Literature DB >> 23686409

Long-term tumor-free survival case of congenital embryonal tumor with various pathological components.

Junko Sato1, Norihito Shimamura, Masato Naraoka, Kiminori Terui, Kenichiro Asano, Etsurou Itou, Hiroki Ohkuma.   

Abstract

PURPOSE: 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.
CONCLUSION: Treatment strategies for malignant indefinite diagnosed tumor need to be discussed.

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Mesh:

Year:  2013        PMID: 23686409     DOI: 10.1007/s00381-013-2052-5

Source DB:  PubMed          Journal:  Childs Nerv Syst        ISSN: 0256-7040            Impact factor:   1.475


  14 in total

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