診断時に骨髄の膠様変性を伴う血球減少を認めた鞍上部原発胚細胞腫瘍の1例 Pituitary germ cell tumor with pancytopenia accompanied by gelatinous degeneration of bone marrow

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著者

    • 矢野 陽子 Yano Yoko
    • 筑波大学附属病院病理診断科|筑波大学大学院人間総合科学研究科病理診断学分野 Department of Diagnostic Pathology, University of Tsukuba Hospital|Department of Diagnostic Pathology, Graduate School of Comprehensive Human Sciences, University of Tsukuba
    • 福島 敬 Fukushima Takashi
    • 筑波大学附属病院小児科|筑波大学医学医療系小児科 Department of Pediatrics, University of Tsukuba Hospital|Department of Child Health, Faculty of Medicine, University of Tsukuba
    • 須磨崎 亮 Sumazaki Ryo
    • 筑波大学医学医療系小児科|茨城県立こども病院 Department of Child Health, Faculty of Medicine, University of Tsukuba|Ibaraki Children's Hospital
    • 小林 千恵 Kobayashi Chie
    • 筑波大学附属病院小児科|筑波大学医学医療系小児科|茨城県立こども病院 Department of Pediatrics, University of Tsukuba Hospital|Department of Child Health, Faculty of Medicine, University of Tsukuba|Ibaraki Children's Hospital
    • 鈴木 涼子 Suzuki Ryoko
    • 筑波大学附属病院小児科|筑波大学医学医療系小児科 Department of Pediatrics, University of Tsukuba Hospital|Department of Child Health, Faculty of Medicine, University of Tsukuba
    • 福島 紘子 Fukushima Hiroko
    • 筑波大学附属病院小児科|筑波大学医学医療系小児科 Department of Pediatrics, University of Tsukuba Hospital|Department of Child Health, Faculty of Medicine, University of Tsukuba
    • 岩淵 敦 Iwabuchi Atsushi
    • 筑波大学附属病院小児科|筑波大学医学医療系小児科 Department of Pediatrics, University of Tsukuba Hospital|Department of Child Health, Faculty of Medicine, University of Tsukuba
    • 室井 愛 Muroi Ai
    • 筑波大学附属病院脳神経外科 Department of Neurosurgery, University of Tsukuba Hospital

抄録

<p>生来健康で活発だった14歳女児.診断の1年前より頭痛と食思不振,意欲低下があり,6か月前より頻回の嘔吐がみられ経口摂取量は更に低下した.病前と比較し–13.5 kg(36%)の体重減少をきたし神経性食思不振症を疑われたが,頭部CTで下垂体上部から第3脳室を占拠する6 cm大の腫瘤性病変を指摘された.血清hCGの上昇と病理組織からGerminoma with STGCと診断し,化学療法はICE療法を選択した.閉塞性水頭症,汎下垂体機能不全,中枢性尿崩症による高張性脱水に加え,2系統の血球減少(Hb 9.0 g/dL,Plt 5.8万/μL)を認めた.骨髄は低形成で膠様変性を伴い,造血回復の遅延が懸念されたため化学療法は減量した.全脳全脊髄照射は回避し,全脳室照射と局所照射を行った.栄養状態の改善に伴い膠様変性は消失,治療終了後血球は正常化した.骨髄に膠様変性をきたす疾患として神経性食思不振症が多く報告されているが,本症例では類似の低栄養状態に加え,下垂体機能不全の要素が関与したと考えられた.</p>

<p>A previously healthy 14-year-old girl complained of headache, appetite loss and depression. She had lost 36% (13.5 kg) of her weight over six months owing to low food intake and frequent vomiting. CT revealed an obstructive hydrocephalus due to a 6 cm mass occupying the third ventricle. She was diagnosed as having pituitary germ cell tumor (germinoma with STGC) on the basis of surgical biopsy results and serum marker levels. Her condition was complicated by panhypopituitarism, diabetes insipidus, anemia (Hb 9.0 g/dL), and thrombocytopenia (Plt count, 58,000/μL). Bone marrow examination revealed marked hypoplasia accompanied by gelatinous degeneration. We performed reduced dose chemotherapy and proton beam therapy targeting the whole ventricle and tumor bed, excluding the craniospinal region. The gelatinous degeneration of the bone marrow was resolved by sufficient nutritional control, hormone replacement therapy, and treatment of the brain tumor. Her blood cell count also normalized subsequently. Most reported cases of gelatinous bone marrow degeneration are found among patients with anorexia nervosa. Not only significant malnutrition but panhypopituitarism may have contributed to her condition.</p>

収録刊行物

  • 日本小児血液・がん学会雑誌

    日本小児血液・がん学会雑誌 55(2), 199-203, 2018

    日本小児血液・がん学会

各種コード

  • NII論文ID(NAID)
    130007428484
  • 本文言語コード
    JPN
  • ISSN
    2187-011X
  • データ提供元
    J-STAGE 
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