title: Long-term tumor-free survival case of congenital embryonal tumor with various pathological components.
abstract: OBJECTIVE
  Treatment strategy of malignant congenital brain tumor is controversial. We report a congenital embryonal tumor case with various pathological components.
  
  
  METHODS
  A normally delivered male infant had an enlarged head circumference at 1 month after birth. The abnormality of the right side of the head was also noted during the routine 4-month health check. The head circumference was 45.1 cm (+2.25, SD); neurological status, however, was normal, with a pediatric GCS of 9 and body weight of 6,370 g (-0.85, SD). Magnetic resonance imaging (MRI) revealed right brain tumor whose size was 99 × 91 × 86 mm. The tumor was enhanced homogeneously with central necrosis, and the margin of the tumor was well circumscribed.
  
  
  RESULTS
  We performed a subtotal removal of the tumor. The pathological diagnosis was meningioma (MIB-1 index was 2 %). The residual tumor gradually shrank, and we performed monthly MRI follow-up. The tumor abruptly recurred 7 months after the operation. The level of patient consciousness deteriorated, and emergency removal surgery was performed. The histological examination showed various types of embryonal components without meningioma-like parts. The pathological diagnosis was an embryonal tumor. The MIB-1 index was 48 %. One month after the second operation, dissemination of the tumor occurred at the right temporal lobe, cerebellum, and in subcutaneous tissue. Chemotherapy (vincristine, cisplatin, cyclophosphamide, and etoposide) was initiated following radiation therapy (3 Gy/day, 8×). Adjuvant therapies were effective, and no tumor recurrence was detected during 34 months follow-up.
  
  
  CONCLUSIONS
  Treatment strategies for malignant indefinite diagnosed tumor need to be discussed.
authors.affiliation: Department of Neurosurgery, Hirosaki University School of Medicine, 5-Zaihuchou, Hirosaki, Aomori, Japan.
authors.forename: Junko
authors.initials: J
authors.lastname: Sato
authors.forename: Norihito
authors.initials: N
authors.lastname: Shimamura
authors.forename: Masato
authors.initials: M
authors.lastname: Naraoka
authors.forename: Kiminori
authors.initials: K
authors.lastname: Terui
authors.forename: Kenichiro
authors.initials: K
authors.lastname: Asano
authors.forename: Etsurou
authors.initials: E
authors.lastname: Itou
authors.forename: Hiroki
authors.initials: H
authors.lastname: Ohkuma
mesh_terms: D000367:Age Factors; D001254:Astrocytoma / Q000473:pathology / Q000601:surgery*; D001921:Brain / Q000473:pathology*; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000601:surgery*; D013534:Survival; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome