Pathology Flashcards.txt

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Pathology Flashcards.txt
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  1. How are the adenohypophysis and intermediate zone formed embryologically?
    Between 3-6 weeks, Rathke's pouch arises as an in-folding of oral ectoderm that loses its connection to the early oral cavity
  2. How does the adenohypophysis tissue appear in adults?
    Epithelial
  3. How is the neurohypophysis formed embryologically?
    Between 3-6 weeks of development, the neuroectoderm from the diencephalon grows downward to meet Rathke's pouch
  4. How does the neurohypophysis tissue appear in adults?
    Neural
  5. What is major structure is superior to the Pituitary Gland?
    Hypothalamus
  6. What structure is directly above the Pituitary Gland?
    Optic Chiasm
  7. What structure is directly behind the Pituitary Gland?
    Brainstem
  8. What structure is directly in front of the Pituitary Gland?
    Sphenoid Sinus
  9. Where is the Pituitary Gland located?
    Embedded in the sphenoid bone in a little pouch known as the sella turcica
  10. What is the Pituitary Gland surrounded by laterally?
    the cavernous sinus
  11. What type of deficits may be seen with pituitary enlargement?
    • Cranial Nerve Deficits
    • Facial pain, diplopia (double vision), ptosis
  12. What artery is located close to the pituitary?
    Basilar Artery
  13. What two arteries are directly lateral to the pituitary and are a source of blood supply?
    Internal Carotid Arteries
  14. What connects the pituitary gland to the hypothalamus?
    Infundibulum (pituitary stalk)
  15. What type of axons make up the optic chiasm?
    Mylinated Axons
  16. Describe the blood supply to the adenohypophysis?
    • Main blood supply is the low pressure venous system: carries hypophysiotropic factors with it from hypothalamus to pituitary; anterior lobe hormones drain into hypophyseal veins and thus into the general circulation
    • Hypophyseal brances of ICAs: indirect supply
  17. What are the two systems within the Hypothalamohypophyseal System?
    • Supraoptic/ Paraventricular --> magnocellular
    • Median Eminence (Tuberoinfundibular) --> parvicellular
  18. What hormones in the median eminence are under anterior pituitary control (stimulating)?
    • TRH --> TSH
    • CRH --> ACTH
    • GnRH --> FSH, LH
    • GHRH --> GH
  19. What hormones are under inhibitory control within the median eminence?
    • Dopamine --> PRL (prolactin)
    • Somatostatin --> GH, TSH
  20. Where do the following hormones secreted by the anterior pituitary, have effect?
    TSH
    ACTH
    FSH
    LH
    GH
    • TSH: thyroid
    • ACTH: adrenal gland
    • FSH: follicle in ovary, spermatogenesis
    • LH: ovulation, testosterone
    • GH: affects tons of tissues
  21. How is the Adenohypophysis described histologically?
    Lobular configuration of tissue with cells arranged in nests and cords with fine vascular network
  22. Where are acidophils located within the adenohypophysis, and what hormones are secreted here?
    • Lateral Wings
    • Somatotrophs (GH) & Lactotrophs (PRL)
  23. Where are basophils located within the hypophysis, and what types of hormones do they secrete?
    • Aggregate at midline of pituitary
    • Corticotrophs (ACTH), Thyrotrophs (TSH), & Gonadotrophs (FSH, LH)
  24. What type of hormones do Chromophobes secrete within the adenohypophysis?
    • Gonadotrophs (FSH, LH)
    • Non-secretory cells
  25. What are the two systems within the Neurohypophysis?
    • Supraoptic (SO)
    • Paraventricular (PV)
  26. Neurons of the supraoptic (SO) or paraventricular (PV) nuclei produce what two hormones, which then are transmitted via fiber tracts to the posterior pituitary?
