Difference of natural history between sporadic, TSC-related, and LAM-related AML.

2021 Fiscal Year Final Research Report

• Project/Area Number: 18K07645
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Review Section: Basic Section 52040:Radiological sciences-related
• Research Institution: Juntendo University
• Principal Investigator: Kuwatsuru Ryohei 順天堂大学, 医学部, 教授 (40225313)
• Co-Investigator(Kenkyū-buntansha): 加藤 仁美 順天堂大学, 医学部, 助教 (10621732) ; 岡田 慎悟 順天堂大学, 医学部, 助教 (30773303)
• Project Period (FY): 2018-04-01 – 2022-03-31
• Keywords: 腎血管筋脂肪腫 / 結節性硬化症 / リンパ脈管筋腫症 / 破裂 / 腎動脈塞栓術

Outline of Final Research Achievements

The total cases who received transcatheter arterial embolization (TAE) is 139 since July 2010 to March 2022. Both prophylactic TAE and TAE for ruptured angiomyolipoma (AML) were included. TAE was performed once in 62 cases and 2 times in 7 cases (10%) in sporadic AML patients. TAE was performed once in 24 cases and more than 2 times in 11 cases (31%) in Tuberous sclerosis（TSC) associated AML patients. TAE was performed once in 23 cases and more than 2 times in 12 cases (34%) in lymphangioleiomyomatosis (LAM) associated AML patients. As a result, TSC or LAM associated AML patients needs more than 3 times higher TAEs than sporadic AML.

Free Research Field

放射線診断学

Academic Significance and Societal Importance of the Research Achievements

本研究では、腎血管筋脂肪腫を、① 散発性腎血管筋脂肪腫、②リンパ脈管筋腫症合併腎血管筋脂肪腫、③ 結節性硬化症合併腎血管筋脂肪腫、④リンパ脈管筋腫症および結節性硬化症合併腎血管筋脂肪腫の4つのグループに分けて、腫瘍および腫瘍内動脈瘤が増大しまたは破裂し動脈塞栓術が必要になる頻度を検討した。症例数が少ないため、③と④を同一グループとして解析したが、今回のグループ分け後の分析により散発性腎血管筋脂肪腫で2回目の動脈塞栓術が必要となる頻度が10%と低く、他の2群では30%以上の症例で2回以上の動脈塞栓術が必要であることを明確にした。従ってこれらの群でより密な経過観察が必要であることが明らかになった。