胎児・新生児の上気道周囲に発生する胚細胞腫瘍に関する検討 Clinical outcomes of germ cell tumors arising around fetal and neonatal upper airways

Access this Article

Author(s)

    • 臼井 規朗 Usui Noriaki
    • 大阪母子医療センター小児外科 Department of Pediatric Surgery, Osaka Women's and Children's Hospital
    • 岩崎 駿 Iwasaki Shun
    • 大阪母子医療センター小児外科 Department of Pediatric Surgery, Osaka Women's and Children's Hospital
    • 山道 拓 Yamamichi Taku
    • 大阪母子医療センター小児外科 Department of Pediatric Surgery, Osaka Women's and Children's Hospital
    • 山西 整 Yamanishi Tadashi
    • 大阪母子医療センター口腔外科 Department of Oral and Maxillofacial Surgery, Osaka Women's and Children's Hospital
    • 曹 英樹 Soh Hideki
    • 大阪母子医療センター小児外科 Department of Pediatric Surgery, Osaka Women's and Children's Hospital

Abstract

<p>胎児や新生児の頭頸部に発生する胚細胞腫瘍は,腫瘍が巨大になると気道狭窄を来すため,出生後に致死的な経過をたどることがある.われわれは,胎児・新生児の上気道周囲に発生する胚細胞腫瘍(以下,本症)の治療経験から,本症の治療手段と問題点について検討した.対象は1989年から2018年までの期間に当院で経験した本症10例とし,臨床データを後方視的に検討した.診断は上顎体7例,頸部奇形腫3例であった.腫瘍の発生部位は前頸部3例,咽頭3例,硬口蓋2例,鼻中隔1例,頭蓋底1例であった.病理組織診断は成熟奇形腫5例,未熟奇形腫5例であった.非出生前診断症例4例は全例が成熟奇形腫で生存退院した.出生前診断は,未熟奇形腫の5例と成熟奇形腫の1例になされた.出生前診断された6例中2例は,胎児・新生児の要因により死亡した.残る4例は出生時の気道確保が困難であると予想されたためEXITを施行し,頸部奇形腫1例を除く3例で気道確保に成功した.EXITで気道確保に成功して腫瘍切除できた2例が生存退院した.胎児・新生児の上気道周囲に発生する胚細胞腫瘍症例において,EXITでの気道確保は有効な手段である.しかし救命には,気道確保の方法だけでなく,出生直後の治療計画を予め検討しておくことも肝要である.</p>

<p>The aim of this study was to assess the clinical outcomes and problems in fetuses and neonates with germ cell tumors arising around the upper airway. A retrospective study was carried out for ten patients with germ cell tumors arising around the upper airways in the fetal and neonatal periods, who received treatment at our institution between 1989 and 2018. The diagnoses were epignathus in seven patients and cervical teratoma in three patients. The pathological diagnoses were mature teratoma in five patients and immature teratoma in five patients. Giant neck masses were prenatally diagnosed in six patients with epignathus or cervical teratoma. The ex utero intrapartum treatment (EXIT) procedure was performed in four patients with evidence of tracheal compression by the tumors, but not in two patients because of intrauterine fetal death or death due to respiratory failure after birth. Although airway management under the EXIT procedure was successful in three patients, it failed in one patient. All patients with mature teratoma survived to discharge, whereas four of the five patients with immature teratoma did not survive to discharge. Only the patients whose tumors were resected after birth survived to discharge. We concluded that the EXIT procedure was effective for the airway management of fetuses prenatally diagnosed with neck masses arising around the airway. In patients with giant neck masses, not only the airway management at delivery, but also the treatment strategy for the tumor itself should be considered.</p>

Journal

  • The Japanese Journal of Pediatric Hematology / Oncology

    The Japanese Journal of Pediatric Hematology / Oncology 56(2), 163-167, 2019

    The Japanese Society of Pediatric Hematology / Oncology

Codes

  • NII Article ID (NAID)
    130007705339
  • Text Lang
    ENG
  • ISSN
    2187-011X
  • Data Source
    J-STAGE 
Page Top