Pituitary germ cell tumor with pancytopenia accompanied by gelatinous degeneration of bone marrow

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  • Kido Takahiro
    Department of Pediatrics, University of Tsukuba Hospital
  • Kobayashi Chie
    Department of Pediatrics, University of Tsukuba Hospital Department of Child Health, Faculty of Medicine, University of Tsukuba Ibaraki Children’s Hospital
  • Yaita Katsuyuki
    Department of Pediatrics, University of Tsukuba Hospital
  • Hosaka Sho
    Department of Pediatrics, University of Tsukuba Hospital
  • Suzuki Ryoko
    Department of Pediatrics, University of Tsukuba Hospital Department of Child Health, Faculty of Medicine, University of Tsukuba
  • Fukushima Hiroko
    Department of Pediatrics, University of Tsukuba Hospital Department of Child Health, Faculty of Medicine, University of Tsukuba
  • Yamaki Yuni
    Department of Pediatrics, University of Tsukuba Hospital
  • Iwabuchi Atsushi
    Department of Pediatrics, University of Tsukuba Hospital Department of Child Health, Faculty of Medicine, University of Tsukuba
  • Muroi Ai
    Department of Neurosurgery, University of Tsukuba Hospital
  • Yano Yoko
    Department of Diagnostic Pathology, University of Tsukuba Hospital Department of Diagnostic Pathology, Graduate School of Comprehensive Human Sciences, University of Tsukuba
  • Fukushima Takashi
    Department of Pediatrics, University of Tsukuba Hospital Department of Child Health, Faculty of Medicine, University of Tsukuba
  • Saito Hisako
    Department of Pediatrics, Tsukuba Medical Center Hospital
  • Sumazaki Ryo
    Department of Child Health, Faculty of Medicine, University of Tsukuba Ibaraki Children’s Hospital

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Other Title
  • 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

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Abstract

<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>

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