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Craniopharyngiomas
- 1. Overview of Craniopharyngiomas Amanuel Firew , Neurosurgical resident June , 2021
- 2. Outline • Introduction of CP • Relevant anatomy for CP • Embryology of Pit gland • Classifications • Clinical presentation • Radiologic features • Options of management 6/15/2021 2
- 3. Introduction • WHO G I partly cystic epth tumour of sellar region derived from embryonic remnants of Rathke’s pouch epithelium, with two clinicopathological variants • Benign tumor in a malignant location • ‘Kaleidoscopic tumors , solid & cystic …whose management is one of the baffling problem to the Neurosurgeon ’ ….Cushing 6/15/2021 3
- 5. 6/15/2021 5 Sellar and suprasellar anatomy relevant to CP
- 6. Sellar and suprasellar anatomy relevant to CP 6/15/2021 6
- 7. Epidemiology • Relatively rare tumor , 1.2 - 4.6% of all IC tumours • 0.5 - 2.5 new cases per 1 million population per year[33 – 54 % peds ] • Most common non-glial-origin intracerebral neoplasm in peds [5-11% of ped IC tumours] • AdamantinomatousCP – Bimodal [peaks at 5-15 & 40-65 yrs ] • PapillaryCP – Exclusively adults[40 – 55 yrs mean age] • No obvious sex or race predilection • No known environmental factors , No clear familial tendency 6/15/2021 7
- 8. CP – Histopathology Adamantinomatous CP • Rathke’s pouch , Embryogenetic theory , CTNNB1 mutation • Solid + Cyst[machine oil] • Epithelial lobules with loose stroma, wet keratin , calcifications & peripheral palisading epth • Inflammatory changes and fibrosis >> adherence to surrounding structures Papillary CP • Rathke’s pouch , Metaplastic theory , BRAF mutation • Predominatly solid [lack of calcifications , cystic elements or intratumoral fluid] • Sheets of well-differentiated NK squamous epth in a papillary configuration, with a broad fibrovascular core 6/15/2021 8
- 9. Location • Most common site is suprasellar cistern , with a minor intrasellar component – 75 % Suprasellar cistern – 20 % intrasellar and – Only 5 % suprasellar , • Papillary CP , are also found in the 3rd V [5 %] • Unusual locations – Sphenoid sinus , CPA • CP involving the sella – Intrasellar – Intra and supra sellar infradiaphragmatic – Intra and supra sellar transdiaphragmatic – Intra and supra sellar supradiaphragmatic • Suprasellar CP – Prechiasmatic – Retrochiasmatic – infrachiasmatic • CP involving the 3rd ventricle – Purely IV – Partially IV 6/15/2021 9
- 10. Classification schemes • Several classification • Growth path and the surgical route used • Principle : Classify along the length of extension in the 10 vertical axis , as related to the optic chiasm, diaphragma sellae , 3rd V , or more recently to the infundibulum 6/15/2021 10
- 11. Yasargil et al., 1990 a.Purely intrasellar-infradiaphragmatic b.Intra- and suprasellar, infra - and supradiaphragmatic c.Supradiaphragmatic, parachiasmatic, extra-ventricular d.Intra and extra-ventricular e.PV f.Purely IV 6/15/2021 11
- 12. Classification schemes Kassam et el , 2008 Type I – Preinfundibular Type II – Transinfundibular Type III – Postinfundibular – IIIa , infundibular recess,hypothalamic – IIIb , interpeduncular fossa Type IV – Isolated 3rd V 6/15/2021 12
- 13. Classification schemes QST classification • On the basis of the tumor origin and on the presence of ASPS • Pituitray stalk – Infradiaphragmatic – extra-arachnoidal – intra-arachnoidal – subarachnoidal 6/15/2021 13
- 14. QST Q – below DS - PIT S – pars tuberalis - IA & EA PS T – RP precursor cell @ top of pars tuberalis 6/15/2021 14
- 15. Clinical picture • Prodrome of 1 – 2 yrs between symptoms and diagnosis • 2nd ary to the disease itself and treatment complications • Mainly secondary to pressure on neuronal structures – ↑ ICP – Visual impairment – Endocrine dysfunction – Cognitive impairment and personality changes 6/15/2021 15
- 16. Endocrine dysfunction GH [75 %] LH/FSH [40 %] ACTH [25 %] TSH [25 %] PRL [<150 ng/ml , 55 % ] Hypopituitarism [85 %] 6/15/2021 16
- 17. Clinical picture • Visual impairment[1/2 of pts] & H/A[60-75 %] , common in adults • Visual impairment[ visual field , acuity , color blindness , papilledema] 6/15/2021 17
- 18. CP imaging • The most heterogeneous radiologic appearance of any suprasellar neoplasm • The cystic and to lesser extent the solid part variable signal on MR • CT , MRI and MRA NCCT • Hypodense soild & cystic part[depends on contents] • Calcification – 90 % • Skull base anatomy 6/15/2021 18
- 19. CP imaging - MRI Adamantinomatous CP • Heterogeneous , lobulated , mass with cystic and solid components , as well as calcifications • Cysts ̶ commonly T1 , T2 and FLAIR Hyper • As protein conc ↑ – T1 signal Δs Hypo → Hyper → Hypo – T2 signal Δs Hyper → Hypo Papillary CP • Usually , Suprasellar & homogenous • Imaging feats – Spherical – Solid – CT iso , T1 iso , T2 hetero – Heterogenously enhancing nodule – Cyst & Calcification uncommon – When cyst occurs , T1 hypo & T2 hyper 6/15/2021 19
- 20. CP imaging - CTA • Not routinely indicated • Carotids laterally , basilar posteriorly , ACOM/ACA superiorly • Adamantinomatous CP , can adhere or surround arterial walls but frank narrowing is rare • Rules out suprasellar aneurysm , when MRI/MRA is inconclusive 6/15/2021 20
- 21. Overview of management • Multidisciplinary • Goal – tumor control or cure • Curative treatment – longterm medical access + lifetime HRT • Optimal treatment – controversial – Extent of resection – Role of RT – Intracystic therapies • Options – Complete microsurgical resection + post op endcrinologic replacement – Subtotal resection + RT – Intracystic therapies 6/15/2021 21
- 22. Surgeries for CP • Transcranial – Pterional [OZ , OF] – Subfrontal – Transcallosal , transcortical , translamina terminalis – 3rd V • Endonasal Transsphenoidal – Endoscopic – Microscopic • Ommaya reservoir for cystic CPs 6/15/2021 22
- 23. Surgical approaches by location 6/15/2021 23
- 24. Surgeries for CP • Pterional craniotomy [removal of SO rim , ant orb roof , & Zyg proc ] – preferred – Direct and shortest distance to parasellar region – Less retraction 6/15/2021 24
- 25. Surgical outcomes • Radiographically confirmed complete resection [60 – 100 %] • Recurrence rate , 0 – 20 % [Usually within 2 – 3 yrs] • Periopertaive MR from [6 - 11 % ]→ [0 – 4 %] in the last decade • Endocrine disturbances , wc are common after radical resect ion as a result of hypothalamic manipulation and pituitary stalk sectioning • 80 % of peds need HRT 6/15/2021 25
- 26. TSS vs Transcranial surgery 6/15/2021 26
- 27. 6/15/2021 27
- 28. Radiotherapy for CPs • Used as 10 treatment after cyst aspiration , biopsy or STR & as salvage in recurrent disease • RT ↓ Progression rate of STR from [55 – 85 %] to [15 – 20 %] • Retrospective data also showed improved local control • For a recurrent / progressive disease – comparable local control of tumor to RT given with the 1st 3 mos post op • Tumor + 5 – 10 mm margin • 54 – 60 Gy [ 1.8 gy daily fraction ] 6/15/2021 28
- 29. Intra cavitary treatment • Most effective and often curative for primary moncystic tumor with thin wall • Significant control of cystic compartment of recurrent CP • Intracavitary iradiation • Intracavitary bleomycin • Intracavitary IFN • Aspiration[simple or serial via ommaya] 6/15/2021 29
- 30. Recurrent CP • Most common complication after treatment • Dx - Imaging surveillance/clinically overt • OS and PFS rates are similar for radical resection compared to STR + RT • Median time of rec , [2-8 yrs] depending on initial modality of treatment • Factors for recurrence – Extent of resection , >5cm , highly calcified tumors , extension beyound suprasellar space , large or calcified intrasellar tumor – Molecular Osteonectin , p53 , PTTG – 1 , EPCAM • Ectopic recurrence – Rare phenomenon[50 reports] – Mostly aCP 6/15/2021 30
- 31. Recurrent CP - treatment • Options for treatment – reoperation for tumor resection , intracystic bleomycin or a b- emitting radionuclide, fractionated RTX , SRS , or some combination thereof • Reoperation – Whenever possible , after both GTR and STR + XTR[Esp. peds and solid tumors] – Intial modality of treatment affects Redo SX • XRT , extensive arachnoid fibrosis • Radical resection , destruction of cleavage plane[ gliotic rxn] – Non candidate patients • Asymptomatic elderly pts • Cystic tumor 6/15/2021 31
- 32. Recurrent CP surgical approaches • Previous craniotomy – challenging , adhesions & scar , lost plane bn tumor & neurohypophyseal tissue • Pterional craniotomy[modified] • TSS – Reserved for Sellar tumor • Intra cavitary therapies – palliative – Cystic tumor – Pts not pursuing aggressive sx • XRT • SRS – Predominnatly solid < 3CM , few mm away from optic apparatus 6/15/2021 32
Editor's Notes
- Pit gland Sup – optic N , optic chiasm & circle of willis Lat – cav sinus & carotid a Post – basilar a & brainstem Diaphragm sellae
- , Common in Asian and african countries
- Cholesterol species in cystic tumors are capable of producing an inflammatory parenchymal reaction. Papillary ca - Predominantly solid , rarely undergo mineralization.If cyst occurs , fluid is less oily and dark.
