Neurosurgery notes/Tumours/Cyst and tumour like lesions/Rathke's cleft cyst

Rathke's cleft cyst

View Details
Status
Done
logo
Parent item
Cyst and tumour like lesions

General

  • Aka: pars intermedia cyst

Definition

  • Slow-growing nonneoplastic lesions that are thought to be remnants of Rathke’s pouch
    • Same origin as
      • Pituitary adenomas
      • Adenohypophysis

Numbers

  • Found incidentally in 13–23% of necropsies.

Origin

  • Anterior wall of Rathke’s pouch
    • RCCs and pituitary adenomas have a similar lineage and rarely they are found together

Pathology

  • Rathke pouch forms during 4th week of embryonic development
  • Wall of cyst lined by a single layer of ciliated columnar epithelium with goblet cells
  • Intraluminal nodule:
    • Macroscopically appears white
    • Adherent to cyst wall or free floating
    • Consist of solid tissue made of desquamated cellular debris
notion image

Clinical presentation

  • Due to mass effect and inflammation
  • Most asymptomatic/incidental
  • Headache
    • 44-81% of symptomatic
    • Sudden onset in 16%
  • Endocrine disturbance
    • 30-60%
    • Males: Hypogonadism
      • Fatigue
      • Dec. libido
    • Females:
      • Premenopausal:
        • Menstrual irregularities
        • Galactorrhea
      • Postmenopausal
        • Panhypopituitarism
  • Diabetes insipidus (37%)
  • Visual disturbance (11-67%)

Imaging

General

  • Well defined non-enhancing midline cyst with in the sella between anterior and intermediate lobes
  • 40% are intrasellar only
  • 60% have supra-sellar component
  • Midline without stalk deviation

CT

  • Low-density cystic lesions.
  • Infrequently can erode the skull base.
  • CT+C: 50% capsular enhancement.
 
CT
CT
CT+C
CT+C

MRI

  • Variable appearance
  • T1
    • Hyperintense
      • 50%
      • Proteinaceous mucinous contents
    • Hypointense
      • 50%
      • Clear low protein fluid
  • T2
    • 70% are hyperintense
    • 30% are iso or hypo-intense
  • T1+C
    • No contrast enhancement of the cyst
    • Thin enhancing rim of compressed pituitary tissue may be seen
  • Intracystic nodule
      • Pathognomonic for Rathke cleft cyst
      • T1: hypertense
      • T1+C: non enhancing
      • T2: hypo-intense
      notion image
       
  • If a fluid level is seen = haemorrhage
notion image
Images
T1
notion image
notion image
T1+C
notion image
notion image
T2
notion image
FLAIR
notion image

Natural history

  • 5% increased in size and 16% actually decreased in size with 27 months
  • 69% showed no growth over 9 years

Treatment

  • Incidental: f/u serial imaging
  • Symptomatic: drained Transphenoidally (microscopically or endoscopically)
    • Debate:
      • Removing cyst wall have been said to reduce recurrence but has high incidence of post-op endocrine dysfunction
      • No evidence of using H2O2 or Etoh irrigation to reduce recurrence rate

Outcome

Surgical

  • Post-cyst decompression (Occur in 97% cases)
      • Improvements
      Rates
      ㅤ
      Visual disturbance
      83-97%
      ㅤ
      Headache
      71%
      ㅤ
      Endocrinopathies
      31-94%
      ㅤ
  • Complications
    • CSF leak
      • 10%
      • Higher rates with extension outside the sella
    • Permanent diabetes insipidus (DI):
      • Cyst drainage: 9%
      • Cyst wall resection: 19–69%

Recurrence of cyst

  • 16–18% over 2–5 years
  • Higher if
    • Purely suprasellar location,
    • Inflammation and reactive metaplasia in the cyst wall,
    • Cyst infection,
    • Use of a fat graft within the cyst cavity

Differential diagnosis

Feature
Craniopharyngioma
Rathke’s cleft cyst
Site of origin
Anterior superior margin of pituitary
Pars intermedia of pituitary
Cell lining
Stratified squamous epithelium
Single layer cuboidal epithelium
Cyst contents
Cholesterol crystals
May be clear or may resemble motor oil
Surgical treatment
Total removal is the goal
Partial excision and drainage
Cyst wall
Thick
Thin