Rathke Cleft Cyst with T2 Hypointensity: MRI Features, Differential Diagnosis, and Evidence-Based Management
Introduction
Rathke cleft cyst (RCC) is a benign,
non-neoplastic epithelial cyst arising from remnants of Rathke’s pouch,
located in the pars intermedia of the pituitary gland. While most RCCs
are asymptomatic and incidentally discovered, certain imaging
characteristics—particularly T1 hyperintensity combined with striking T2
hypointensity—represent a distinct and diagnostically important subtype.
This article presents a classic case of Rathke cleft cyst with T2
hypointensity, based on the attached MRI study of a 25-year-old woman with
oligomenorrhea and mild hyperprolactinemia. Using this case, we comprehensively
review the pathophysiology, epidemiology, clinical presentation, imaging
features, differential diagnosis, diagnostic approach, treatment strategies,
and prognosis, grounded in widely cited contemporary literature.
Pathophysiology of Rathke Cleft Cyst with T2
Hypointensity
Rathke cleft cysts originate from embryologic remnants of Rathke’s
pouch, which normally involutes during pituitary development. Failure of
complete regression leads to cyst formation between the anterior and
posterior pituitary lobes.
The hallmark imaging feature in this case—T1 hyperintensity and T2
hypointensity—is attributed to highly proteinaceous or mucoid cyst
contents. Elevated protein concentration causes:
- Shortened T1
relaxation time → high T1 signal
- Shortened T2
relaxation time → low T2 signal
This biochemical composition differentiates this RCC subtype from serous
or CSF-like cysts and has significant diagnostic value.
Epidemiology
- RCCs are identified in 13–22%
of autopsy series
- Peak detection in young
to middle-aged adults
- Slight female
predominance
- Most lesions are incidental
- T2 hypointense RCCs are less
common but well-described in neuroradiology literature
Clinical Presentation
Patient Summary (from attached case)
- Age: 25 years
- Gender: Female
- Symptoms: Oligomenorrhea
- Laboratory
findings: Prolactin level 60
ng/mL
The mild elevation of prolactin (<100 ng/mL) is consistent with a stalk
effect, caused by compression of the pituitary stalk, rather than
autonomous hormone secretion.
Imaging Features (MRI-Based Diagnosis)
MRI is the gold standard for diagnosing Rathke cleft cysts.
Figure-Based Imaging Interpretation
Figure 1. Sagittal T1 Fat-Saturated MRI
A well-defined midline intrasellar cyst demonstrates high signal intensity
on T1, consistent with protein-rich cystic content.
Figure 2. Sagittal T2 MRI
The lesion shows marked T2 hypointensity, a classic feature of
proteinaceous Rathke cleft cysts.
Figure 3. Sagittal T2 MRI (Alternative Slice)
Persistent T2 hypointensity confirms the uniform internal composition of the
cyst.
Figure 4. Sagittal T2 MRI
The cyst slightly expands the pituitary gland, without suprasellar extension or
optic chiasm compression.
Figure 5. Sagittal T1 Contrast-Enhanced Fat-Saturated MRI
No internal enhancement is observed. A subtle enhancement of the cyst wall fold
may be present.
Figure 6. Sagittal T1 Contrast-Enhanced Fat-Saturated MRI
The absence of solid enhancing components effectively excludes pituitary
adenoma.
Figure 7. Sagittal T2 MRI
Posterior displacement of the T1 bright spot of the posterior pituitary
gland is noted, a key distinguishing feature from adenomas.
Figure 8. Axial FLAIR MRI
The cyst remains hypointense relative to brain parenchyma, supporting a
non-CSF, proteinaceous composition.
Key Radiologic Hallmarks of Rathke Cleft Cyst
- Midline, pars intermedia
location
- T1 hyperintensity
- T2 hypointensity
- No or minimal rim
enhancement
- Posterior displacement of
posterior pituitary bright spot
- Mild pituitary stalk
bowing
- No cavernous sinus invasion
Differential Diagnosis
|
Entity |
Key Distinguishing Features |
|
Pituitary adenoma |
Off-midline, solid
enhancement, lateral displacement of stalk |
|
Craniopharyngioma |
Calcification, mixed
cystic-solid components |
|
Arachnoid cyst |
Follows CSF signal on all
sequences |
|
Epidermoid cyst |
Diffusion restriction |
|
Hemorrhagic adenoma |
Fluid-fluid levels, evolving
signal |
Diagnosis
Diagnosis of Rathke cleft cyst with T2 hypointensity is primarily
radiologic. Clinical and laboratory findings support the diagnosis but are not
definitive alone.
Key diagnostic elements include:
- Typical MRI signal
characteristics
- Midline location
- Lack of solid enhancement
- Mild endocrine
disturbance consistent with stalk compression
Treatment
Conservative Management
- Asymptomatic or mildly
symptomatic patients
- Periodic MRI follow-up
- Endocrine monitoring
Surgical Management
- Indicated for:
- Visual field defects
- Severe headaches
- Progressive endocrine
dysfunction
- Preferred approach: Endoscopic
transsphenoidal drainage
Prognosis
- Excellent overall
prognosis
- Low recurrence rate after
surgery
- Endocrine dysfunction may
persist if chronic
- Malignant transformation
does not occur
Quiz
Question 1. A midline
intrasellar cyst with T1 hyperintensity and T2 hypointensity most likely
represents which lesion?
A. Pituitary adenoma
B. Craniopharyngioma
C. Rathke cleft cyst
D. Arachnoid cyst
Correct Answer: C
Explanation: Proteinaceous RCCs classically show T1 high and T2 low
signal.
Question 2. Mild
hyperprolactinemia (<100 ng/mL) in Rathke cleft cyst is best explained by:
A. Prolactinoma
B. Hypothalamic dysfunction
C. Stalk effect
D. Hormonal hypersecretion
Correct Answer: C
Explanation: Compression of dopaminergic inhibition causes mild
prolactin elevation.
Question 3. Which MRI
feature best differentiates Rathke cleft cyst from pituitary adenoma?
A. Intrasellar location
B. T1 hyperintensity
C. Lack of enhancement
D. Posterior displacement of posterior pituitary bright spot
Correct Answer: D
Explanation: Adenomas usually displace the bright spot laterally.
References
- Osborn AG, et al., Diagnostic
Imaging: Brain, 3rd ed., Elsevier, 2020.
- Barkovich AJ, Pediatric
Neuroimaging, 6th ed., Lippincott Williams & Wilkins, 2019.
- Trifanescu R et al.,
“Rathke’s cleft cysts,” Clin Endocrinol, vol. 76, no. 2, pp.
151–160, 2012.
- El-Mahdy W, Powell M,
“Transsphenoidal management of RCC,” Neurosurgery, 2001.
- Kucharczyk W et al., “MR
imaging of RCC,” Radiology, 1987.
- Bonneville JF et al., MRI
of the Pituitary Gland, Springer, 2016.
- Zada G et al., “Surgical
management of RCC,” J Neurosurg, 2011.
Comments
Post a Comment