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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); Intimal sarcoma (1290865001)
 
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

Uncommon and peculiar soft tissue sarcomas: Multidisciplinary review and practical recommendations for diagnosis and treatment. Spanish group for Sarcoma research (GEIS - GROUP). Part I.
Martínez-Trufero J, Cruz Jurado J, Gómez-Mateo MC, Bernabeu D, Floría LJ, Lavernia J, Sebio A, García Del Muro X, Álvarez R, Correa R, Hernández-León CN, Marquina G, Hindi N, Redondo A, Martínez V, Asencio JM, Mata C, Valverde Morales CM, Martin-Broto J
Cancer Treat Rev 2021 Sep;99:102259. Epub 2021 Jul 11 doi: 10.1016/j.ctrv.2021.102259. PMID: 34311246
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
Pulmonary Valve Intimal Sarcoma.
Al-Azzam FM, Said SM, Schaff HV
Ann Thorac Surg 2016 Sep;102(3):e221-e222. doi: 10.1016/j.athoracsur.2016.01.021. PMID: 27549548
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

Diagnosis

Sarcoma of the Lung and Mediastinum.
Yoshida KI, Yoshida A
Surg Pathol Clin 2024 Jun;17(2):243-255. Epub 2023 Dec 15 doi: 10.1016/j.path.2023.11.008. PMID: 38692808
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
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

Therapy

Single-Cell Profiling of Sarcomas from Archival Tissue Reveals Programs Associated with Resistance to Immune Checkpoint Blockade.
Luthria K, Shah P, Caldwell B, Melms JC, Abuzaid S, Jakubikova V, Brodtman DZ, Bose S, Amin AD, Ho P, Biermann J, Tagore S, Ingham M, Schwartz GK, Izar B
Clin Cancer Res 2024 Oct 1;30(19):4530-4541. doi: 10.1158/1078-0432.CCR-23-2976. PMID: 39083415Free PMC Article
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
Intimal sarcoma of the pulmonary artery without (18)F-fluorodeoxyglucose uptake.
Masuda S, Itagaki K, Naganuma M, Suzuki N, Kurotaki H, Nagaya K
Asian Cardiovasc Thorac Ann 2020 Jun;28(5):282-285. Epub 2020 Jun 2 doi: 10.1177/0218492320932746. PMID: 32483975
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

Prognosis

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
Intimal spindle cell sarcoma masquerading as adult-onset symptomatic pulmonic stenosis: a case report and review of the literature.
Manmadhan A, Malhotra SP, Weinberg CR, Reyentovich A, Latson LA Jr, Bhatla P, Saric M
J Cardiothorac Surg 2017 Oct 30;12(1):93. doi: 10.1186/s13019-017-0654-9. PMID: 29084562Free PMC Article
Pulmonary Valve Intimal Sarcoma.
Al-Azzam FM, Said SM, Schaff HV
Ann Thorac Surg 2016 Sep;102(3):e221-e222. doi: 10.1016/j.athoracsur.2016.01.021. PMID: 27549548
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
Intimal sarcoma of the pulmonary artery without (18)F-fluorodeoxyglucose uptake.
Masuda S, Itagaki K, Naganuma M, Suzuki N, Kurotaki H, Nagaya K
Asian Cardiovasc Thorac Ann 2020 Jun;28(5):282-285. Epub 2020 Jun 2 doi: 10.1177/0218492320932746. PMID: 32483975
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
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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