Giant supratentorial enterogenous cyst: report of a case, literature review, and discussion of pathogenesis

Neurosurgery. 2004 Mar;54(3):759-63; discussion 763. doi: 10.1227/01.neu.0000109538.07853.7f.

Abstract

Objective and importance: To describe a histologically well-documented adult case of a giant supratentorial enterogenous cyst (EC). Fewer than 15 cases of supratentorial ECs are on record: 8 associated with the brain hemispheres or the overlying meninges, 4 with the sellar region, and 2 with the optic nerve.

Clinical presentation: A 31-year-old woman complained of long-standing mild left brachial and crural motor deficit precipitated by headache and signs of intracranial hypertension. Magnetic resonance imaging revealed a huge cyst overlying the frontoparietal brain.

Intervention: Symptoms were relieved by evacuation of the cyst content by means of a Rickam's reservoir, and the lesion was subsequently removed in toto. Histological and immunohistochemical examination of the cyst wall clearly established the enterogenous nature of its epithelium. Follow-up for up to 2 years after intervention showed no sign of recurrence, and symptoms, including treatment-resistant seizures in the postoperative period, have entirely subsided.

Conclusion: Supratentorial ECs, distinctly rare in adult patients, may in some cases present as giant lesions. Total removal seems to be curative once careful examination has eliminated the possibility of a metastasis from an unknown primary. A correct histological diagnosis is important because, in contrast to other benign cysts of similar location and size, ECs may be prone to intraoperative dissemination.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Arachnoid Cysts / pathology
  • Arachnoid Cysts / surgery
  • Biomarkers, Tumor / analysis
  • Carcinoembryonic Antigen / analysis
  • Central Nervous System Cysts / pathology
  • Central Nervous System Cysts / surgery*
  • Craniotomy
  • Diagnosis, Differential
  • Epithelium / pathology
  • Female
  • Follow-Up Studies
  • Frontal Lobe / pathology
  • Frontal Lobe / surgery*
  • Humans
  • Intracranial Hypertension / pathology
  • Intracranial Hypertension / surgery
  • Keratins / analysis
  • Magnetic Resonance Imaging
  • Meningeal Neoplasms / pathology
  • Meningeal Neoplasms / surgery*
  • Mucin-1 / analysis
  • Neural Tube Defects / pathology
  • Neural Tube Defects / surgery*
  • Parietal Lobe / pathology
  • Parietal Lobe / surgery*
  • Supratentorial Neoplasms / pathology
  • Supratentorial Neoplasms / surgery*

Substances

  • Biomarkers, Tumor
  • Carcinoembryonic Antigen
  • Mucin-1
  • Keratins