Hypothalamic obesity syndrome: rare presentation of CNS+ B-cell lymphoblastic lymphoma

Pediatr Blood Cancer. 2012 Nov;59(5):930-3. doi: 10.1002/pbc.24058. Epub 2011 Dec 27.

Abstract

Hypothalamic obesity syndrome can affect brain tumor patients following surgical intervention and irradiation. This syndrome is rare at diagnosis in childhood cancer, but has been reported with relapse of acute lymphoblastic leukemia. Here we present a case of hypothalamic obesity syndrome as the primary presentation of a toddler found to have CNS+ B-cell lymphoblastic lymphoma. Cytogenetic studies on diagnostic cerebrospinal fluid revealed MLL gene rearrangement (11q23). Hyperphagia and obesity dramatically improved following induction and consolidation chemotherapy. We describe a novel presentation of hypothalamic obesity syndrome in CNS B-cell lymphoblastic lymphoma, responsive to chemotherapy.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Child, Preschool
  • Chromosomes, Human, Pair 11 / genetics*
  • Gene Rearrangement*
  • Histone-Lysine N-Methyltransferase
  • Humans
  • Hyperphagia / diagnostic imaging
  • Hyperphagia / drug therapy
  • Hyperphagia / genetics
  • Hypothalamic Neoplasms* / diagnostic imaging
  • Hypothalamic Neoplasms* / drug therapy
  • Hypothalamic Neoplasms* / genetics
  • Male
  • Myeloid-Lymphoid Leukemia Protein / genetics*
  • Obesity* / diagnostic imaging
  • Obesity* / drug therapy
  • Obesity* / genetics
  • Precursor B-Cell Lymphoblastic Leukemia-Lymphoma* / diagnostic imaging
  • Precursor B-Cell Lymphoblastic Leukemia-Lymphoma* / drug therapy
  • Precursor B-Cell Lymphoblastic Leukemia-Lymphoma* / genetics
  • Radiography

Substances

  • KMT2A protein, human
  • Myeloid-Lymphoid Leukemia Protein
  • Histone-Lysine N-Methyltransferase