General
- A histologically benign, partly cystic epithelial tumour of the sellar region presumably derived from embryonic remnants of the Rathke pouch epithelium, with two clinicopathological variants (adamantinomatous and papillary) that have distinct phenotypes and characteristic mutations
- Adamantinomatous and papillary craniopharyngioma has now been separately classified due to their different clinical demographics, radiologic features, histopathologic findings, genetic alterations, and methylation profiles.
Numbers
- 1.2-4.6% of all intracranial tumours
- 0.5-2.5 new cases per 1 million population per year
- More frequent in Japan
- Incidence of 3.8 cases per 1 million children
- Bimodal distribution
- Adamantinomatous in children (peak age 5-10yo)
- More cystic
- Dystrophic calcification ~90%
- CTNNB-1 mutation
- Papillary in adults
Grading
- Grade I
Localisation
- Most common site for both subtypes is the suprasellar cistern, with a minor intrasellar component
- Unusual locations
- Sphenoid sinus
- Cerebellopontine angle
- Craniopharyngiomas, mainly the papillary variant, are also found in the third ventricle
- Although benign, project “finger-like” processes into hypothalamus
Cell of origin
- Ectodermal-derived epithelial cell remnants of Rathke pouch and the craniopharyngeal duct
- Occur between the roof of the pharynx and the floor of the third ventricle, most frequently along the anterior infundibulum and the anterior superior surface of the adenohypophysis - sites of the previous Rathke pouch and involuted duct that links these structures.
- Craniopharyngiomas are of neuroendocrine lineage because
- Tumour cells can (rarely) express
- Pituitary hormones
- Chromogranin-A
- hCG
- Craniopharyngiomas share stem cell markers (e.g. SOX2, OCT4, KLF4, and SOX9) with the normal pituitary gland
Various classification
Histopathology
Ki-67 proliferation index
- Higher than might be expected given the relative indolence of the neoplasms
- Adamantinomatous type: concentrated along the peripherally palisading cells
- Papillary type: randomly
- No consistent relationship between proliferation index and recurrence has been established.
- Be aware that Ki67 labelling and CTNNB1 mutation analysis of tissue have poor prognostic value
Immunophenotype
- +
- Pancytokeratin
- CK5/6
- CK7
- CK14
- CK17
- CK19
- EMA
- Claudin-1
- Beta-catenin
Clinical features
- Non-specific
- Visual deficits (62-84%)
- Adults > children
- Endocrine deficiencies (52-87%)
- Children > adults
- Deficiencies of
- Child: Short stature
- Adults:
- Changes in body composition (fat mass>lean body mass),
- Decreased BMD,
- Impaired quality of life,
- Increased mortality rates.
- Male:
- Infertility
- Decreased testosterone secretion →
- Decreased energy
- Dec. libido
- Hot flashes
- Dec. muscle mass (and perhaps strength) after many years
- Female: ovarian hypofunction → decreased estradiol secretion.
- Irregular periods or amenorrhea,
- Anovulatory infertility,
- Hot flashes,
- Vaginal atrophy (final)
- Cortisol deficiency
- Severe: loss of peripheral vascular tone → vascular collapse → death
- Moderate: Vascular tonacity failure → postural hypotension and tachycardia.
- Mild/chronic: lassitude, fatigue, anorexia, weight loss, decreased libido, hypoglycemia, and eosinophilia
- Fatigue, cold intolerance, decreased appetite, constipation, facial puffiness, dry skin, bradycardia, hyporeflexia, and anaemia.
- Diabetes insipidus
- 17% of children
- 30% of adults.
Growth hormone (75%)
Luteinizing hormone / follicle-stimulating hormone (40%)
Adrenocorticotropic hormone (22-25%)
Thyroid-stimulating hormone (25%)
Central diabetes insipidus (7–31%)
- Cognitive impairment and personality changes (50%)
- Signs of increased intracranial pressure are frequent, especially in cases with compression or invasion of the third ventricle.
- Hypothalamic dysfunction
- To objectify the presence of hyperphagia, the Dykens Hyperphagia Questionnaire12 may be used.
- For assessment, history taking, the Epworth Scale criteria,14 actigraphy, and PSG may be used.
- Epworth sleepiness scole
- How likely are you to doze off or fall asleep in the following situation? With scale from 0 to 3, where 0 - no chance; 1 - slight chance; 2 - moderate chance; 3 - high chance, and what is your total score?