    • ADH (vasopressin)
    • Oxytocin
  27. What are the two functions of Oxytocin?
    • "Milk let-down" Reflex
    • Uterine Contractions
  28. What is the function of Vasopressin/ADH?
    Controls resorption of water in the collecting duct to facilitate urine concentration (prevents from peeing a lot)
  29. Describe the blood supply to the Neurohypophysis
    Receives blood from Internal Carotids only
  30. What hormones are produced by the Neurohypophysis?
    None -- posterior pituitary is a storage area only
  31. How does the Neurohypophysis appear histologically?
    Posterior lobe looks neural; the fibrillar appearance could be confused with a glial tumor
  32. What are three pathologies commonly seen in the Pituitary?
    • Hypersecretion (Hyperpituitarism)
    • Hyposecretion (Hypopituitarism)
    • Mass Effects
  33. What are three possible causes of Hyperpituitarism?
    • Hypothalamic Hypersecretion: rare
    • Loss of Negative Feedback: normally, target gland hormones feedback to the hypothalamus-pituitary axis
    • Autonomous Pituitary Secretion: most commonly due to functional pituitary adenomas
  34. What is the most common cause of Hyperpituitarism, and what is the most common cause of this?
    Autonomous Pituitary Secretion: most commonly due to functional pituitary adenomas
  35. This type of pituitary adenoma, seen in 10% of the population, is described as an adenoma of < 1 cm in diameter. Therefore, this adenoma type cannot produce mass effects. If these are non-secreting and have no mass effect, there is no treatment necessary.
    Microadenoma
  36. This type of pituitary adenoma can produce mass effects even if non-funcitonal. These are usually treated even if not secretory due to the possibility of mass effects.
    Macroadenoma
  37. Prolactin-producing cells in the normal gland are laterally placed and are both _______ and ________.
    • Acidophilic
    • Chromophobic
  38. What tonically inhibits prolactin release?
    Dopamine
  39. What two things can lead to hyperprolacinemia?
    • DA antagonists
    • Physical damage to teh hypothalamus/stalk
  40. What normally stimulates prolactin production? Keeping this in mind, what can lead to hyperprolactinemia?
    • TRH
    • Hypothyroidism can lead to hyperprolactinemia
  41. How do Prolactinomas typically present in women?
    • Typically microadenomas
    • Present with galactorrhoea, menstrual irregularity (amenorrhea), and infertility
  42. How do Prolactinomas typically present in men?
    • Typically macroadenomas
    • Present with galactorrhoea, visual field abnormalities, headaches, and impotence
  43. What is the most common type of pituitary adenoma?
    Prolactinoma
  44. What visual field deficit is commonly seen in Prolactinoma?
    Bitemporal Hemianopsia due to compression of crossing fibers in the optic chiasm. Can't see lateral visual field
  45. Normal somatotrophs (GH) are ________.
    Acidophilic
  46. Secretion of GH is regulated by what two hormones?
    • GHRH
    • Somatostatin
  47. GH has an indirect action on cells via what hepatic somatomedin?
    IGF-1
  48. What are some of the symptoms associated with Hypersomatotropism? How two hormones are involved?
    • Symptoms: Prepubertal -- Giantism; Postpubertal -- Acromegaly (thick skin; enlarged hands, feet, jaws, forehead)
    • Hormones: GH, PRL
  49. What are the three metabolic effects associated with Hypersomatotropism?
    • Hypertension
    • Glucose Intolerance
    • Osteoporosis
  50. Corticotrophs are ________ in the central adenohypophysis. ACTH stimulates the production of ________ hormones.
    • Basophils
    • Adrenal
  51. Excess glucocorticoids lead to characteristic signs including what 7 features?
    • Obesity
    • Hypertension
    • DM
    • Amenorrhea
    • Hirsuitism
    • Atrophy
    • Depression
  52. Excess glucocorticoids lead to charactistic signs including obesity, hypertension, DM, amenorrhea, hirsuitism, atrophy, and depression. When diagnosed clinically, what is this known as?
