Fetal and neonatal rhabdoid tumor

J Pediatr Surg. 2010 Mar;45(3):619-26. doi: 10.1016/j.jpedsurg.2009.12.011.


Purpose: Few studies have focused on the behavior of rhabdoid tumor (RT) in the fetus and neonate. The purpose of this review is to show that perinatal RTs are associated with unusual findings and a poor prognosis.

Methods: The author conducted a 40-year systematic review of the literature. Clinical presentation, pathology, management, and outcome of 72 fetuses and neonates with RTs are discussed.

Results: Seventy-two fetuses and neonates presented with RTs detected prenatally (n = 12) and during the neonatal period (n = 60). The review consisted of 3 main groups: extrarenal noncentral nervous system (CNS) RT, renal RT, and CNS RT. There were some group differences in survival: extrarenal non-CNS RT (3/33 or 9.1%), renal RT (2/27 or 7.4%), and CNS RT (2/12 or 16.7%). Metastatic RT was present at diagnosis in more than half the patients (41/72 or 57%) who had a survival of 2.3%. The overall survival was 9.7%. For statistical results, there was no significant difference in survival among the 3 groups by type of tumor (P = .692). chi(2) analysis for survival with and without metastases was not valid due to small sample size.

Conclusions: The review shows that extrarenal RT was more common than either renal RT or CNS RT groups that is different than that observed in older individuals. Concomitant brain tumors were found in almost a third of fetuses and neonates. The CNS involvement occurred more often in patients with renal RT than in those with extrarenal RT. Metastatic disease at diagnosis was noted in more than half of the patients. Higher stage and presence of a CNS tumor were significant determinants in survival.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Central Nervous System Neoplasms / epidemiology*
  • Central Nervous System Neoplasms / pathology
  • Central Nervous System Neoplasms / therapy
  • Combined Modality Therapy
  • Female
  • Fetal Diseases / diagnosis
  • Fetal Diseases / epidemiology*
  • Fetal Diseases / therapy
  • Humans
  • Incidence
  • Infant, Newborn
  • Infant, Newborn, Diseases / diagnosis
  • Infant, Newborn, Diseases / epidemiology
  • Infant, Newborn, Diseases / therapy
  • Kidney Neoplasms / congenital
  • Kidney Neoplasms / epidemiology*
  • Kidney Neoplasms / pathology
  • Kidney Neoplasms / therapy
  • Male
  • Pregnancy
  • Prenatal Diagnosis*
  • Prognosis
  • Rhabdoid Tumor / congenital
  • Rhabdoid Tumor / diagnosis
  • Rhabdoid Tumor / epidemiology*
  • Rhabdoid Tumor / therapy
  • Risk Assessment
  • Sex Distribution
  • Survival Analysis