- The geographic center of craniopharyngiomas is the suprasellar cistern in approximately 75% of patients; approximately 20% are intrasellar and suprasellar; approximately 5% are purely intrasellar From a surgical perspective, craniopharyngiomas involving the sella can be categorized as being purely intrasellar, intra- and suprasellar infradiaphragmatic, intra- and suprasellar transdiaphragmatic, and intra- and suprasellar supradiaphragmatic (Effenterre & Boch, 2002) With regard to suprasellar craniopharyngioma, tumors can be categorized as being prechiasmatic, retrochiasmatic, and/or infrachiasmatic. Purely intraventricular tumors arise above the floor of the third ventricle in the region of the tuber cinereum. Partially intraventricular tumors arise below the floor of the third ventricle and penetrate the ventricle after invasion of the parenchyma. Develops from epithelial nest cells of ratheke’s pouch , located along an axis from sella turcica , pitituitary stalk to hypothalamus & floor of 3rd vent.Gradually enlarges as partially calcified , heterogenous solid and cystic mass.Extend along the path of least resistance into basal cisterns or invaginate into 3rd vent Neurosurgical atlas Although generally centered on or near the infundibulum, the clinical presentation of a craniopharyngioma depends on the tumor’s exact location relative to structures surrounding it: the pituitary gland and stalk, optic apparatus, and the third ventricle and its floor (the hypothalamus) Craniopharyngiomas typically arise from nests of metaplastic adenohypophyseal cells of the pituitary stalk. Except for the 5% that are purely intraventricular, most of these lesions originate from the parasellar space with their nodule and extend their cystic section into the third ventricle. These tumors adhere to and encase some or all of the following structures: the optic nerves and chiasm, pituitary gland and stalk, circle of Willis, brainstem, hypothalamus, third ventricle, and the frontal/temporal lobes.
- relationship bn tumors & the adjacent structures and to clarify the true origin & growth pattern of CP from the histological level Multiple clinicoradiologic classifications degree of vertical and horizontal extension, displacement of opt ic nerves and chiasm, anatomic regions involved by tumor overall size
- Neurosurgical atlas In adults, visual disturbances and headaches are the most common presenting neurologic findings. Neurocognitive changes due to infiltration of the hypothalamus are also common, although endocrine dysfunction is variable and frequently not clinically significant. In children, elevated intracranial pressure is more common, while endocrine dysfunction is usually related to growth hormone insufficiency; occult visual findings are also often present in children. Hydrocephalus occurs in one-third of both populatio Visual deficit , 62 - 84 % , com in adults Endocrine defcy , 52 – 87 % , com in peds GH defcy in 75% LH/ FSH defcy in 40% ACTH defcy 25% TSH defcy 25% DI 17% [peds] and 30%[adults] Cognitive impairment and personality changes – Hypothalamus involvement [half of pts] Obesity and hyperphagia (signs of hypothalamic dysfunction) Signs of increased intracranial pressure are frequent, especially in cases with compression or invasion of the third ventricle Craniopharyngiomas can be small and clinically inconsequential, or massive and life-threatening, with large lesions simultaneously involving the anterior, middle, and posterior cranial fossae (Sener, 1993). Although histologically benign, they can locally invade the brain parenchyma and cause vasogenic edema (Saeki et al., 2003). Leakage of cyst contents into the subarachnoid space or ventricular system can result in chemical meningitis (Kulkarni et al., 2000) Rarely, remote recurrences have been reported by direct transplantation of tumor or presumed seeding of the subarachnoid spaces (Gupta et al., 1999;Ito et al., 2001; Bianco et al., 2006)
- The cystic and to a lesser extent the solid portions can demonstrate variable density on CT imaging, and variable signal characteristics on MR imaging (Karnaze et al., 1986; Puseyet al., 1987; Eldevik et al., 1996; Wolfe & Heros, 2010) On occasion, tumor cysts may show fluide fluid levels (Abrahams et al., 1989). Craniopharyngiomas can be calcified or noncalcified. Although a suprasellar location is most common, these neoplasms can also arise primarily in the sella; rarely, they can arise in unusual locations such as the third ventricle sphenoid sinus, or nasopharynx (Kanungo et al., 1995; Deutsch et al., 2001; Behari et al.,2003).