    Cushing Syndrome
  53. Excess glucocorticoids lead to characteristic signs including obesity, hypertension, DM, amenorrhea, hirsuitism, atrophy, and depession. When this arises from an ACTH-producing pituitary adenoma, what is it called?
    Cushing Disease
  54. Endogenous Cushing Syndrome is usually caused by what?
    Cushing Disease
  55. This is an adenoma hemorrhage and/or infarcts causing rapid expansion of the sella mass. Null cell adenomas are most prone to this phenomenon.
    Pituitary Apoplexy
  56. This syndrome has a varied presentation. Symptoms of hypothyroidism are seen, such as weight gain, cold intolerance, and fatigue. Symptoms of secondary adrenal failure can also be seen: nausea/vomiting, weakness, hypotension, and hyponatremia. In addition, decreased sex hormone production can also be seen, such as testicular atrophy and amenorrhea.
    Hypopituitarism
  57. These are adenomas that grossly invade the dura or erode the sella turcica
    Invasive Adenomas
  58. This is very rare. A diagnosis requires metastatic tumor, spread in the CSF (cord, SA space), and brain invasion.
    Pituitary Carcinoma
  59. What is a suprasellar cause of hypopituitarism?
    Germinoma
  60. 20% of all adenomas fall into this category. If clinically important, this is due to mass effects and there may be evidence of hypopituitarism.
    Null Cell Adenomas
  61. This most often results from postpartum pituitary necrosis. In this, pituitary hyperplasia during pregnancy induces a vascular vulnerability with prolonged/ excessive hemorrhage during/ after delivery. This can present with difficulties lactating in the postpartum period.
    There is secondary necrosis of the pituitary tissue due to the weak blood supply combined with oxygen deprivation due to hemorrhage during delivery.
    Sheehan Syndrome
  62. This is a syndrome resulting from herniation of leptomeninges through an enlarged sellar diaphragm. Compression of normal pituitary by arachnoid/CSF gives this syndrome the appearance responsible for its name. This is more common in obese, multiparous women.
    "Empty Cell" Syndrome
  63. Concerning Posterior Pituitary Syndromes, there are no real syndromes associated with Oxytocin. What are the syndromes associated with ADH?
    • Excess: Syndrome of inappropriate ADH (SIADH): trauma, small cell CA; can lead to significant hyponatremia
    • Deficiency: Diabetes Insipidous: can be central or peripheral and this impacts therapy with ADH analogues; hypernatremia
  64. These are paired endocrine organs
    Adrenal Glands
  65. What two things make up the Adrenal Cortex?
    • Outer Cortex
    • Inner Medulla
  66. What is the Adrenal Cortex derived from embryologically?
    Mesoderm of Urogenital Ridge
  67. Where is the yellow-brown color of the Adrenal Cortex derived from?
    Lipochrome pigments & lipid precursors (cholesterol & cholesterol esters) of steroid hormones
  68. Normal adult adrenal glands are ____ to ____ grams each.
    What effect does acute stress have on the adrenal glands?
    What effect does prolonged stess have?
    • 4-8 grams each
    • Acute Stress: lipid depletion and reduction in weight
    • Prolonged Stress: can induce hyperplasia of cortical cells and increase weight to more than twice normal
  69. What are the three layers of the Adrenal Cortex?
    • Zona Glomerulosa
    • Zona Fasciculata
    • Zona Reticularis
  70. This layer of the adrenal cortex is subscapular and narrow. It is derived of clusters of cells with scant cytoplasm and few lipid droplets
    Zona Glomerulosa
  71. This layer of the adrenal cortex is a broad middle layer. It comprises 75% of the total cortex. It is derived of columns of cells with clear cytoplasm containing lipid precursors
    Zona Fasciculata
  72. This layer of the adrenal cortex is narrow. It abuts the medulla, and is derived of clusters of more darkly staining cells with lipochrome pigments
    Zona Reticularis
  73. What steroid hormone, secreted by the zona glomerulosa, promotes reabsorption of sodium (and water) and excretion of potassium?