- Sitting and reading
- Watching television
- Sitting inactive, in a public space
- Lying down to rest in the afternoon when circumstances permit
- Sitting and talking to someone
- Sitting quietly after a lunch without alcohol
- As a passenger in car for an hour without a break
- In a car, while stopped for a few minutes in traffic
- For assessment of body temperature, core temperature may be measured during several days at fixed times.
- The occurrence of severe obesity can be reduced with resections that spare the hypothalamus
- Pathophysiology
- Nucleus in hypothalamus
- That regulate hunger, satiety, and energy balance.
- Ventromedial hypothalamus (VMH)
- Arcuate nucleus (AN)
- Periventricular nucleus (PVN)
- That receive signals from
- Peripheral hormones (leptin, insulin, ghrelin, neuropeptide Y),
- Which are produced by adipocytes, the islet cells, and the gut after food intake (satiety hormones)
- VMH, AN or PVN are damaged → the signals of the peripheral hormones cannot be properly integrated → increased hunger feeling with subsequent caloric intake.
- Also, due to damage to the efferent pathways, energy expenditure may be decreased.
- The combination of increased hunger feelings with decreased energy expenditure causes weight gain, which can be extreme and rapid.
- Condition associated with severe hyperphagia
- Prader–Willi syndrome
- Smith Magenis syndrome
- Hypothalamic hamartoma
- Craniopharyngioma
- Chiasmatic hypothalamic glioma
- Most children with (general) obesity also have an increased caloric intake, which may be interpreted as hyperphagia, however just a small percentage of these children have true hyperphagia caused by hypothalamic dysfunction.
- A distinction may be made between those children whose hyperphagia can be controlled by adequate parenting styles or those who cannot be controlled by any means.
- In the latter, the hyperphagia can disrupt family life, with children stealing food from class mates or from the refrigerator, requiring 24/7 supervision
- Schematic overview of the nuclei in the human hypothalamus. The hypothalamus consists of many hypothalamic nuclei, which are all highly connected through neural pathways. The connection between the AN and paraventricular nucleus is emphasized. Afferent and efferent blood vessels provide a pipeline for pituitary hormones, as well as hunger and satiety hormones that stimulate hypothalamic neuron orexigenic and anorexigenic responses, respectively.
- AN, arcuate nucleus; CRH, corticotropin-releasing hormone; DM, dorsomedial hypothalamic nucleus; OC, optic chiasm; PA, preoptic area; PH, posterior hypothalamic nucleus; SCN, suprachiasmatic nucleus; SO, supraoptic nucleus; VMN, ventromedial hypothalamus
- Diencephalic syndrome (DS):
- This phenomenon resulting in hypophagia is observed in young children with Prader–Willi syndrome, and also in young children (new-born until the age of 4) with craniopharyngioma or suprasellar LGG.
- Current hypotheses for
- Weight loss focus on
- Loss of appetite
- Increased energy expenditure
- Increased lipolysis by pituitary B-lipotropin
- Caused by growth hormone (GH) overproduction with partial GH resistance
- The fact that hypothalamic hypophagia is mainly seen in young children may be due to the fact that the hypothalamus matures during childhood and thus hypothalamic dysfunction may result in different outcomes, depending on age.
- Hypophagia and anorexia have, however, also been described in older children and even in adults with suprasellar tumors, such as craniopharyngioma.
- Structures in the brain involved the neurocognitive consequences seen in patients with hypothalamic damage.
- Intelligence is most often not affected but school achievements may decline due to the concentration disruption, memory loss, or behavioral problems. Also complaints of (chronic) fatigue may hinder learning capabilities
- The hypothalamus is an integral part of two different networks of the limbic system:
- A hippocampus (HC)-centered network essential for episodic memory (A)
- Episodic memory deficits in HS usually result from lesions to the mammillary bodies (MB) in the posterior part of the hypothalamus or their connecting fibers: fornical fibers projecting from the hippocampus to the MB, or fibers of the mammillothalamic tract projecting from the MB to the anterior thalamic nucleus (A).
- Damage to the Papez circuit may lead to problems in control anxiety or memory problems; children may repeatedly ask for the day planning or get upset when plans suddenly change.
- An amygdala (AMY)-centered network relevant for social–emotional functioning (B).
- Deficits in social–emotional functioning in HS may result from lesions to hypothalamic nuclei anterior to the MB and, for example, from tumor-related or treatment-related damage to other regions of the AMY-centered network (B).
