Pituitary tumours
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The pituitary gland, also known as the hypophysis, regulates many other endocrine glands in the body. It can develop tumors called pituitary adenomas. Pituitary adenomas are typically treated using surgery to remove the tumor, radiation therapy, or medication depending on the size and location of the tumor and whether it is causing the pituitary gland to overproduce or underproduce hormones. The presentation, diagnosis, and management of pituitary adenomas were discussed in detail.
Pituitary tumours
- 1. PRESENTED BY :Dr SANDIP KUMAR BARIK DEPT OF RADIOTHERAPY MODERATOR: Dr RAJENDRA KUMAR
- 2. INTRODUCTION ļ Pituitary or hypophysis cerebri is an endocrine gland situated in relation to the base of the brain ļ It is called the Master of endocrine orchestra ļ It produces a number of hormones which control the secretions of many other endocrine gland of the body
- 3. ANATOMY ļ The pituitary gland or hypophysis is an endocrinabout 15 mm in ant-post and 12 mm in supero inferior axis ļ It weighs about 0.5 gm. ļ The pituitary gland occupies a cavity of the sphenoid bone called sella turcica ļ Roof is formed by diaphragm sellae ļ The stalk of pituitary is attached above to the floor of third ventricle
- 4. Anatomy(cont..) ļ Relations ļ Superiorly:Diaphragma sellae,optic chiasma,infundibular recess of 3rd ventricle ļ Inferiorly:Hypophyseal fossa and its venous channels ļ On each side :The cavernous sinus with its content Modified from Lechan RM. Neuroendocrinology of Pituitary Hormone Regulation. Endocrinology and Metabolism Clinics 16:475-501, 1987
- 5. ļ The anterior and intermediate lobe arises from the Rathkeās pouch ļ The posterior lobe or neurohypophysis arises from the downward pocketing of third ventricle. ļ Posterior lobe releases hormones the Oxytocin and Vasopressin which are synthesised in the supraoptic and paraventricular nuclei in hypothalamus ļ Anterior lobe releases hormones ACTH,TSH,GH,FSH,LH,Prolactin
- 6. EPIDEMIOLOGY ļ Pituitary neoplasm account for 10% to 15% of diagnosed primary intracranial neoplasm ļ 3% -25% pituitary glands are identified by autopsy ļ 10% of healthy population has pituitary abnormality detected by MRI ļ Approximately 70% are endocrinologically active ļ Incidence of macroadenomas is similar between males and females ļ However clinical manifestations of microadenomas are more in women
- 7. EPIDEMIOLOGY (Contā¦) ļ 70% of adenomas present between the ages 30 -50 yrs ļ Women have high incidence of pituitary adenomas(15-44 yrs) ļ Annual incidence ranges from 0.5 to 0.7/100,000 ļ Etiology of most adenomas is unknown ļ A genetic predisposition to develop adenomas has been described in MEN I syndrome Carney complex Isolated familial somatotropinomas(IFS)
- 8. NATURAL HISTORY ļ Usually has a long natural history with an insidious onset of symptoms ļ Symptoms are usually present for years prior to diagnosis ļ When small pituitary tumour tends to be smooth round tumours ļ Macroadenomas are known for their local invasive properties ļ Malignant behaviour with distant metastases is rare
- 9. CLINICAL PRESENTATIONS ļ The presenting symptoms may be due to Hormonal malfunction Due to local tumour growth and pressure effect ļ Endocrine abnormalities may be a consequence of hyper or hypo secretion of pituitary hormones. ļ Hypopituitarism ļ Hyperpituitarism Cushings syndrome Hyperprolactinomas Hyperthyroidism Acromegaly
- 10. HYPOPITUITARISM ļ Growth hormone deficiency:Short stature(Dwarfism) ļ Gonadotrophins deficiency:Infertility,decreased sexual functions,loss of secondary sexual characters,menstrual irregularities ļ TSH deficiency :Hypothyroidism ļ ACTH deficiency :Hypocortisolism ļ Prolactin deficiency :Lactation failure ļ Vasopressin deficiency :Diabetes insipidus
- 11. HYPERPITUITARISM ļ HYPERPROLACTINEMIA ļ Most common cause of pituitary hormone hypersecretion ļ Amennorhoea ļ Galactorrhoea ļ Infertility ļ INCREASED GH ļ Acromegaly in adults ļ Frontal bossing ļ Increased hand foot size ļ Mandibular enlargement,Prognathism ļ Large fleshy nose ļ Proximal muscle wasting,carpal tunnel syndrome,macroglossia ļ Gigantism in children