    Aldosterone
  74. Minaralocorticoid secretion is regulated primarily by serum ________ levels and ______-________-system (no significant response to ACTH under normal conditions)
    • Potassium
    • Renin-Angiotensin
  75. The secretion of cortisol and sex steroids is regulated by what molecule?
    ACTH
  76. What stimulates pituitary corticotrophs to secrete ACTH?
    Hypothalamic CRH (corticotrophin releasing hormone)
  77. This dysfunction of the Adrenal Cortex is characterized by cortical hyperplasia, cortical adenomas, and cortical carcinoma.
    Adrenocortical Hyperfunction
  78. This defect, seen in Adrenocortical Hyperfunction, is usually the result of pituitary hypersecretion of ACTH. It may also occur with secretion of ectopic ACTH by a nonendocrine neoplasm. It is bilateral (endogenous stimulation); and has multiple hyperplastic cortical nodules.
    Cortical Hyperplasia
  79. This defect, seen in Adrenocortical Hyperfunction, is relatively common, and results in a yellow-orange nodule of variable size. It is usually less than 30 grams, and can be functional (produces aldosterone, cortisol, or sex steroids) or non-functional. Most are nonfunctional and may not be detected clinically.
    Cortical Adenoma
  80. This defect, seen in Adrenocortical Hyperfunction, is rare. It is highly malignant, and associated with a poor prognosis. Most are functional, and are usually large and infiltrative at the time of diagnosis.
    Cortical Carcinoma
  81. What are the two diseases of the Adrenal Cortex?
    • Adrenocortical Hyperfunction
    • Adrenocortical Hypofunction
  82. This type of hypercortisolism is caused by low serum ACTH
    Primary Hypercortisolism
  83. This type of hypercortisolism is caused by high serum ACTH
    Secondary Hypercortisolism
  84. What effect does increased cortisol production have on pituitary ACTH?
    Decreases it
  85. What accounts for the majority (70%) of cases of endogenous hypercortisolism?
    Pituitary Cushing Syndrome
  86. This type of Cushing Syndrome is associated with a cortisol-secreting cortical neoplasm or bilateral nodular hyperplasia. If it is caused by a neoplasm, the contralateral adrenal becomes arrophic. This is a type of primary hypercortisolism.
    Adrenal Cushing Syndrome
  87. What are the two types of primary hypercortisolisms?
    ACTH-Independent Cushing Syndrome
  88. In this type of Cushing Syndrome, the adrenals function autonomously to produce excess cortisol independent of pituitary ACTH. The increased cortisol leads to decreased pituitary ACTH.
    Cortisol levels do not decrease with dexamethasone (synthetic glucocorticoid which normally suppresses pituitary ACTH)
    ACTH-Independent Cushing Syndrome
  89. This type of Cushing Syndrome is associated with bilateral diffuse or nodular adrenal cortical hyperplasia.
    Cortisol levels decrease with dexamethasone (synthetic glucocorticoid which suppresses pituitary ACTH)
    Pituitary Cushing Syndrome
  90. What are the two types of Secondary Hypercortisolisms?
    • Pituitary Cushing Syndrome
    • Paraneoplastic Cushing Syndrome
  91. This type of Cushing Syndrome is due to secretion of ectopic ACTH by nonpituitary tumors, e.g. small cell carcinoma of lung or other neoplasms. The adrenal glands undergo bilateral cortical hyperplasia.
    Cortisol levels do not decrease with dexamethasone (synthetic glucocorticoid which normally suppresses pituitary ACTH).
    Adrenal enlargement is usually overshadowed by a rapid downhill course related to cancer.
    Paraneoplastic Cushing Syndrome
  92. This type of Cushing Syndrome is due to administration of exogenous glucocorticoids (anti-inflammatory Rx).
    This is the most common cause of Cushing Syndrome.
    It results in bilateral adrenal cortical atrophy (mostly zona fasciculata and zona reticularis) due to suppression of pituitary ACTH.
