Hypothalamic obesity syndrome: rare presentation of CNS+ B-cell lymphoblastic lymphoma.
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.
Section of Pediatric Hematology/Oncology, Riley Hospital for Children, Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN 46202, USA.
Troy C
TC
Quigg
Nadine G
NG
Haddad
Jeffrey C
JC
Buchsbaum
Chie-Schin
CS
Shih
D002675:Child, Preschool; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics*; D015321:Gene Rearrangement; D011495:Histone-Lysine N-Methyltransferase; D006801:Humans; D006963:Hyperphagia / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000235:genetics; D007029:Hypothalamic Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000235:genetics; D008297:Male; D051788:Myeloid-Lymphoid Leukemia Protein / Q000235:genetics*; D009765:Obesity / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000235:genetics; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000235:genetics; D011859:Radiography
C497422:KMT2A protein, human; D051788:Myeloid-Lymphoid Leukemia Protein; D011495:Histone-Lysine N-Methyltransferase