Teratoma or enterogenous cyst? The histopathological and clinical dilemma in co-existing occult neural tube dysraphism

Folia Neuropathol. 2006;44(1):24-33.

Abstract

Background: Better understanding of embryology, histopathology and genetics of dysraphic conditions have lead to an expansion of this concept to entities with a similar microscopic appearance (e.g. enterogenous cysts, colloid cyst of the III-rd ventricle) or rated among neoplasms (e.g. mature teratoma), creating a certain conceptual confusion. Currently the diagnosis of "teratoma" is being substituted by "enterogenous cyst" or "teratomatous cyst".

Aim of paper: Clarification of concepts in this field and presentation of the experience of the Department of Neurosurgery of the Children's Memorial Health Institute associated therewith.

Material and method: Since January 1990 through April 2005 we had treated 7 children with the final diagnosis of "an enterogenous cyst". The mean age of the children was 10.5 years and the mean follow-up time was 4.1 years. The study was performed by a retrospective analysis of medical records, imaging studies and histological preparations.

Results: the study group included: 3 cases of type I enterogenous cyst, 1 case of type II cyst and 3 cases of type III cyst (in 2 cases a former diagnosis of "mature teratoma" has been revised). Within this follow-up time, a good outcome was obtained in 3 cases, moderate disability - in 3 cases and severe disability - in 1 case.

Conclusions: (1) Enterogenous cyst often co-exists with other dysraphic features; (2) Clinical signs of an enterogenous cyst are non-typical and depend on location of the lesion. The presence of secreting gastric mucosa may lead to chemical myelitis; (3) Severity of the postoperative neurological deficit is due to the developmental nature of the lesion and common vascular supply; (4) Late results of treatment are satisfactory, provided the lesion is excised radically; (5) Enterogenous cyst may be diagnosed in the case of a tumor composed of tissues originating from 1, 2 or 3 embryonic layers coexisting with dysraphic stigmata, congenital vertebral abnormalities or a mediastinal tumor of the same type. Types II and III of enterogenous cyst were historically diagnosed as "adult teratoma".

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Central Nervous System Cysts / complications*
  • Central Nervous System Cysts / pathology*
  • Central Nervous System Cysts / surgery
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Female
  • Humans
  • Male
  • Neurosurgical Procedures
  • Spinal Dysraphism / complications*
  • Teratoma / complications*
  • Teratoma / pathology*
  • Teratoma / surgery