Apr;38(4):461-9. doi: 10.1097/PAS.0000000000000184. PMID: 24625414

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