case report: atypical rathkes cleft cyst
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NATIONAL HOSPITAL NATIONAL HOSPITAL OF PEDIATRICSOF PEDIATRICS
AN ATYPICAL RATHKES CLEFT CYST AN ATYPICAL RATHKES CLEFT CYST AND AND
DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS
Reporter: DR. Hong Nhung Le
Imaging Diagnostic Department
NATIONAL HOSPITAL OF PEADIATRICS
INDIVIDUAL INFORMATIONINDIVIDUAL INFORMATION
• Name: HOAI LINH PHAM
• Sex: Female
• Date of birth: November, 9th, 2000
• Address: 516 Alley, Tran Tat Van street, Kien An district, Hai Phong city
• Telephone number:01696309762
• Date of examination: June, 21st, 2012- NHP
CLINICAL MANIFESTATIONCLINICAL MANIFESTATION
• Transient headache last for 3 months in recent year
• At the time of examination : headache attacks 3 times a week in average
• No visual disturbance, no hemianopsia.
• Individual history: normal development
• Family history: no special finding.
SUBCLINICAL TESTSUBCLINICAL TEST• Bone Age: approximately 10 years
• Endocrinological Test: Normal pituitary funtion
GH= 1.8 µg/l (BT <5.0 µg/l)
Prolactin 7.3 µg/l (BT <15.0 µg/l)
Thyrotropin 1.0 mU/l (BT 0.1–4.0 mU/l)
Luteinizing hormone 21.30 IU/l (BT 15–67 IU/l);
Follicle-stimulating hormone 15.50 IU/l (BT 20–40 IU/l);
Adrenocorticotropic hormone 16.3 pg/ml (BT 4.4–48.0 pg/ml)
Cortisol 10.8 nmol/L (BT 3.2–13.9 nmol/L)
MRI FindingsMRI Findings(T2W, Axial)(T2W, Axial)
Cystic mass: D=15mm
MRI FindingsMRI Findings(T1W, sagital)(T1W, sagital)
Cystic mass in the Sellar and supprasellar extension
MRI FindingsMRI Findings((FLAIR coronal)FLAIR coronal)
Hypersign compared to CSF
mass
MRI FindingsMRI Findings(T1W Axial-Postcontrast)(T1W Axial-Postcontrast)
MRI FindingsMRI Findings(T1W Axial-Postcontrast)(T1W Axial-Postcontrast)
MRI FindingsMRI Findings(T1W Axial-Postcontrast)(T1W Axial-Postcontrast)
MRI RESULTMRI RESULT
Cystic mass in the sella and suprasellar extension:
AN ATYPICAL RATHKES CLEFT CYST?
DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS
• Rathkes cleft cyst• Epidermoid cyst • Craniopharygioma (CR)
RATHKES CLEFT CYST
EPIDERMOID CYST
CRANIOPHARY-GIOMA
DEMOGRAPHICS At any ageGender: F>M
At any age Adamatinomatous: 5-15 yearPapilary: above 50 yearGender: M=F
FEATURE - 40% infrasella, 60% suprasellar extension- Size: 5-15mm
- Suprasellar- Varying size
- 75% suprasella; 21% combination; 4% infrasella- Size >5cm
CLINICAL SIGNS • Asymtomatic• Symtomatic: - Pituitary disfuntion - Headache - Visual disturbance
- Visual disturbance
- Headache - Visual disturbance- Pituitary disfuntion
DIFFERENTIAL DIAGNOSIS
RATHKES CLEFT CYST
EPIDERMOID CYST
CRANIOPHARY-GIOMA
MRI - Varying signal. - Intracystic nodule : 70%.- FLAIR:hypersign - No internal enhance;
+/- rim of compressed pituitary
- Varying signal- TIWI: hypersignal- No enhance or
minimal rim enhance,
- Restriction on DWI
- 90%Calcified, solid, cyst
- FLAIR=hyper
- 90% Enhance =rim(capsule)+nodule(solid)
CT-SCANNER - 75% hypointense,- Nonenhanced
From -100 to +30 HU Adamatinomatous: 90% calci
DIAGNOSTICCHECKLIST
Intracystic nodule FAT on CT Strong enhancement
DIFFERENTIAL DIAGNOSIS
RATHKES CLEFT CYST
EPIDERMOID CYST
CRANIOPHARY-GIOMA
PROGNOSIS - Most stable.- May shrink and disappear.- Noneoplasm
- Most stable- Noneoplasm
- Slow growing benign neoplasm- Survival>10Y:60%
TREATMENT - Conservative- Aspiration/excision if Symtomatic
- Primary sugery - Surgery and radiation