- Damage to the nucleus accumbens may lead to addiction or obsession; children with severe hypothalamic damage often demonstrate the urge for hoarding or collecting items in excessive amounts
- Damage to brain regions within these networks or to their connecting fibers contributes to the neurobehavioral and psychiatric abnormalities in HS.
- RSC, retrosplenial cortex; THAL, thalamus; VSP, ventral striatopallidum
Clinical signs and symptoms
ㅤ | Diagnosis | Clinical sign or symptom |
Eating disordersᵃ | Hyperphagia | Extensive hunger, binge-eating, overweight or obesity |
ㅤ | Hypophagia | Failure to thrive, underweight |
Behavioral disordersᵇ | Obsessive compulsive symptoms | Fixate on certain topics, repetitive behavior or rituals, difficulty with changes |
ㅤ | Hoarding | Collecting items not needed |
ㅤ | Rage | Uncontrollably angry |
Sleep disorderᶜ | Sleep apnea | Snoring, fatigue |
ㅤ | -Hypersomnia/insomnia - Early morning awakening | Daytime sleepiness, falling asleep at random places during the day, frequently wake at night, waking up extremely early |
Temperature regulation disordersᵈ | Hypothermia | Cold feeling, shivering, temperature < 36 °C |
ㅤ | Hyperthermia | Temperature > 37.5 °C without infection |
ㅤ | Temperature dysregulation | Frequently cold or warm hands, feet and face (cheeks) at unusual moments |
Endocrine dysfunction | Central precocious puberty | Girls: Tanner stage B2 < 8 years of age Boys: Testicular volume ≥4 ml < 9 years of age |
ㅤ | GH deficiency | Decreased growth velocity/short stature |
ㅤ | TSH deficiency | Fatigue, decreased growth velocity, increasing BMI |
ㅤ | ACTH deficiency | Hypoglycemia, prolonged fever, abdominal or head aches |
ㅤ | LH/FSH deficiency | Late puberty/no pubertal development |
ㅤ | Diabetes insipidus with/without adipsia | Polyuria/polydipsia |
a, Hyperphagia defined as extensive hunger/food obsession, binge-eating.
b, Behavioral problems defined as presence of obsessive compulsive symptoms (defined as fixate on certain topics, repetitive behavior or rituals, difficulty with changes), hoarding (collects items not needed), rage. To objectify behaviour, the Prader–Willi Syndrome Behavior Questionnaire may be used.
c, Sleep disorder symptoms defined as difficulty falling asleep, daytime sleepiness or falling asleep at random places during the day, regularly waking up extreme early or diagnosed with sleep apnea.
d, Temperature regulation disorder symptoms defined as presence of (episodes of) hypothermia or hyperthermia, or a story of frequently cold or warm hands, feet, and face (cheeks) at unusual moments.
Obesity and hyperphagia
Hypophagia/failure to thrive (diencephalic syndrome)
Behavioural problems (obsessive compulsive symptoms, hoarding, rage)
Spread
- Dissemination in the subarachnoid space or implantation along the surgical track or path of needle aspiration is a rare complication
Investigation
Radiology
MRI
- MRI with dedicated pituitary views in both sagittal and coronal planes routine imaging modality in assessment of children and young people with suspected craniopharyngioma
- For grading of the extent of hypothalamic involvement
- Preoperative grading of hypothalamic involvement to inform hypothalamic-sparing surgery should be performed
- Use of the most replicated grading system decreases the risk of adipsia, hyperphagia, and obesity.
- Grading (Puget et al 2007)
- Grade 0 indicates no hypothalamic involvement;
- Grade 1 indicates the tumour abutting or displacing the hypothalamus
- Grade 2 indicates that the hypothalamus is not identifiable separately from the tumour.