    Iatrogenic Hypercortisolism
  93. Iatrogenic Hypercortisolism is associated with _____ serum ACTH
    Low
  94. What hormone serves to:
    Increase appetite
    Promote fat deposition (face, neck, and trunk)
    Induce gluconeogenesis & inhibit uptake of glucose by cells with resultant hyperglycemia, glucosuria, & polydipsia
    Increase protein breakdown causing loss of collagen & bone resorption
    Impair inflammatory response
    Glucocorticoids (Cortisol)
  95. What syndrome is associated with moon facies, buffalo hump, truncal obesity, abdominal striae, hirsutism, impaired glucose tolerance, proximal muscle weakness, osteoporosis, menstrual disorders, emotional lability, easy bruisability, and susceptibility to infection?
    Cushing Syndrome
  96. Adrenogenital or virilizing syndromes are due to excess _________.
    Androgens
  97. What regulates adrenal androgen formation?
    ACTH
  98. What two things can cause excess ACTH production?
    • Adrenocortical Neoplasms (usually carcinomas)
    • Congenital Adrenal Hyperplasia
  99. This is a group of inherited metabolic errors each characterized by deficiency or lack of a particular enzyme in the biosynthesis of cortical steroids, particularly cortisol.
    Steroidogenesis is channeled into other pathways, leading to increased androgens and virilization.
    Congential Adrenal Hyperplasia
  100. In Congenital Adrenal Hyperplasia, various metabolic errors all:
    Block or partially block the production of _________ and shunt the steroid pathway toward _________ production.
    Decrease the ___________ ___________ on the pituitary.
    Increase serum _________.
    Lead to bilateral cortical _________ and increased synthesis of __________.
    • Cortisol; Androgen
    • Feedback Inhibition
    • ACTH
    • Hyperplasia; Testosterone
  101. A deficiency in what enzyme shunts the steroid pathway away from cortisol and toward androgen production? This is the most common cause of Congenital Adrenal Hyperplasia (90% of cases). The deficiency may range from partial to total.
    Virilizing-21 Hydroxylase Deficiency
  102. What does a Virilizing-21 Hydroxylase Deficiency cause in females? In males?
    • Ambiguous Genitalia
    • Precocious Puberty & Exaggerated Secondary Sex Characteristics
  103. What does a total lack of Virilizing-21 Hydroxylase cause?
    • Deficient cortisol synthesis
    • Interferes with aldosteron production
    • Salt wasting leads to hyponatremia and hyperkalemia
  104. A deficiency in this enzyme shunts the steroid pathway away from cortisol and towards androgens. It also causes a buildup of an aldosterone precursor which is a potent mineralocorticoid.
    This results in hypernatremia, hypokalemia, and hypertension.
    Lack of 11-hydroxylase
  105. Hyperaldosteronism is a chronic excess in mineralocorticoids due to excess aldosterone secretion. What two metabolic derangements does this cause?
    • Sodium Retention (hypernatremia & hypertension)
    • Potassium Secretion (hypokalemia, hypokalemic alkalosis, & increased urine potassium)
  106. This is hyperaldosteronism due to autonomous overproduction of aldosterone with suppression of the renin-angiotensin system and decreased plasma renin activity. This may be related to an adrenocortical neoplasm (commonly an adenoma, rarely a carcinoma) or it may be related to an idiopathic bilateral adrenocortical hyperplasia
    Primary Hyperaldosteronism
  107. This is hyperaldosteronism due to activation of the renin-angiotensin system resulting in increased plasma renin. It is encountered in conditions related to: renal hypoperfusion (ischemia), arterial hypovolemia, renin producing tumors (e.g. some renal cell carcinomas).
