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Child's Nervous System
Published in: Child's Nervous System 2/2016

01-02-2016 | Case Report

Cavernous angioma after chemotherapy for desmoplastic/nodular medulloblastoma associated with anhidrotic ectodermal dysplasia

Authors: Fumiyuki Yamasaki, Takeshi Takayasu, Ryo Nosaka, Hiroshi Kawaguchi, Kazuhiko Sugiyama, Masao Kobayashi, Kaoru Kurisu

Published in: Child's Nervous System | Issue 2/2016

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Abstract

Purpose

While cavernous angioma (CVA) after cranial irradiation has been documented, its development after high-dose chemotherapy with autologous peripheral blood stem cell transplantation (PBSCT) has not. We present a patient with desmoplastic/nodular medulloblastoma (DNMB) associated with anhidrotic ectodermal dysplasia (AED) who developed CVA 2 years after high-dose chemotherapy and PBSCT.

Methods

A 1-year-old boy with ingravescent vomiting was admitted to our institute. He presented with a large head, a depressed nasal bridge, low-set ears, thick lips with peg-shaped teeth, hypohidrosis, sparse hair, thin atrophic skin, scaly dermatitis with frontal bossing, and a bulging anterior fontanel. Neuroradiological examination revealed multiple cerebellar masses with heterogeneous enhancement and speckled calcifications and severe obstructive hydrocephalus. The histological diagnosis of surgical specimens was DNMB, and he underwent postoperative multiple-drug chemotherapy with autologous PBSCT. The outcome was favorable and he did not undergo radiotherapy.

Results

After 2 years, intracranial hemorrhage was detected at his regular radiological check-up and he again underwent surgery. The histological diagnosis was CVA.

Conclusions

To our knowledge, this is the first report of AED-associated DNMB and CVA.
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Metadata
Title
Cavernous angioma after chemotherapy for desmoplastic/nodular medulloblastoma associated with anhidrotic ectodermal dysplasia
Authors
Fumiyuki Yamasaki
Takeshi Takayasu
Ryo Nosaka
Hiroshi Kawaguchi
Kazuhiko Sugiyama
Masao Kobayashi
Kaoru Kurisu
Publication date
01-02-2016
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 2/2016
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-015-2848-6

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