- Not routinely used in paeds
- DTI
- Perfusion-weighted imaging
- MRS
CT
- For diagnosis or extent of calcification
- (55–95% of craniopharyngiomas have calcification)
Ophthalmic
- Adults and children who is cooperative
- Visual fields testing
- Fundoscopy
- Acuity testing
- Infants (< 6 years of age) not cooperative
- Optic atrophy, or papilledema correlates with poorer visual outcomes
- Pattern visual evoked potentials
- Optical coherence tomography (OCT)
- Assessing retinal nerve fibre layer thinning in children and young people with more severe degrees of visual acuity or field loss
Endocrine
- Assessment of
- AFP
- β-hCG
- Prolactin
- Insulin-like growth factor 1
- to determine adrenal status
- TSH & free thyroxine (FT4)
- Be aware that deteriorating serial thyroid function tests (low or normal TSH concentrations with repeatedly low, borderline low, or falling FT4 concentrations at least 1–2 weeks apart) are sufficient for diagnosis of central hypothyroidism, without the need for a thyrotropin releasing hormone test that does not adequately discriminate between hypothalamic and pituitary causes of thyroid dysfunction
- Central hypothyroidism should be defined by the presence of a low or normal TSH with repeatedly low or falling (by >20%) FT4 concentrations
- LH/FSH, testosterone, and oestradiol
- Paired early morning plasma and urine osmolalities and electrolytes
- Morning levels of cortisol and ACTH
- If no dexamethasone has been given
- A random cortisol measurement taken before administration of any dexamethasone might be useful in documenting pretreatment status of the hypothalamic–pituitary–adrenal axis in children and young people presenting acutely with raised intracranial pressure.
- Gold standard insulin tolerance test might be substituted by the standard synacthen test (sensitivity 77–91%, positive predictive value 97–99%)30,31
- In the non-acute situation, offer combined dynamic pituitary function tests of growth hormone and cortisol reserve, and, if age-appropriate, gonadotrophin secretion when feasible and before any steroid therapy when possible
- Formal water deprivation test
- In children with polyuria and polydipsia, a water deprivation test might not always be necessary and could be hazardous
- Might help to confirm central diabetes insipidus in children and young people with a known suprasellar tumour and a history of polydipsia or polyuria where other metabolic causes have been excluded, and in the absence of an inappropriately dilute polyuria with plasma hyperosmolality (urine to plasma osmolality ratio <1·0), especially if the posterior pituitary bright spot is absent on MRI
- Coexisting central diabetes insipidus might not manifest until glucocorticoid replacement has commenced.
Neuropsychology
- Performing a formal psychological assessment at diagnosis
Hypothalamic dysfunction - Diagnostic criteria
ㅤ | Score | Total score |
Clinical criteria | ㅤ | ㅤ |
Hyperphagiaᵃ | 0 = no 1 = mild (can be controlled by parental restriction or patient itself) 2 = mild after specific intervention for hyperphagia OR severe (cannot be controlled by parents or patient itself or steals food) | 1 = minor criterion 2 = major criterion |
Hypophagia/failure to thrive (diencephalic syndrome) | 0 = none 1 = mild (can be stimulated to eat by parents/caregivers) 2 = severe (cannot be stimulated to eat by parents/caregivers or requires tube feeding for eating) | 1 = minor criterion 2 = major criterion |
Body mass index (kg/m²)ᵇ | 0 = Normal weight or overweight (BMI using the Cole criteria, or age and gender specific) 2 = Normal weight after specific intervention for hypothalamic obesity OR overweight after specific intervention for hypothalamic obesity** OR obesity (BMI using the Cole criteria** or age and gender specific) | 2 = major criterion |
Behavioral problemsᶜ | 0 = none 1 = mild (can be corrected by parents/caregivers) OR score > 22.2 on PWSBQ 2 = mild after specific intervention for hypothalamic behavioral problems OR severe (cannot be corrected by parents/caregivers, requires specialist treatment) OR score > 55.7 on PWSBQ | 1 = minor criterion 2 = major criterion |
Sleep disorderᵈ | 0 = no, Epworth Scale score 0–10 1 = mild (one or more sleep symptoms without disruption of school and/or family) OR Epworth Scale score 11–17 2 = mild after specific intervention such as melatonin OR severe (disrupts school and/or family, diagnosis of obstructive sleep apnea syndrome OR Epworth Scale score > 18) | 1 = minor criterion 2 = major criterion |
Temperature regulation disorderᵉ | 0 = no 1 = mild, core-temperature multiple times between 35 and 36 °C or above 37.5 °C 2 = severe (needs intervention such as specialized heat clothing), core-temperature measured below < 35 °C | 1 = minor criterion 2 = major criterion |
Pituitary dysfunctionᶠ | 0 = no pituitary dysfunction 1 = partial or complete pituitary dysfunction (with or without DI [with adequate thirst feeling] or SiADH) OR history of central precocious puberty 2 = severe (partial or complete) pituitary dysfunction including DI and adipsia, (inadequate thirst feeling) | 1 = minor criterion 2 = major criterion |
Radiological (MRI) criteria (for children with suprasellar tumors) | ㅤ | ㅤ |
Müller gradingᵍ | 0 = grade 0: no hypothalamic involvement or lesion; 1 = grade I: hypothalamic involvement or lesion of the anterior hypothalamus that does not involve the hypothalamic area of the mammillary bodies and beyond; 2 = grade II: hypothalamic involvement or lesion of the anterior and posterior hypothalamic area | 1 = minor criterion 2 = major criterion |
Pre-test probability (for children with no suprasellar tumors) | ㅤ | ㅤ |
Genetic syndrome or diagnosis present? | 0 = no known genetic syndrome or diagnosis associated with hypothalamic dysfunction 1 = genetic syndrome or diagnosis present which may be associated with hypothalamic dysfunction (eg, Smith–Magenis syndrome) 2 = genetic syndrome or diagnosis present of which has been proven to be associated with hypothalamic dysfunction, such as Prader–Will syndrome or ROHHADNET syndrome | 1 = minor criterion 2 = major criterion |
- Hypothalamic syndrome (HS) may be considered present in case:.