    Clinical manifestations include hypernatremia, hypokalemia, and high serum renin
    Secondary Hyperaldosteronism
  108. What are the clinical manifestations associated with primary hyperaldosteronism?
    • Hypernatremia: hypertension
    • Hypokalemia: chronic fatigue, neuromuscular symptoms (weakness, rarely paralysis), and cardiac arrhythmias
    • Decreased Renin production from kidney and LOW SERUM RENIN
  109. This syndrome is most frequently seen in middle-aged females. It is usually caused by a solitary aldosterone-secreting adenoma <2 cm in size. The remainder of cases are associated with bilateral cortical hyperplasia.
    Conn Syndrome (primary hyperaldosteronism)
  110. Unlike cortical adenomas associated with Cushing Syndrome, aldosterone-secreting adenomas do not usually suppress ______ secretion. Therefore, the adjacent adrenal cortex and contralateral adrenal are not __________.
    • ACTH
    • Atrophic
  111. What are the clinical manifestations associated with secondary hyperaldosteronism?
    • Hypernatremia
    • Hypokalemia
    • High Serum Renin
  112. This may be caused by acute or chronic adrenocortical insufficiency, or by decreased stimulation of the adrenals due to deficiency of ACTH.
    Hypoadrenalism
  113. This form of Hypoadrenalism results from progressive destruction of the adrenal cortex. Clinical manifestations of insufficiency require compromise of at least 90% of adrenal cortex and significantly decreased levels of glucocorticoids and mineralocorticoids.
    Clinical manifestations of this are insidious in onset, as there must be a significant decrease in glucocorticoids and mineralocorticoids. Progressive weakness and fatigue are seen, along with GI problems including anorexia, weight loss, N&V, diarrhea, hypotension, adn hyperpigmentation of skin.
    Primary Chronic Adrenocortical Insufficiency -- Addison's Disease
  114. What symptoms are associated with Addison Disease (primary chronic hypoadrenalism)?
    • Progressive weakness & fatigue
    • GI symptoms -- norexia, weight loss, N&V, diarrhea
    • Hyperpigmentation of skin
    • Hyponatremia
    • Hyperkalemia
    • Volume Depletion
    • Hypotension
    • Hypoglycemia (caused by decreased glucocorticoid levels)
  115. What causes the hyperpigmentation of skin seen in Addison Disease (primary hypoadrenalism)?
    Increased levels of ACTH precursor, pro-opiomelanocortin, that stimulates melanocytes
  116. How is the diagnosis of Addison Disease (primary hypoadrenalism) made?
    • Decreased serum and urine cortisol
    • Lack of adrenal response to ACTH stimulation
  117. More than 90% of cases of Addison Disease are attributed to what four disorders?
    • Autoimmune Adrenalitis
    • Tuberculosis
    • AIDS
    • Metastatic Cancers
  118. Autoimmune adrenalitis may be idiopathic and occur as an isolated disease, or it may occur in association with other autoimmune diseases as part of a sporadic or inherited clinical syndrome. Which accounts for the majority (60-70%) of cases?
    Idiopathic
  119. The majority of patients with Addison Disease associated with autoimmune adrenalitis have autoantibodies to __________ _________ resulting in autoimmune destruction of steroidogenic cells and autoantibodies to key steroidogenic enzymes.
    Adrenal Antigens
  120. Autoimmune adrenalitis related Addison Disease may occur in association with other autoimmune diseases as part of a sporadic or inherited clinical syndrome. What are two such syndromes?
    • Autoimmuo Polyendocrine Syndrome type I (APS1)
    • Autoimmuo Polyendocrine Syndrome type II (APS2)
  121. This form of Autoimmune related Addison Disease is characterized as:
    autoimmune adrenalitis
    autoimmune hypoparathyroidism
    autoimmune disease involving other organs
    mucocutaneous Candidiasis
    abnormalities of skin, dental enamel, and nails
    ? defect in T suppressor cell function
    Autoimmune Polyendocrine Syndrome Type I (APS1)
  122. This form of Autoimmune related Addison Disease is associated with:
    autoimmune adrenalitis
    autoimmune thyroid disease and/or insulin-dependent diabetes
    associated with polymorphisms in HLA loci
    Autoimmune Polyendocrine Syndrome Type II (APS2)
  123. This form of hyopadrenalism is caused by decreased stimulation of adrenals due to ACTH deficiency associated with various disorders of hypothalamus or pituitary. These disorders may include cancer, infection, infarction, or irradiation involving hypothalamus or pituitary.