RECURRENCE - Rate<1/3 - Rate<1/3 - Size > 5 cm: ~80%- Size <5 cm: ~20%
DIFFERENTIAL DIAGNOSIS
TYPICAL RATHKES CLEFT CYSTTYPICAL RATHKES CLEFT CYST
Intracystic nodule
INTRACYSTIC NODULEINTRACYSTIC NODULE (Continuing)(Continuing)
Hypersignal on T1W Hyposignal on T2W
ATYPICAL RATHKES CLEFT CYSTATYPICAL RATHKES CLEFT CYST
TYPICAL EPIDERMOID CYSTTYPICAL EPIDERMOID CYST T1 W coronal Precontrast:T1 W coronal Precontrast:
Cystic mass in the suprasella
TYPICAL EPIDERMOID CYSTTYPICAL EPIDERMOID CYST T1 W coronal PostcontrastT1 W coronal Postcontrast
Rim enhanced mass
EPIDERMOID CYST EPIDERMOID CYST DWI-ADCDWI-ADC
Restriction on DWI
TYPICAL CRANIOPHARYGIOMATYPICAL CRANIOPHARYGIOMA
Strong enhancement at capsule and solid structure
DISCUSSIONDISCUSSION
• Imaging technique on MRI.
• Embryology of Rathkes pouch and Rathkes cleft cyst.
• Diagnostic checklist.
• Treatment strategy
IMAGING TECHNIQUEIMAGING TECHNIQUE
• High resolution:2-3mm (thick)
• Sagital T1 pre+postcontrast • Coronal T1 pre+postcontrast• Axial T2W
• Dynamic gadolium enhance coronal T1 for microadenoma (20s subsequence)
EMBRYOLOGYEMBRYOLOGY
A: Infundibulum and Rathke's pouch develop from neural ectoderm and oral ectoderm, respectively.
B: Rathke's pouch constricts at base.
C: Rathke's pouch completely separates from oral epithelium.
D: Adenohypophysis is formed by development of pars distalis, pars tuberalis, and pars intermedia; neurohypophysis is formed by development of pars nervosa, infundibular stem (median eminence)
DIAGNOSTIC CHECKLISTDIAGNOSTIC CHECKLIST
• On MR images, Rathke's cleft cysts (RCC) show various signal intensities.
• The key figure considered to be indicative of RCC is intracystic nodule.
• Finding intracystic nodule difficult and overlook when similar to signal of cystic surrounding.
TREATMENT STRATEGYTREATMENT STRATEGY
• Symtomatic Rathkes cleft cyst (RC)and Epidermoid cyst (EC) have the same treatment strategy.
• Symptomatic RCC or EC should be treated carefully with simple evacuation, irrigation, and biopsy via a transsphenoidal route.
• Craniopharygioma require a different treatment strategy, including the choice of meticulous dissection from the hypothalamus or radiation or both.
CONCLUSIONCONCLUSION
• Our case demonstrates any potential lesion may occur. We should take the follow-up examination regularly by MRI to evaluate the lesion’s progress (6),(10)
• If the headache or any other symtom involving the cyst development, decision for extensive surgery must be made on the basis of histopathologic analysis. (11)
REFERENCEREFERENCE
4. Voelker JL, Campbell RL, Muller J. Clinical, radiographic, and pathological features of symptomatic Rathke's cleft cysts. J Neurosurg 1991;74:535-544
5. Keyaki A, Hirano A, Llena JF. Asymptomatic and symptomatic Rathke's cleft cysts. Histological study of 45 cases. Neurol Med Chir (Tokyo)1989;29:88-93
6. El-Mahdy W, Powell M. Transsphenoidal management of 28 symptomatic Rathke's cleft cysts, with special reference to visual and hormonal recovery. Neurosurgery 1998;42:7-17
10. Osborn W Diagnostic Imaging 2000;:875-877; 892-895 11 Woo Mok Byun, Oh Lyong Kim, and Dong sug Kim MR Imaging Findings of
Rathke's Cleft Cysts: Significance of Intracystic Nodules AJNR Am J Neuroradiol 2000 21: 485-488
THANKS FOR YOUR THANKS FOR YOUR ATTENTION!ATTENTION!
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