- ≥3 major criteria OR.
- “At least” four minor criteria OR.
- “At least” one minor radiological and two major OR.
- Two major and “at least” two minor OR.
- One major radiological and major obesity OR.
- One major radiological criterion and “at least” two minor criteria.
- a, Hyperphagia
- Defined as extensive hunger/food obsession, binge-eating.
- To objectify the presence of hyperphagia, the 13-item Dykens Hyperphagia Questionnaire may be used.
- b, Underweight, overweight, and obesity in
- Infant CBTS (0-2 years) is defined according to the international cut-off points of the World Health Organization using BMI < −2.0 SDS, BMI > 2.0 SDS, and BMI > 3.0, respectively.
- CBTS aged ≥2 years were defined according to the international cut-off points of Cole et al., using BMI thinness grade 2 (equal to a z-score of −2), and the international cut off points of BMI by sex and age for overweight and obesity.17,18
- *Specific intervention for hypothalamic obesity includes treatment with dextro-amphetamine, caffeine and ephedrine-HCl, mazindol, methylphenidate, octreotide, diazoxide and metformin, beloranib, exenatide, liraglutide tesomet, oxytocin and naltrexone, carbetocin or bariatic surgery.
- c, Behavioral problems
- Defined as presence of
- Obsessive compulsive symptoms (defined as fixate on certain topics, repetitive behavior or rituals, difficulty with changes),
- Hoarding (collects items not needed),
- Rage.
- Behavioral problems can be assessed using the PWS Behavioral Questionnaire (PWSBQ) or available neuropsychological investigation data may be used as a surrogate for hypothalamic dysfunction-related behavioral problems.
- d, Sleep disorder symptoms defined as difficulty falling asleep, daytime sleepiness or falling asleep at random places during the day, regularly waking up extreme early or diagnosed with sleep apnea. For further assessment, history taking, the Epworth Scale criteria, actigraphy and PSG may be used.
- e, Temperature regulation disorder
- Defined as presence of (episodes of) hypothermia or hyperthermia, or a story of frequently cold or warm hands, feet and face (cheeks) at unusual moments.
- For assessment of body temperature, core temperature may be measured during several days at fixed times.
- Grading is done according the CTC system:
- CTC grade 1: mild (a story of frequently cold or warm hands, feet, and face at unusual moments. A story of: “my child never makes fever”).
- CTC grade 2: moderate (35-36 °C or hyperthermia).
- CTC grade 3: severe (33-35 °C).
- CTC grade 4: life-threatening (<33 °C).
- f, Partial hypopituitarism
- Is defined as any anterior pituitary disorder (eg, growth hormone deficiency (GHD), thyroid-stimulating hormone deficiency (TSHD), adrenocorticotropic hormone deficiency (ACTHD), gonadotrophin-releasing hormone deficiency (GnRHD), hyperprolactinemia or syndrome of inappropriate ADH secretion (SiADH).
- g, Hypothalamic damage in children with a suprasellar brain tumor
- May be scored on MRI using the Muller grading consisting of:
- Grade 0: no hypothalamic involvement,
- Grade I: hypothalamic involvement of the anterior hypothalamus not involving the hypothalamic area beyond mammillary bodies,
- Grade II: hypothalamic involvement of the anterior and/or solely posterior hypothalamic area, ie, involving the area beyond the mammillary bodies.