    Secondary Hypoadrenalism -- Decreased Pituitary ACTH
  124. In secondary Hypoadrenalism, the decreased ACTH secretion from the pituitary and decreased serum ACTH lead to:
    • Decreased Cortisol and Androgens
    • Normal or near normal Aldosterone Synthesis (no hyponatremia or hyperkalemia)
    • Lacks cutaneous hyperpigmentation of primary Addison's because levels of ACTH percursor (pro-opiomelanocortin) are not elevated
  125. How is secondary Hypoadrenalism diagnosed?
    • Low serum ACTH
    • Exogenous administration of ACTH leads to rise in serum cortisol
  126. A primary acute adrenocortical insufficiency occurs in a variety of clinical settings: it can occur as a crisis in patients with chronic adrenocortical insufficiency precipitated by stress (trauma, infection, surgery, etc.). When this happens, the adrenal glands are incapable of responding with an increase in steroid output. This crisis may result in hypotension, shock, and vascular collapse. What is it called?
    Addisonian Crisis
  127. A primary acute adrenocortical insufficiency occurs in a variety of clinical settings: is can occur in patients maintained on exogenous corticosteroids. This may be precipitated by a rapid withdrawal of steroids or failure to increase steroid doses in response to acute stress. This in turn may precipitate a crisis as a result of an inability of atrophic adrenals to produce glucocorticoid hormones. What is this called?
    Iatrogenic Crisis
  128. A primary acute adrenocortical insufficiency occurs in a variety of clinical settings: it can occur as a result of a massive ___________ ___________. This may destroy the adrenal cortex as a result of: trauma (esp in newborns with prolonged & difficult labor), in patients maintained on anti-coagulant therpay, in postsurgical patients who develop disseminated intravascular coagulation (DIC), or as a complication of bacteremia.
    Adrenal Hemorrhage
  129. This is an uncommon catastrophic syndrome that can occur at any age but is somewhat more common in children. It is comprised of an overwhelming bacterial infection due to Neissseria Meningitidis or other organisms. Patients develop DIC with widespread purpura, particularly of the skin. There is massive bilateral adrenal hemorrhage that leads to rapid adrenocortical insufficiency. The massive bilateral adrenal hemorrhage converts the glands to sacs of clotted blood. Patients develop rapidly progressive hypotension leading to shock. This is fatal if therapy is not instituted immediately -- death follows within hours to a few days.
    Waterhouse-Friderichsen Syndrome
  130. These cells are present within the adrenal medulla, and other endocrine organs, and are widely dispersed. They may be derived from cells that migrated from the neural crest to endocrine glands during embryogenesis, from cells programmed by the neural crest, or from non-endocrine tissues during neoplastictransformation.
    Neuroendocrine Cells
  131. Tumors derived from ________ cells may present as mass lesions (nonfunctional) or may produce hormones or other substances with functional activity.
    Neuroendocrine
  132. What endocrine disease is often associated with the thyroid gland? It is derived from parafollicular cells or neuroendocrine cells that secrete calcitonin. The neuroendocrine cells form nests, and acellular deposits of amyloid (derived from altered calcitonin polypeptides) may be present in adjacent stroma. This can occur sporadically, or in association with other endocrine neoplasms as part of Multiple Endocrine Neoplasia (MEN) Syndrome.
    Medullary Carcinoma of the Thyroid
  133. This is a group of genetically inherited diseases resulting in proliferative lesions (hyperplasias, adenomas, and carcinomas) of multiple endocrine organs.
