Pediatric Hamartoma of the Tuber Cinereum

  • Etiology: Congenital malformation characterized by heterotopia of gray matter in regions of mamillary bodies of tuber cinereum
  • Imaging MRI:
    — Round non-enhancing pedunculated or sessile solid mass hypothalamic mass
    — Contiguous with tuber cinereum
    — Rarely associated with large cysts
    — Normal posterior pituitary bright spot
    — T1WI: Isointense to gray matter
    — T2WI: Slightly hyperintense to gray matter
  • DDX: Hypothalamic astrocytoma, Langerhans cell histiocytosis, germ cell tumor
  • Complications:
  • Treatment:
  • Clinical:
    — Pedunculated or parahypothalamic hamartomas tend to have isosexual precocious puberty by 2 years and sessile intrahypothlamic hamartomas present with gelastic seizures in early childhood
    — Lesions are most commonly isolated but in 10% of cases are associated with Pallister-Hall syndrome

Radiology Cases of Hamartoma of the Tuber Cinereum

MRI of hamartoma of the tuber cinereum / hypothalamic hamartoma
Sagittal T2 (above), T1 without contrast (below left) and T1 MRI with contrast (below right) of the brain show a suprasellar mass that is solid and heterogenous which is isointense on T1 and T2 and is nonenhancing.
MRI of pediatric hamartoma of the tuber cinererum
Sagittal T1 MRI without contrast of the brain (above) shows a normal posterior pituitary bright spot. There is an oval solid hypothalamic mass that is isointense to gray matter that is contiguous with the tuber cinereum. Sagittal T1 MRI with contrast (below) shows no contrast enhancement of the mass.