Differential diagnosis
Differential for juxta/supra sellar mass
- Craniopharyngioma (20% of juxta/supra sellar masses in adults; 54% in children)
- Rathke cleft cyst (RCC)
- Non neoplastic lesion thought to represent remnants of rathke's pouch
- Primary intrasellar
- Found incidentally in 13-32 autopsies
- RCC have similar lineage to pituitary adenoma
- Rarely found together
- RCC usually have low density cystic lesion on CT
- Only half show capsular enhancement
- MRI appearance of RC is variable
- Rule of thumb: lesion with nodule in sella is usually RCC
- Similar to craniopharyngiomas because
- Rathke cleft cyst show extensive squamous metaplasia
- Both have papillae and goblet cells.
- Different to craniopharyngiomas
- Papillary: Rathke cleft cysts lack BRAFV600E mutations, although cross-reactive staining of cilia can be seen
- Adamantinomatous: Rathke cleft cysts, beta-catenin localizes to the cell membrane, whereas adamantinomatous craniopharyngiomas is in nucleus
Feature | Craniopharyngioma | RCC |
Site of origin | Anterior superior margin of pituitary | Pars intermedia of pituitary |
Cell lining | Stratified squamous epithelium | Single layer of cuboidal epithelium |
Cyst contents | Cholesterol crystals | Motor oil like fluid |
Surgical treatment | Complete resection is goal | Partial excision and drainage |
Cyst wall | Thick | Thin |
- Meningioma
- Pituitary tumour
- Germ cell tumour
- Hypothalamic glioma
- Optic glioma
- Metastasis
- Chordoma
- Parasitic infection: cysticercosis
- Epidermoid cyst
- Are distinguished by the presence of a uniloculated cavity lined by squamous epithelium and filled with flaky, dry keratin.
- Suprasellar arachnoid cyst
- Sarcoidosis
- Bone abnormalities (giant cell tumour, chondromyxoid fibroma, brown tumour or hyperparathyroidism, bone spur, extramedullary haematpoiesis
- Xanthogranulomas of the sellar region
- Histologically composed of
- Cholesterol clefts
- Macrophages (xanthoma cells),
- Multinucleated giant cells,
- Chronic inflammation,
- Necrotic debris
- Haemosiderin deposits
- A reactive lesion to remnants of Rathke cleft cyst
- Differentiating from adamantinomatous
- Adamantinomatous epithelium are usually absent or < 10% of the tissue
- Epithelial cells in xanthogranulomas do not exhibit nuclear accumulation of beta-catenin
Aetiology of the hypothalamic syndrome in children
Condition | Incidence range (per 100,000 persons per year) | % | Cohort |
Craniopharyngioma | 0.12-0.21 | 50 | Pediatric |
Germ cell tumor | 0.06-0.09 | 30 | Pediatric/adult population |
Chiasmatic hypothalamic glioma | 3.00-4.00 | 80 | Pediatric |
Rathke’s cleft cyst | 0.51%-3.5% of sellar and parasellar lesions | 20 | Pediatric/adult population |
Langerhans cell histiocytosis | 0.46-0.89 | 20 | Pediatric |
Prader–Willi syndrome | 3.30-10.00 | 100 | Pediatric/adult population |
Septo-optic dysplasia | 0.05 | 20 | Pediatric/adult population |
ROHHADNET syndrome | 100 cases reported worldwide | 100 | Pediatric/adult population |
- Conditions in which HS have been described. Modified from Muller et al. In addition to the above mentioned etiologies, hypothalamic dysfunction has also been described following cranial radiation therapy for brain tumors and following (repetitive) brain injury.
- HS, hypothalamic syndrome; ROHHADNET, Rapid-onset obesity with hypoventilation, hypothalamic, autonomic dysregulation, and neural endocrine tumor.
Differential diagnosis for 3rd ventricular lesion
- Colloid cyst
- Craniopharyngioma
- 7% of 3rd ventricle masses
- Most have punctate calcification
- Squamous epithelial rests in region of lamina terminalis are thought to give rise to these lesions
- Astrocytoma
- Teratoma
- Choroid plexus papilloma
- Cysticercosis
- Dermoid
- Choroid plexus carcinoma
- AVN
Made with Bullet