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Pheochromocytoma

By Amber Stephens,2014-06-13 19:41
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Pheochromocytoma

    Cause/Risk Factors Symptoms Buzzwords Other

    (sddianjian)

    ()

    Disease

    ? Cortisol 55-75% of endogenous cases, more common in women Pituitary Hypersecretion of ACTH Obesity, Moon Face, Buffalo Hump, Osteoporosis Moon Face Diffuse Bilateral Adrenal Hyperplasia w/ thick yellow cortex Cushings Disease (usually a microadenoma) Hirsutism, ? Infections, HT, Thin Skin, Buffalo Hump Suppression by High Dose Dexamethasone Easy Bruising, Red Stria & Muscle Weakness Inferior Petrosal Sinus gradient of 3:1

    Nonpituitary Neoplasm Secretion ? Cortisol Most common in men Cushings Syndrome (Small Carcinoma, Bronchial & Obesity, Moon Face, Buffalo Hump, Osteoporosis Small Cell Carcinoma Diffuse Bilateral Adrenal Hyperplasia w/out nodularity (Ectopic ACTH or CRH) Pancreatic Carcinoids, Thymoma, Hirsutism, ? Infections, HT, Thin Skin, ? of Bronchial Carcinoids suppressed by ? dose dexamethasone Pheochromocytoma, Gastrinoma) Easy Bruising, Red Stria & Muscle Weakness

    ? Cortisol Adrenal Adenoma Cushings Syndrome Obesity, Moon Face, Buffalo Hump, Osteoporosis ? CRH & ACTH Adrenal Carcinoma ? CRH & ACTH (Adrenal Hypersecretion) Hirsutism, ? Infections, HT, Thin Skin, Atrophy of adjacent cortex & contralateral adrenal cortex Adrenal Nodular Hyperplasia Easy Bruising, Red Stria & Muscle Weakness

    ? Cortisol Most common source is Prednisone Cushings Syndrome Obesity, Moon Face, Buffalo Hump, Osteoporosis Exogenous Cortisol Excess Prednisone ? Pituitary ACTH & Small Adrenal Glands with thin cortex (Exogenous Cortisol Excess) Hirsutism, ? Infections, HT, Thin Skin, Zona Glomerulosa not affected Easy Bruising, Red Stria & Muscle Weakness

    ? Cortisol Pseudo-Cushings Syndrome Depression, Alcoholism, Obesity, Obesity, Moon Face, Buffalo Hump, Osteoporosis (Temporary Cortisol Excess) Eating Disorder, Stress & Illness Hirsutism, ? Infections, HT, Thin Skin, Easy Bruising, Red Stria & Muscle Weakness

    ? Aldosterone Primary Aldosteronism HT, ? Serum KAldosterone secreting Adenoma (? Renin Conn’s Syndrome)

    Small yellow encapsulated tumor found usually on left & in ? +, ? Extracellular fluid, Alkalosis ? Renin & ? Aldosterone Low Plasma Renin Activity (PRA) Weakness, Paesthesias, Visual Disturbances Aldosterone > 10 ng/dL after IV Saline (Saline Suppression Test) Headaches, Tetany, Cardiac decompensation

    ? Aldosterone Bilateral idiopathic hyperplasia = less common than Conn’s Primary Aldosteronism HT, ? Serum K+Idiopathic , ? Extracellular fluid, Alkalosis Treat w/ spironolactone & something for HT (? Renin Adrenal Hyperplasia) ? Renin & ? Aldosterone Weakness, Paesthesias, Visual Disturbances Low Plasma Renin Activity (PRA) Headaches, Tetany, Cardiac decompensation Aldosterone > 10 ng/dL after IV Saline (Saline Suppression Test)

    ? Aldosterone Renal Artery Stenosis Secondary Aldosteronism HT, ? Serum K+Renin producing tunor , ? Extracellular fluid, Alkalosis (? Renin) ? Renin & ? Aldosterone High Plasma Renin Activity (PRA) Chronic edema Weakness, Paesthesias, Visual Disturbances Headaches, Tetany, Cardiac decompensation

    Black Licorice ? Aldosterone Hyporenemic Hypoaldosteronism Chewing Tobacco Hypertension

    ? ACTH & DHEA ? virilization Most common cause of CAH & of ambiguous genitalia Congenital Adrenal Hyperplasia 21-Hydroxylase Deficiency “Classic” = ? Aldosterone & Cortisol; ? Renin Hallmark is increased 17-Hydroxyprogesterone (21-Hydroxylase Deficiency) (CYP21A2 Deficiency) “Classic w/out wasting” = nl Ald & ? Cortisol Treatment = replace aldosterone & cortisol “Non-classical” = nl Aldosterone & Cortisol

    ? ACTH & DHEA ? virilization Congenital Adrenal Hyperplasia ? Deoxycortisone 11?-Hydroxylase Deficiency (11?-Hydroxylase Deficiency) ? Aldosterone, Renin & Cortisol

    ? Aldosterone & ACTH Congenital Adrenal Hyperplasia ? Cortisol & Sex Steroids 17?-Hydroxylase Deficiency

    Adrenal Pathology

    Adrenal Pathology (contd.)

    Disease Cause/Risk Factors Symptoms Buzzwords Other

    Immediate steroid need 1) Immediate need for steroids glands unable to respond Cortical Hypofunction Rapid steroid withdrawal ? ACTH 2) Rapid Withdrawal of steroids (Primary Acute Insufficiency) Mass Destruction (DIC,Hemorrhage) 3) Massive destruction hemorrhage, anticoagulants, DIC Waterhouse-Friderichsen Bacteremic Infection Adrenal Hemorrhage ? Cortical Hypofunction ? ACTH More common in children Syndrome

    Autoimmune (majority) Destruction of adrenal cortex only Cortical Hypofunction TB, Histoplasmosis, Carcinoma Fatigue, muscle weakness, weight loss, GI upset (Primary Chronic Insufficiency) ? ACTH Thin cortex w/ lymph infiltrates unaffected medulla AIDS, Amyloidosis, Sarcoidosis Hypoglycemia, Salt craving, Prerenal Azotemia (Addison’s Disease) Hemochromatosis Acidosis, Hypotension & Hyperpigmentation

    Cortical Hypofunction Autoimmune (majority) (Primary Chronic Insufficiency) TB, Histoplasmosis, Carcinoma 1) Adrenal insufficiency same as above ? ACTH Thin cortex w/ lymph infiltrates unaffected medulla (Addison’s Disease) AIDS, Amyloidosis, Sarcoidosis 2) Hypoparathyroidism & Candidiasis Subtype I Hemochromatosis

    Cortical Hypofunction Autoimmune (majority) (Primary Chronic Insufficiency) TB, Histoplasmosis, Carcinoma 1) