{"id":4422318,"date":"2025-01-11T13:27:05","date_gmt":"2025-01-11T19:27:05","guid":{"rendered":"https:\/\/myendoconsult.com\/learn\/topics\/understanding-pituitary-pathology\/"},"modified":"2025-01-13T06:46:37","modified_gmt":"2025-01-13T12:46:37","slug":"understanding-pituitary-pathology","status":"publish","type":"oen_topic","link":"https:\/\/myendoconsult.com\/learn\/topics\/understanding-pituitary-pathology\/","title":{"rendered":"Understanding Pituitary Pathology"},"content":{"rendered":"\n<h2 class=\"wp-block-heading\">MANIFESTATIONS OF SUPRASELLAR DISEASE<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Common Etiologies<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Tumors \/ Mass Lesions<\/strong>\n<ul class=\"wp-block-list\">\n<li>Craniopharyngioma<\/li>\n\n\n\n<li>Dysgerminoma (germinoma)<\/li>\n\n\n\n<li>Glioma (e.g., hypothalamic, optic nerve, third ventricle)<\/li>\n\n\n\n<li>Meningioma<\/li>\n\n\n\n<li>Suprasellar extension of a pituitary tumor<\/li>\n\n\n\n<li>Sellar chordoma<\/li>\n\n\n\n<li>Hamartoma<\/li>\n\n\n\n<li>Gangliocytoma<\/li>\n\n\n\n<li>Ependymoma<\/li>\n\n\n\n<li>Metastatic neoplasm<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Cystic \/ Inflammatory \/ Granulomatous Lesions<\/strong>\n<ul class=\"wp-block-list\">\n<li>Suprasellar arachnoid cyst<\/li>\n\n\n\n<li>Lymphocytic hypophysitis<\/li>\n\n\n\n<li>Granulomatous diseases (sarcoidosis, tuberculosis, <a href=\"https:\/\/myendoconsult.com\/learn\/topics\/langerhans-cell-histiocytosis\/\" data-wpil-monitor-id=\"87\">Langerhans cell histiocytosis<\/a>, Wegener granulomatosis)<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Endocrine and Non-Endocrine Sequelae<\/h3>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Visual Pathway Involvement<\/strong>\n<ul class=\"wp-block-list\">\n<li>Proximity to the optic chiasm \u2192 potential vision loss (bitemporal hemianopsia or other field defects), headaches, and recurrent emesis.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Hypothalamic Dysfunction<\/strong>\n<ul class=\"wp-block-list\">\n<li>The hypothalamus controls appetite, sleep\u2013wake cycle, water metabolism, thermoregulation, anterior pituitary function, circadian rhythms, and autonomic inputs.<\/li>\n\n\n\n<li>Mass lesions may disrupt one or multiple hypothalamic regions (preoptic, supraoptic, tuberal, mammary) or zones (periventricular, medial, lateral).<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Examples of Specific Regional Involvement<\/strong>\n<ul class=\"wp-block-list\">\n<li><strong>Posterior Hypothalamus (Mammary Region)<\/strong>: Damage \u2192 hypersomnolence.<\/li>\n\n\n\n<li><strong>Anterior Hypothalamus (Preoptic Region)<\/strong>: Hyperactivity, insomnia, altered sleep\u2013wake cycle, dysthermia (acute hyperthermia or chronic hypothermia).<\/li>\n\n\n\n<li><strong>Ventromedial Hypothalamus<\/strong>: Lesions \u2192 hyperphagia, obesity (destruction of the satiety center).<\/li>\n\n\n\n<li><strong>Lateral Hypothalamus<\/strong>: Lesions \u2192 hypophagia, weight loss, cachexia (destruction of feeding centers).<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong><a href=\"https:\/\/myendoconsult.com\/learn\/topics\/diabetes-insipidus\/\" data-wpil-monitor-id=\"88\">Diabetes Insipidus<\/a> (DI)<\/strong>\n<ul class=\"wp-block-list\">\n<li>From destruction of vasopressin-producing magnocellular neurons (supraoptic and paraventricular nuclei) or disruption of the <a href=\"https:\/\/myendoconsult.com\/learn\/pituitary-stalk-thickening\/\" data-wpil-monitor-id=\"92\">pituitary stalk<\/a>.<\/li>\n\n\n\n<li>Polydipsia\/hypodipsia from damage to osmoreceptors in anterior medial\/lateral preoptic regions.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Anterior Pituitary Dysfunction<\/strong>\n<ul class=\"wp-block-list\">\n<li>Lesions of the tuberal region \/ floor of the third ventricle \u2192 <a href=\"https:\/\/myendoconsult.com\/learn\/primary-vs-secondary-hypothyroidism\/\" data-wpil-monitor-id=\"93\">secondary hypothyroidism<\/a>, secondary adrenal insufficiency, secondary hypogonadism, and GH deficiency.<\/li>\n\n\n\n<li><strong>Hyperfunction Syndromes<\/strong>: Certain hypothalamic tumors (hamartomas, gangliocytomas, germ cell tumors) may secrete releasing hormones (e.g., GnRH \u2192 precocious puberty; CRH \u2192 Cushing syndrome; GHRH \u2192 <a href=\"https:\/\/myendoconsult.com\/learn\/topics\/acromegaly-notes\/\" data-wpil-monitor-id=\"89\">acromegaly<\/a>).<\/li>\n<\/ul>\n<\/li>\n<\/ol>\n\n\n\n<figure class=\"wp-block-image size-full\"><img loading=\"lazy\" decoding=\"async\" width=\"3450\" height=\"2700\" src=\"https:\/\/myendoconsult.com\/learn\/wp-content\/uploads\/pit-hormones-location.png\" alt=\"\" class=\"wp-image-4422775\" srcset=\"https:\/\/myendoconsult.com\/learn\/wp-content\/uploads\/pit-hormones-location.png 3450w, https:\/\/myendoconsult.com\/learn\/wp-content\/uploads\/pit-hormones-location-300x235.png 300w, https:\/\/myendoconsult.com\/learn\/wp-content\/uploads\/pit-hormones-location-768x601.png 768w, https:\/\/myendoconsult.com\/learn\/wp-content\/uploads\/pit-hormones-location-1536x1202.png 1536w, https:\/\/myendoconsult.com\/learn\/wp-content\/uploads\/pit-hormones-location-2048x1603.png 2048w\" sizes=\"auto, (max-width: 3450px) 100vw, 3450px\" \/><figcaption class=\"wp-element-caption\">Pituitary Gland and its Function<\/figcaption><\/figure>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">CRANIOPHARYNGIOMA<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Overview<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Epidemiology<\/strong>: Most common tumor in the pituitary region in children\/adolescents; ~3% of all intracranial tumors (~10% of childhood brain tumors).<\/li>\n\n\n\n<li><strong>Pathology<\/strong>: Benign epithelioid tumor from squamous remnants of Rathke pouch. Can be large (&gt;6 cm), often suprasellar, sometimes invading the third ventricle.<\/li>\n\n\n\n<li><strong>Location<\/strong>: Usually above the sella turcica, compressing the optic chiasm; can also be intrasellar, eroding the sella floor.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Clinical Presentation<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Mass Effect<\/strong>\n<ul class=\"wp-block-list\">\n<li>Vision loss (chiasm compression)<\/li>\n\n\n\n<li><a href=\"https:\/\/myendoconsult.com\/learn\/desmopressin-mechanism-of-action-diabetes-insipidus\/\" data-wpil-monitor-id=\"90\">Diabetes insipidus<\/a> (hypothalamic\/stalk invasion)<\/li>\n\n\n\n<li>Hypothalamic dysfunction (obesity, hyperphagia, hypersomnolence, temperature regulation disturbances)<\/li>\n\n\n\n<li>Anterior pituitary insufficiency (GH deficiency, hypogonadism, <a href=\"https:\/\/myendoconsult.com\/learn\/courses\/diagnosis-and-treatment-of-primary-adrenal-insufficiency\/\" data-wpil-monitor-id=\"94\">adrenal insufficiency<\/a>, hypothyroidism)<\/li>\n\n\n\n<li>Hyperprolactinemia (stalk effect)<\/li>\n\n\n\n<li>Signs of increased intracranial pressure (headache, projectile vomiting, papilledema)<\/li>\n\n\n\n<li>Hydrocephalus (obstructed CSF flow)<\/li>\n\n\n\n<li><a href=\"https:\/\/myendoconsult.com\/learn\/mnemonic-cranial-nerves-dirty-surprise\/\" data-wpil-monitor-id=\"91\">Cranial nerve<\/a> palsies (cavernous sinus involvement)<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<figure class=\"wp-block-image size-full\"><img decoding=\"async\" src=\"https:\/\/myendoconsult.com\/learn\/wp-content\/uploads\/cavernous-sinus-illustration-update-1.jpg\" alt=\"\" class=\"wp-image-3302764\"\/><figcaption class=\"wp-element-caption\">Anatomical relations of the pituitary gland<\/figcaption><\/figure>\n\n\n\n<h3 class=\"wp-block-heading\">Imaging and Histology<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Skull Radiographs\/CT<\/strong>: Irregular suprasellar calcifications.<\/li>\n\n\n\n<li><strong>MRI<\/strong>: Multilobulated cystic structure (often cholesterol-rich fluid). May be primarily suprasellar or extend intrasellar.<\/li>\n\n\n\n<li><strong>Pathology<\/strong>: Whorls\/cords of epithelial cells in a loose stellate network, sometimes with keratohyalin (adamantinomatous variant).<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Treatment and Prognosis<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Options<\/strong>: Observation (in selected cases), transsphenoidal or craniotomy resection, stereotactic radiotherapy, or combinations thereof.<\/li>\n\n\n\n<li><strong>Complications<\/strong>: Post-treatment anterior\/posterior pituitary hormone deficits are common.<\/li>\n\n\n\n<li><strong>Recurrence<\/strong>: ~40% due to tumor adherence to surrounding structures, requiring long-term follow-up.<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">EFFECTS OF PITUITARY TUMORS ON THE VISUAL APPARATUS<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Typical Visual Disturbance<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Bitemporal Hemianopsia<\/strong>\n<ul class=\"wp-block-list\">\n<li>Most frequent result of suprasellar extension.<\/li>\n\n\n\n<li>Tumor compresses the crossing nasal fibers at the central chiasm.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Variations in Chiasmal Position<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Prefixed \/ Postfixed \/ Lateral Displacements<\/strong>\n<ul class=\"wp-block-list\">\n<li>Different chiasm positions can alter the pattern of visual field defects (e.g., homonymous hemianopsia if optic tract is compressed, bilateral central scotomas if posterior chiasm is affected).<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Other Visual Defects<\/strong>\n<ul class=\"wp-block-list\">\n<li>Unilateral central scotoma, amblyopia in one eye, or inferior quadrantanopia if specific chiasm regions or optic pathways are compressed.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Recovery and Limitations<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li>If the pressure is relieved surgically or medically, vision may improve depending on the degree\/duration of tract compression.<\/li>\n\n\n\n<li>A tough diaphragma sellae or higher position of the chiasm may delay onset of visual symptoms but may allow lateral or inferior tumor extension.<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">NONTUMOROUS LESIONS OF THE PITUITARY GLAND AND STALK<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Etiologies<\/h3>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Lymphocytic Hypophysitis<\/strong>\n<ul class=\"wp-block-list\">\n<li>Autoimmune; often in late pregnancy or postpartum.<\/li>\n\n\n\n<li>Causes pituitary enlargement, headaches, hormone deficiencies (often ACTH deficiency).<\/li>\n\n\n\n<li>MRI: Homogeneous, enhancing sellar mass that may involve the stalk.<\/li>\n\n\n\n<li>Variable recovery; some have permanent deficits, others regain partial function.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Granulomatous Disorders<\/strong>\n<ul class=\"wp-block-list\">\n<li><strong>Sarcoidosis, Tuberculosis, Langerhans Cell Histiocytosis, Wegener Granulomatosis<\/strong><\/li>\n\n\n\n<li>Can involve hypothalamus, pituitary stalk, gland \u2192 hypopituitarism (including DI).<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Head Trauma<\/strong>\n<ul class=\"wp-block-list\">\n<li>Skull base fracture, stalk section, traumatic vasospasm \u2192 pituitary infarction.<\/li>\n\n\n\n<li>Leads to anterior\/posterior pituitary failure of varying degrees.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Iron Overload<\/strong>\n<ul class=\"wp-block-list\">\n<li>Hemochromatosis, hemosiderosis \u2192 iron deposition in pituitary (siderosis), often selectively impairing gonadotropin secretion.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Empty Sella Syndrome<\/strong>\n<ul class=\"wp-block-list\">\n<li><strong>Secondary<\/strong>: Post-surgical, post-radiation, or post-infarction enlargement of the sella with no substantial gland filling it.<\/li>\n\n\n\n<li><strong>Primary<\/strong>: Defect in diaphragm sellae allows CSF to enter\/enlarge the sella (may accompany benign intracranial hypertension).<\/li>\n\n\n\n<li>Often pituitary function remains intact, with the gland compressed against the sellar floor.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Genetic\/Developmental Anomalies<\/strong>\n<ul class=\"wp-block-list\">\n<li><strong>Transcription Factor Mutations<\/strong> (HESX1, LHX3, LHX4, PROP1, POU1F1, TBX19) \u2192 combined pituitary hormone deficiencies.<\/li>\n\n\n\n<li><strong>Midline Anomalies<\/strong>: Cleft palate, encephalocele, optic nerve hypoplasia; can cause <a href=\"https:\/\/myendoconsult.com\/learn\/chapters\/pituitary-gland\/\" data-wpil-monitor-id=\"332\">pituitary aplasia\/hypoplasia or ectopic gland<\/a>.<\/li>\n<\/ul>\n<\/li>\n<\/ol>\n","protected":false},"excerpt":{"rendered":"<p>MANIFESTATIONS OF SUPRASELLAR DISEASE Common Etiologies Endocrine and Non-Endocrine Sequelae CRANIOPHARYNGIOMA Overview Clinical Presentation Imaging and Histology Treatment and Prognosis EFFECTS OF PITUITARY TUMORS&hellip;<\/p>\n","protected":false},"featured_media":0,"template":"","oen_topic_chapter":[682],"class_list":["post-4422318","oen_topic","type-oen_topic","status-publish","hentry","oen_topic_chapter-pituitary-gland"],"_links":{"self":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic\/4422318","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic"}],"about":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/types\/oen_topic"}],"version-history":[{"count":6,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic\/4422318\/revisions"}],"predecessor-version":[{"id":4422781,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic\/4422318\/revisions\/4422781"}],"wp:attachment":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/media?parent=4422318"}],"wp:term":[{"taxonomy":"oen_topic_chapter","embeddable":true,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic_chapter?post=4422318"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}