- 12. ļ INCREASED ACTH ļ Causes cushing syndrome ļ Central obesity ļ Plethoric moon facies ļ Purple striae,increased bruisability ļ Glucose intolerence ļ Acne,hirsuitism ļ Proximal muscle weakness ļ Hypertension ļ Amennorhoea,infertility
- 13. FEATURES OF SELLAR MASS LESION ļ PITUITARY ļ Hypopituitarism ļ OPTIC CHIASMA ļ Bitemporal Hemianopia ļ Superior temporal defect ļ CAVERNOUS SINUS ļ Ophthalmoplegia ļ Ptosis ļ Diplopia ļ OTHERS ļ Head ache ļ Hydrocephalus ļ Dementia
- 14. DIAGNOSTIC WORKUP ļ± Detailed History and complete physical examination ļ± Confirmation of diagnosis ļ Radiological Examination MRI-preferred modality better visualisation of soft tissue and vascular structure CT Scan ļ Biopsy āIn a case of non secreting lesion
- 15. ļ± STAGING WORKUP: Chest x ray USG Whole abdomen ļ± General condition: Complete blood count Kidney function tests liver function test Urine analysis
- 16. ļ± HORMONAL ANALYSIS ļ Serum Prolactin level ļ Growth hormone:basal growth hormone level IGF-I Glucose suppression,insulin tolerence ļ ACTH Hypersecretion: Serum ACTH,Dexamethasone supression test 24 hrs urine for 17-hydroxy corticosteroids and free cortisol ļ Gonadal function:FSH,LH,Esradiol,Testosterone ļ Thyroid function test ļ Adrenal function:basal plasma,urinary steroids cortisol response to insulin induced hypoglycaemia
- 17. CLASSIFICATION OF PITUITARY TUMOURS ļ ANATOMICAL SIZE ļ Microadenoma(<10 mm) ļ Macroadenoma(>10 mm) ļ PHYSIOLOGICAL ļ Ant pituitary 1. Prolactin 2. Growth hormone 3. Adrenocorticotrophic hormone 4. Leutinizing hormone 5. Follicle stimulating hormone 6. Thyroid stimulating hormone ļ Post pituitary 1. Oxytocin 2. Vasopressin
- 18. Classification(Contā¦) ļ ACCORDING TO CLINICAL SYMPTOMS ļ Functional ļ Non functionaL ļ ACCORDING TO EXTENT OF EXPANSION OR EROSION OF SELLA ļ Grade 0: Intrapituitary microadenoma with normal sellar appearance ļ Grade I: Nml-sized sella with asymmetric floor ļ Grade II: Enlarged sella with an intact floor ļ Grade III: Localized erosion of sellar floor ļ Grade IV: Diffuse destruction of floor
- 19. Classification(Contā¦) ļ ACCORDING TO SUPRASELLAR EXTENSION ļ Type A: Tumor bulges into the chiasmatic cistern ļ Type B: Tumor reaches the floor of the 3rd ventricle ļ Type C: Tumor is more voluminous with extension into the 3rd ventricle up to the foramen of Monro ļ Type D: Tumor extends into temporal or frontal fossa
- 20. PATHOLOGICAL CLASSIFICATIONS ļ Ant Pituitary has 5 specific cell types ļ Somatotrophs:produces growth hormone,acidophilic ļ Lactotrophs:produces prolactin,acidophilic ļ Corticotrophs:produces ACTH,MSH,basophilic ļ Thyrotrophs:produces TSH,basophilic ļ Gonadotrophs:FSH,LH,basophilic ļ Post pituitary:pituicytes and non myelinated fibres
- 21. MANAGEMENT ļ Observation ļ Surgery ļ Radiotherapy
- 22. OBSERVATION ļ In asymptomatic non secreting microadenomas ļ Small asymptomatic prolactinomas 2 -4 mm no testing required 5-9 mm MRI can be done once yearly ļ Indications for intervention Tumour growth on imaging symptoms of hypersecretion development of visual field defects
- 23. < 10 mm > 10 mm Evaluate for: Evaluate for ā¢ Hormonal Hypersecretion Hormonal ā¢ Hormonal Hyposecretion Hypersecretion ā¢ Visual Changes/defects Hormonal or Visual Normal Abnormalities No Abnormalities Observe Observe Treatment
- 24. SURGERY INDICATIONS ļ It is the first line treatment for most symptomatic pituitary tumours ļ Useful when medical or radiotherapy fails ļ When prompt relief from mass effect and hormone secretion is required ļ Pituitary apoplexy
- 25. ļ TYPES ļ± MICROSCOPIC TRANSSEPTAL TRANSSPHENOIDAL ļ Current standard surgical procedure ļ Safe procedure with mortality rate 0.5% ļ Contraindications are sphenoid sinusitis,ectatic midline carotid arteries,lateral surpasellar extent
- 26. ļ± ENDOSCOPIC TRANSNASAL TRANSSPHENOIDAL ļ Allows better visualisation of pituitary gland,ghyophyseal stalk,cavernous sinuses,optic nerve and suprasallar areas ļ± .TRANSCRANIAL ļ Requires craniotomy and retraction of frontal lobes ļ Used for large invasive tumours with significant suprasellar extension ļ When transsphenoidal approach is contraindicated
