Myxoid type and non-myxoid type of intimal sarcoma in large vessels and heart: review of histological and genetic profiles of 20 cases.
Aorta
Heart
Intimal sarcoma
Undifferentiated pleomorphic sarcoma
Vena cava
Journal
Virchows Archiv : an international journal of pathology
ISSN: 1432-2307
Titre abrégé: Virchows Arch
Pays: Germany
ID NLM: 9423843
Informations de publication
Date de publication:
Apr 2022
Apr 2022
Historique:
received:
19
08
2021
accepted:
13
01
2022
revised:
21
12
2021
pubmed:
17
2
2022
medline:
26
4
2022
entrez:
16
2
2022
Statut:
ppublish
Résumé
Intimal sarcoma is one of the most common and well-known primary malignant neoplasms of the aorta and heart. The authors reviewed cases of intimal sarcoma from histological, immunohistochemical and genetic perspectives. Twenty cases of intimal sarcoma were retrieved. Immunohistochemistry and FISH of MDM2 and PDGFRA genes were performed. All 20 tumours were composed of spindle-shaped, stellate, oval or polygonal tumour cells with irregular hyperchromatic nuclei arranged in a haphazard pattern, accompanied by nuclear pleomorphism and frequent mitotic figures. Other histological findings were as follows: abnormal mitosis in 10 cases (50%), necrosis in 15 cases (75%), myxoid stroma in 12 cases (60%), cartilaginous formation in 1 case (5%), haemorrhage in 12 cases (60%) and fibrinous deposition in 14 cases (70%). The tumours were positive for MDM2 in 16 cases (80%), ERG in 4 cases (20%), alpha-smooth muscle actin in 6 cases (30%), desmin in 5 cases (25%) and AE1/AE3 in 4 cases (20%). Immunohistochemical positivity was focal in each case. Loss of H3K27me3 expression was noted in 2 cases (10%). MDM2 and PDGFRA gene amplifications were detected in 11 cases (55%) and 1 case (5%), respectively. Fisher's exact test revealed a significant correlation between MDM2 gene amplification and myxoid stroma (p = 0.0194). No parameters showed any association with the anatomical location of the tumours. It was suggested that myxoid histology of intimal sarcoma may be associated with MDM2 gene amplification and that intimal sarcoma may be divided into myxoid and non-myxoid types.
Identifiants
pubmed: 35171325
doi: 10.1007/s00428-022-03293-9
pii: 10.1007/s00428-022-03293-9
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
919-925Subventions
Organisme : Japan Society for the Promotion of Science
ID : 19H03444
Informations de copyright
© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.
Références
Bode-Lesniewska B, Deblec-Rychter M, Tavora F (2020) Intimal sarcoma. The WHO Classification of Tumours Editorial Board, eds. WHO Classification of Tumours. Lyon, France: IARC Press; 315–317
Neuville A, Collin F, Bruneval P et al (2014) Intimal sarcoma is the most frequent primary cardiac sarcoma: clinicopathologic and molecular retrospective analysis of 100 primary cardiac sarcomas. Am J Surg Pathol 38:461–469
doi: 10.1097/PAS.0000000000000184
Keel SB, Bacha E, Mark EJ et al (1999) Primary pulmonary sarcoma: a clinicopathologic study of 26 cases. Mod Pathol 12:1124–1131
Sebenik M, Ricci A, DiPasquale B et al (2005) Undifferentiated intimal sarcoma of large systemic blood vessels: report of 14 cases with immunohistochemical profile and review of the literature. Am J Surg Pathol 29:1184–1193
doi: 10.1097/01.pas.0000159774.70288.7d
Staats P, Tavora F, Burke AP (2014) Intimal sarcomas of the aorta and iliofemoral arteries: a clinicopathological study of 26 cases. Pathology 46:596–603
doi: 10.1097/PAT.0000000000000182
Burke AP, Virmani R (1993) Sarcomas of the great vessels. A clinicopathologic study. Cancer. 71:1761–73
doi: 10.1002/1097-0142(19930301)71:5<1761::AID-CNCR2820710510>3.0.CO;2-7
Ito Y, Maeda D, Yoshida M et al (2017) Cardiac intimal sarcoma with PDGFRβ mutation and co-amplification of PDGFRα and MDM2: an autopsy case analyzed by whole-exome sequencing. Virchows Arch 471:423–428
doi: 10.1007/s00428-017-2135-x
Jimbo N, Komatsu M, Itoh T et al (2019) MDM2 dual-color in situ hybridization (DISH) aids the diagnosis of intimal sarcomas. Cardiovasc Pathol. 43:107142
doi: 10.1016/j.carpath.2019.07.001
Bode-Lesniewska B, Zhao J, Speel EJ et al (2001) Gains of 12q13-14 and overexpression of mdm2 are frequent findings in intimal sarcomas of the pulmonary artery. Virchows Arch 438:57–65
doi: 10.1007/s004280000313
Sirvent N, Coindre JM, Maire G et al (2007) Detection of MDM2-CDK4 amplification by fluorescence in situ hybridization in 200 paraffin-embedded tumor samples: utility in diagnosing adipocytic lesions and comparison with immunohistochemistry and real-time PCR. Am J Surg Pathol. 31:1476–89
doi: 10.1097/PAS.0b013e3180581fff
Miettinen M, Wang ZF, Paetau A et al (2011) ERG transcription factor as an immunohistochemical marker for vascular endothelial tumors and prostatic carcinoma. Am J Surg Pathol 35:432–441
doi: 10.1097/PAS.0b013e318206b67b
Prieto-Granada CN, Wiesner T, Messina JL et al (2016) Loss of H3K27me3 expression is a highly sensitive marker for sporadic and radiation-induced MPNST. Am J Surg Pathol 40:479–489
doi: 10.1097/PAS.0000000000000564