- Cyst contents – cholesterol , protein , TAG , desquamated epithelium , methemoglobin Papillary CP - Distinict from adamantinomatous as histological apearances do Hyperintense signal on T2-weighted and FLAIR sequences in the brain parenchyma adjacent to a craniopharyngioma is uncommon (Figures 3.3, 3.8), even with very large lesions; if present, this may reflect vasogenic edema due to (1) parenchymal invasion by the tumor, (2) microscopic leakage of cyst contents, or (3) compression of the optic chiasm/tracts Unlike glial tumors, advanced MR imaging techniques such as MR spectroscopy, MR perfusion imaging, and diffusion tensor imaging do not ordinarily play an important role in the assessment of sellar/suprasellar lesions (Plaza et al., 2013)
- The cyst(s) can be hypodense or hyperdense relative tonormal parenchyma, and they can be very small or very large. The density of the cyst(s) onCT imaging is a function of the cyst contents; the higher the protein concentration, the denserthe cyst. CT is superior to MR imaging in detecting calcification. Calcification tends to be morestriking in pediatric as compared to adult tumors (Sorva et al., 1987; Harwood-Nash, 1994).Calcification is present in the vast majority of lesions; it may be coarse or stippled, and itcan be located along the cyst walls and/or within the nodular portions. Although enhancedMR imaging has replaced enhanced CT imaging in the evaluation of the brain, the cyst wallson CT imaging typically enhance after contrast administration, as do the solid, nodular portions of the tumor. Craniopharyngiomas are avascular on conventional angiography (Baker, 1972). If warranted, CT angiography can be obtained to most elegantly establish the relationship of the tumor to the adjacent arterial vasculature; there is little role for conventional diagnostic angiography in this setting. Larger lesions may displace the internal carotid arteries laterally, thebasilar artery posteriorly, and the anterior communicating artery complex/anterior cerebralarteries superiorly. Although adamantinomatous craniopharyngioma can adhere to, or surround arterial walls, frank vascular narrowing is very rare; arterial vasospasm induced bychemical meningitis from cyst rupture has been reported (Shida et al., 1998). In the unusual circumstance where MR imaging and MR angiography are inconclusive in ruling out a suprasellar aneurysm, CT angiography can also be very helpful.
- Ectopic tumor recurrence distant from the primary site as a result of implantat ion at the t ime of init ial resect ion w as seen in 7% of recurrent tumors treated b y the senior author ( J .H.W .). Centers at w hich few er than tw o radical resect ions per y ear w ere performed had a good outcome in only 52% compared w ith 87% for inst itut ions in w hich radical resect ions w ere performed more often
- No studied dose – response relationship >60 gy , high risk of optic neuropathy and necrosis
- Recurrent cp – most present with vis deterioration[acuity aswell as field cut].H/A , HCP , hor disturbance and sz are other manifestation. Time of recurrence depend on modality of treatment STR – definite progression will occur STR + RT ….20 % will recur in 4.8 yrs.5yr treatment free survival – 89 %.10 yr treatment free survival – 76% Ectopic rec – along surgical tract or csf pathways EXTENT OF RESECTION – SINGLE FACTOR MOST ASSTD WITH REC