    Multiple Endocrine Neoplasia (MEN) Syndrome
  134. This structure is embryologically, functionally, and structurally distinct from the adrenal cortex. It is composed of specialized neural crest (neuroendocrine) cells termed chromaffin cells. This is normally a major source of catecholamines, and also contains a variety of other bioactive amines and peptides.
    Adrenal Medulla
  135. The adrenal medulla is a major source of what hormones?
    Catecholamines (norepinephrine and epinephrine)
  136. The adrenal medulla is composed of two cell types derived from the neural crest. What are they? What are the two important neoplasm types associated with the adrenal medulla?
    • Ganglion Cells & Pheochromocytes
    • Neoplasms derived from Ganglion Cells (neuroblastic tumors) & Pheochromocytomas
  137. This family of tumors are derived from embryonal neural crest cells. This is a common childhood tumor, most common in infants and children <5 (median age of diagnosis is 18 months). Most cases arise in the adrenal medulla or along the sympathetic ganglia in the abdomen or posterior mediastinum.
    Neuroblastomas
  138. What is the typical presentation of a neuroblastoma?
    • Generally present as abdominal mass, weight loss, malaise, fever in children < 2 yo.
    • Older children may present with effects of metastases to bone, lung, or GI tract (e.g. bone pain, respiratory symptoms, GI problems)
  139. What do most neuroblastomas secrete (90%)?
    • Catecholamines
    • There are elevated blood catecholamines and elevated urinary catecholamine metabolites
  140. Are patients with neuroblastomas typically hypertensive?
    No
  141. How is a laboratory diagnosis of Neuroblastoma made?
    Elevated metabolic catecholamine by-products in urine: metanephrine, normetanephrine, vanillylmandelic acid (VMA), homovanilic acid (HVA)
  142. Grossly, this is usually large, soft, gray, and relatively well circumscribed. It has areas of hemorrhage, necrosis, and cystic change often present. It may invade adjacent structures (kidneys, renal vein, vena cava).
    Histologically, it has "small, blue, round cell tumors" along with Homer-Wright Rosettes.
    Neuroblastoma
  143. What are the two neuroblastomas that demonstrate potential for spontaneous or therapy-induced differentiation of primitive neuroblasts into mature elements? (spontaneous tumor regression is rarely reported in infants)
    • Ganglioneuroblastoma
    • Ganglioneuroma
  144. What is the typical course for neuroblastomas in children <2? In older children?
    • Tumors remain localized and are amenable to complete resection
    • Tumors are often unresectable and may present with widespread metastases (liver, lung, and bone marrow are common sites)
  145. What is the prognosis of neuroblastomas dependent on?
    • Age & Stage
    • Children < 18 mo have excellent prognosis regardless of stage
    • Children > 5 yr have guarded prognosis regardless of stage
  146. This is a tumor that is composed of adrenal medullary cells that synthesize & release catecholamines. Patients often present with hypertension (dominant clinical manifestation in 90% of pts).
    Pheochromocytoma
  147. Patients with this tumor type commonly demonstrate chronic sustained elevation in BP punctuated by paroxysmal episodes of an abrupt increase in BP associated with tachycardia, palpitations, headache, sweating, tremor, and anxiety. Cardiac complications may include congestive heart failure, pulmonary edema, myocardial infarction, and cerebrovascular accidents
    Pheochromocytoma
  148. How are Pheochromocytomas diagnosed?
    Elevated urinary catecholamine metabolites: vanillylmandelic acid (VMA) & metanephrine
  149. What is the clinical significance of Pheochromocytomas?
    They are a surgically correctable form of hypertension
  150. What is the "10% tumor"?
    10% extra-adrenal
    10% of sporadic adrenal ones are bilateral in adrenal glands
    10% biologically malignant
    10% familial -- inherited as AD disease or in association with MEN syndrome
    Pheochromocytomas
  151. Histologically, these are arranged in well-defined (Zellballen) with a rich vascular network.
    Pheochromocytomas

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