Low-grade astrocytoma in a child with encephalocraniocutaneous lipomatosis

Author(s)

Brassesco, Maria Sol ^{[1, 2]} ; Valera, Elvis Terci ^[2] ; Becker, Aline Paixao ^[3] ; Castro-Gamero, Angel Mauricio ^[4] ; Machado, Andre de Aboim ^[5] ; Santos, Antonio Carlos ^[5] ; Scrideli, Carlos Alberto ^[2] ; Oliveira, Ricardo Santos ^[6] ; Machado, Helio Rubens ^[6] ; Tone, Luiz Gonzaga ^[2]

Affiliation

^[1] USP, Pediat Lab, Hosp Clin, Fac Med Ribeirao Preto, BR-14048900 Ribeirao Preto, SP - Brazil

^[2] Univ Sao Paulo, Div Pediat Oncol, Dept Pediat, Sao Paulo - Brazil

^[3] Univ Sao Paulo, Dept Pathol, Sao Paulo - Brazil

^[4] Univ Sao Paulo, Dept Genet, Sao Paulo - Brazil

^[5] Univ Sao Paulo, Div Radiol, Sao Paulo - Brazil

^[6] Univ Sao Paulo, Div Neurosurg, Dept Surg & Anat, Fac Med Ribeirao Preto, Sao Paulo - Brazil

• Document type: Journal article
• Source: JOURNAL OF NEURO-ONCOLOGY; v. 96, n. 3, p. 437-441, FEB 2010.
• Web of Science Citations: 11

Abstract

Encephalocutaneous lipomatosis (ECCL), or Haberland syndrome, is an uncommon congenital disorder with unique cutaneous, ocular and neurological features. In the present article, we describe a 3-year-old boy with ECCL who developed an extensive and recurring intraventricular low-grade glioma with atypical pathological features and elevated mitotic index. Cytogenetic analysis from tumor sample was also performed. This is the first report of a low-grade astrocytoma occurring in a child with ECCL. Whether or not the origin of the tumor is associated to the pathogenesis of the underlying syndrome is a matter for further investigation. (AU)

• FAPESP's process: 06/04827-3 - Analysis of aneuploidies by comparative genomic hybridization (CGH) in pediatric patients with acute lymphoid leukemia
• Grantee: María Sol Brassesco Annichini
• Support Opportunities: Scholarships in Brazil - Post-Doctoral