Myxoid type and non-myxoid type of intimal sarcoma in large vessels and heart: review of histological and genetic profiles of 20 cases

Yuichi Yamada, Izumi Kinoshita, Yoshiko Miyazaki, Yuki Tateishi, Yusuke Kuboyama, Takeshi Iwasaki, Kenichi Kouhashi, Hidetaka Yamamoto, Shin Ishihara, Yu Toda, Yoshihiro Ito, Yosuke Susuki, Kengo Kawaguchi, Mikiko Hashisako, Yui Yamada-Nozaki, Daisuke Kiyozawa, Taro Mori, Takeo Yamamoto, Kenji Tsuchihashi, Kazumi KuriwakiMunenori Mukai, Masataka Kawai, Keiko Suzuki, Hirotake Nishimura, Kenji Bando, Junya Masumoto, Mana Fukushima, Junichi Motoshita, Hiroki Mori, Akira Shiose, Yoshinao Oda

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6 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)919-925
Number of pages7
JournalVirchows Archiv
Issue number4
Publication statusPublished - Apr 2022

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Molecular Biology
  • Cell Biology


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