SUPRASELLAR MASS: CT SCAN OF A CHILD

Dr Rekha Khare MD Radiology

CLINICAL PRESENTATION

A male child of about eight months was brought by his parents to CT room for head CT scan

Parents noticed the bulging of left eye for few weeks

Vision was poor/ blind left eye There was no other constitutional symptoms

CLINICAL EXAMINATION

Apparently child was found normal except PROPTOSIS Left eye

Routine laboratory exam were with in normal limit

COMMON CAUSES OF PROPTOSIS

V_ Vascular (arterio venous malformation) E_ Endocrinal ( thyrotoxicosis ) I_ Infection/ Inflammation N_ Neoplasm ( Primary/ Secondary ) ( Local/ Intracranial ) VEIN……

WHAT TO SEE ON CT SCAN?

eye ball eye muscles ( extra ocular ) retro bulbar space optic nerve bony socket adjoining paranasal sinus/ nasal cavity intracranial tissue

PLAIN CT SCAN OF BRAIN

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CONTRAST ENHANCED CT SCAN

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CT FINDINGS IN OUR CASE

An about 22x22mm hypodense globular mass, highly enhanced slightly heterogeneous in suprasellar region

Mass is extending to left frontal lobe and deepening the floor of sella

Mass is markedly entering to left orbital fossa through optic chiasma, encasing optic nerve sparing extra ocular muscles

DIFFERENTIAL DIAGNOSIS

In contrary to adult age most common suprasellar tumor in child are with decreasing frequency ……

: Craniopharyngioma :Chiasmatic/ Hypothalamic low grade glioma : Germinoma : Lesion attributed to langerhans cell

histiocytosis

SATCHMOE….ANOTHER WAY OF D/D SUPRA/ PARA/ INTRASELLAR MASS

Satchmoe : nick name of “Louis Armstrong”

1. S: Sarcoidosis, sellar tumor2. A: Adenoma, Aneurysm3. T: Teratoma, Tubercular granuloma4. C: Craniopharyngioma, Cyst-Rathke’s cleft 5. H: Hemartoma,Hypothalamic glioma6. Histiocytosis 7. M: Metastasis8. O: Optic nerve glioma, Germinoma9. E: Epidermoid, Eosinophilic granuloma

CRANIOPHARYNGIMA

Large intra and supra sellar mass with cystic& enhancing component as well as calcification in children are virtually

pathognomonic for Craniopharyngioma…… perhaps with only Dermoid in the D/D

**Our case shows no calcification at all

PATHOLOGICAL TYPE

Admantinomatous ….paediatric age group Papillary…..Adult age group Mixed……15%

EPIDEMIOLOGY

There is Bimodal age presentation with 1st peak between 10-14 age almost exclusively of Admantinomatous type

2nd peak young to middle aged mostly papillary sub type

Incidence about 6% of all expanding lesion in childhood

CLINICAL SIGNS/ SYMPTOMS

Increased intracranial tension (headache)

Endocrine dysfunction (Delayed puberty, short stature)

Visual problem

RADIOLOGICAL FEATURES

95% suprasellar, frequently distorting optic chiasma or compressing mid brain causing hydrocephalus

Occasionally intraventricular homogenous soft tissue mass with calcification commonly at 3rd ventricle

CT SCAN FINDINGS

Cystic near CSF density Soft tissue density vivid enhancement Calcification in 90% cases

**calcification is uncommon in papillary variety

CHIASMATIC HYPOTHALAMIC GLIOMA

4-8% childhood intracranial tumor, rare in adults

Common in first decade less than 1yr Male: Female incidence is equal 20-30% children are associated with

neurofibromatosis Progress rapidly

CH GLIOMA CONTD…..

Chiasmatic glioma can involve anything between optic nerve to visual cortex

Almost 25% optic pathways gliomas are confined to optic disc and nerve

40-75% may involve optic chiasma

CLINICAL SIGNS/ SYMPTOMS

Painless PROPTOSIS

Hydrocephalus if involving foramen of monro

**HISTOLOGICALLY: Low grade Glioma

PITUITARY ADENOMA

Rarely present in children

Pituitary micro adenoma: less than 10mm in size

Pituitary macro adenoma: more than 10mm in size expansion of sella is common

Most pediatric pituitary adenomas present after the onset of puberty and present with frequent headaches, changes in visual acuity and, in females, menstrual dysfunction.

Most (19/20) were secretory, with prolactinomas being the most common type.

imaging findings are same like any other suprasellar mass

SUPRASELLAR MENINGIOMA

Benign tumor of meninges usually solid may contain necrotic/ cystic lesion and calcification

Frequently invade the sella turcica

Such tumor can also encase the optic nerve with in optic canal and fossa orbitalis

ARACHNOID CYST AND RATHKE’S CLEFT CYST

Rarely in suprasellar region in children

Clinical symptom: visual symptom, hydrocephalus,

macrocephaly, pyramidal tract sign, precocious puberty

MISCELLANEOUS LESION

A. Hypothalamic hemartoma:

usually small and asymptomatic presenting in two decades

Clinical symptom: hyperactivity syndrome with hyperplasia, behavior disorder, precocious puberty,

gelastie seizure

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B. Lesion of INFUNDIBULUM: : Germinoma : Hypophysitis : Histiocytosis X

SUPRASELLAR GERMINOMA 20% of all Germ cell tumor

Hypo intense on T1, Hyper intense on T2 and extremely intense contrast enhancement

It can create diagnostic dilemma on imaging , can get easily confused with optic/ chiasmatic glioma.

If serum and CSF studies for tumor marker are negative , surgical exploration for biopsy and histological confirmation helps differentiation

FINAL DIAGNOSIS

Our probable diagnosis: Suprasellar germinoma OR Chiasmatic

Hypophyseal Glioma

Patient was sent to advanced center for final diagnosis and further management

Unfortunately there was no feed back

CONCLUSION

Paediatric brain tumor have always been a challenge

They are heterogeneous set of pathologies involving different age groups in childhood and also differ widely from their adult counterparts as far as adjuvant therapies are concerned

Unlike Meningioma and Pituitary Adenoma in adults, Craniopharyngioma, Optic Glioma and Germinoma form the main lesions in paediatric age group

CONTD…..

While safe surgery is the Key in most of adult tumors, Chemo and Radiotherapy are the chief modality in some of these paediatric tumor like GERMINOMA and OPTICO- CHIASMATIC GLIOMA

As epidemiology and management of these tumors is different that’s why it requires dedicated multidisciplinary team approach

REFERENCES

1. Differential Diagnosis of suprasellar mass in children: Warmnth Metz M, Gnekow AK, Muller H, Solynosi L K.Klin Paediatri 2004 Nov Dec 216(6):323;30

2. Pituitary region mass: Dr Ayush Goel and Dr Frank Gaillard etal. Radiopaedia.org/article/Pituitary

3. Sella turcica and parasellar region: Walter Kucharezyk and Marieke Hazewinkel The Radiology assistant

4. Cranipharyngioma: Dr Frank Gaillard etal. Radiopaedia.org/article/craniopharyngioma

REF……CONTD.

5. Suprasellar Meningioma_ a case report: MG Kgoro, A Speclman The south African Radiographer vol.51 no 2 Nov 2013

6. Pituitary macro adenoma: Dr Henry Knipe and Yuranga W etal. Radiopaedia.orgt

7. Paediatric Suprasellar Lesion:. CE Deopujari, Ashish Kumar, VS Karmarkar, NK Biyani,

M Mhatre and NJ shah Journal of Paediatric Neurosciences 2011 Oct(6

supple): 546-555

Thank you Have a good day