A case of <i>MYCN</i>-amplified stage 4 neuroblastoma relapsed with catecholamine-negative tumor mainly composed of MIBG-non-avid component

DOI
  • Yaegashi Miki
    Department of Pediatric Oncology, Fukushima Medical University Hospital Department of Pediatrics, Fukushima Medical University
  • Sano Hideki
    Department of Pediatric Oncology, Fukushima Medical University Hospital
  • Takahashi Nobuhisa
    Department of Pediatric Oncology, Fukushima Medical University Hospital
  • Ohara Yoshihiro
    Department of Pediatric Oncology, Fukushima Medical University Hospital Department of Pediatrics, Fukushima Medical University
  • Kobayashi Shogo
    Department of Pediatric Oncology, Fukushima Medical University Hospital
  • Tanaka Hideaki
    Department of Pediatric Surgery, Fukushima Medical University
  • Shimizu Hirofumi
    Department of Pediatric Surgery, Fukushima Medical University
  • Hashimoto Yuko
    Department of Diagnostic Pathology, Fukushima Medical University
  • Mochizuki Kazuhiro
    Department of Pediatric Oncology, Fukushima Medical University Hospital
  • Hosoya Mitsuaki
    Department of Pediatrics, Fukushima Medical University
  • Kikuta Atsushi
    Department of Pediatric Oncology, Fukushima Medical University Hospital

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Other Title
  • MIBG集積陰性成分を主体としたカテコラミン非産生の腫瘍が再発した<i>MYCN</i>増幅Stage4神経芽腫の1例

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Abstract

<p>We present a case of stage 4 neuroblastoma of the left adrenal gland with MYCN amplification in a 2.5-year-old boy. At the time of diagnosis, the tumor had a component that was partially negative for 123I-metaiodobenzylguanidine (MIBG) with MYCN amplification. Treatment was completed in accordance with the high-risk protocol, and complete remission was achieved. However, 14 months after treatment completion (at 4.5 years of age), the patient developed a facial nerve palsy. Cancer recurrence was observed in the left cerebellopontine angle, left orbital bone, and bone marrow. The recurrent lesion was weakly positive/negative for MIBG, the urinary catecholamine level was not elevated, and the bone marrow metastatic cells were negative for tyrosine hydroxylase. MIBG-non-avid neuroblastoma has a better prognosis than MIBG-avid neuroblastoma. However, among MIBG-non-avid tumors, non-catecholamine-secreting neuroblastomas have an increased likelihood of containing more undifferentiated and aggressive components and require caution for early relapse.</p>

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