Case report: atypical Rathkes cleft cyst

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!