- 27. COMPLICATIONS OF SURGERY ļ CSF rhinorrhoea ļ Meningitis ļ Haemorrhage ļ Stroke ļ Damage to pituitary ļ Visual loss
- 28. RADIOTHERAPY ļ INDICATIONS 1. Hypersecretion and mass effect due to large tumours 2. Incomplete resection of tumour 3. Progressive disease after surgery 4. Recurrent tumours
- 29. RADIOTHERAPY ļ TECHNIQUES ļ± Conventional External Beam Radiotherapy ļ Manual planning ļ 2D Planning ļ 3D CRT ļ± Fractionated Stereotactic Radiation Therapy ļ± Gammaknief Radiosurgery
- 30. MANUAL AND 2D PLANNING ļ Positioning ļ Supine with neck flexed and head at 45 degrees ļ Pituitary board can be used to achieve this ļ Immobilisation done with thermoplastic mask ļ VOLUME ļ The entire pituitary gland with extensions and a margin of 1-1.5 cm
- 31. ļ PORTALS ļ Two parallel and opp lat fields and one anterior or vertex beam that enters above the eyes ļ The centre of the pituitary is located at a point 2-2.5 anteriorly to tragus and 2-2.5 cm superiorly to that point ļ Taking this point as centre a field of( 4*4)cm-(6*6) cm is marked ļ ENERGY ļ 4-10 Mev or Co 60 ļ DOSE ļ Nonfunctioning tumours 45-50.4 Gy@1.8 Gy/# ļ Functional tumours 50.4-54 Gy
- 32. 3D PLANNING ļ Image based treatment planning using a 3D technique is the standard of care ļ Defining the tumour volume ļ MRI,CT as well as clinical and surgical findings should be used to define the tumour volume ļ CT simulation assists in defining treatment volume ļ GTV is the pituitary adenomas including any extention into adjacent anatomic regions ļ CTV :GTV+5 mm in a clear defined tumour or entire sella and cavernous sinus with invasive tumours ļ PTV:CTV+5mm
- 33. FRACTIONATED STEREOTACTIC RADIOTHERAPY(FSRT) ļ FSRT is characterised by improved patient localisation,tighter volume definition more conformal isodose distributions ļ It has better safety profile and efficacy ļ IMMOBILISATION Aim is to achieve a patient positioning error of less than 3mm by various means like Invasive halo ring Radiocamera bite block Non invasive Head frames
- 34. Stereotactic(contā¦) ļ TARGET VOLUME DELINEATION ļ GTV is designed with help of MRI and extent of cavernous sinus invasion should be included ļ No additional margins is required for CTV ļ PTV:CTV +2-3 mm margin ļ TREATMENT PLANNING ļ Depends on the delivery systems available ļ Options include Multiple spherical shots Dynamic conformal arches Nonisocentric robotic delivery ļ DOSE 50.4 Gy in 28#@1.8Gy/#
- 35. STEREOTACTIC RADIOSURGERY ļ Accepted treatment for smaller,radiologically well defined tumours located at a distance (3-5 mm) from optic apparatus ļ Contraindicated if optic chiasma is closer than (3 -5)mmto the tumour ļ Delivery systems include linear accelerator and gamma knife ļ Head is fixed with an appropriate stereotactic head frame and a high resolution imaging study is obtained ļ MRI used for gamma knief while ct scan for linear accelerator ļ Gamma knife uses smallest collimators and maximum number of isocentres . ļ The dose to optic chiasma is limited to <8-9 Gy ļ DOSE ļ Non functioning (12-20Gy) ļ Functioning (15-30 Gy)
- 37. RESULTS MODALITY SURGERY SURG+POST GAMMA KNIEF VS OP RT RADIOSURGERY SURG+POSTO VS P RT RT ALONE RESULTS Park et.al Grigsby et al Maschiro.et al 10 yrs Proggression Tumour control at recurrence rate free survival at 5 5 yrs is 93.6% and 2.3%with yrs 96% and 20 endocrinological rt,50.5%only yrs 88% improvement is surgery 80.3% CONCLUSION Post op RT Surg+rt had a Results are similar should be greater control to #EBRT but preffered of local disease gamma knief seems to be safer in terms of complications
- 38. COMPLICATIONS OF RADIOTHERAPY ļ ACUTE REACTIONS 1. Fatigue 2. Focal alopecia 3. Otitis ļ CHRONIC REACTIONS 1. Hypopituitarism 2. Damage to optic apparatus 3. Secondary brain tumours 4. Brain necrosis
- 39. CONCLUSION ļ Pituitary tumors are slow growing tumours. ļ Surgery is the first choice of treatment ļ Radiation is generally used as an adjuvant or salvage therapy ļ Surgery followed by post op radiation produce better results ļ Newer treatment modalities like gamma knife produce less complications
- 40. THANKYOU