Nephrogenic rest vs immature teratoma associated with lumbosacral lipomyelomeningocele: a case report and review of the literature

Childs Nerv Syst. 2023 Jun;39(6):1685-1689. doi: 10.1007/s00381-023-05867-7. Epub 2023 Feb 6.

Abstract

Background: Lipomyelomeningoceles (LMMs) are subcutaneous lipomas with dural penetration that often present with spinal cord tethering and may lead to neurological deterioration if untreated. This report describes a rare case of an LMM associated with immature nephroblastic tissue, representing a nephrogenic rest (NR) or, less likely, an immature teratoma.

Clinical presentation: An 8-day-old infant girl presented to the clinic with a sacral dimple. Imaging demonstrated a tethered spinal cord with low-lying conus medullaris and an LMM. A firm mass was noted in the subcutaneous lipoma. Detethering surgery and removal of the lipoma and mass were performed at the age of 6 months. Pathological examination identified the mass as cartilage, fat, and immature nephroblastic tissue consistent with NR tissue or, less likely, a teratoma with renal differentiation.

Conclusion: This presentation of an LMM associated with an immature teratoma or NR poses a risk of malignant transformation in patients. As a result, careful surgical dissection, resection, and close clinical follow-up are recommended for these patients.

Keywords: Immature teratoma; Lipomyelomeningocele; Nephrogenic rest.

Publication types

  • Review
  • Case Reports

MeSH terms

  • Female
  • Humans
  • Infant
  • Lipoma* / complications
  • Lipoma* / diagnostic imaging
  • Lipoma* / surgery
  • Meningomyelocele* / complications
  • Meningomyelocele* / surgery
  • Neural Tube Defects* / surgery
  • Teratoma* / complications
  • Teratoma* / diagnostic imaging
  • Teratoma* / surgery

Supplementary concepts

  • Lipomyelomeningocele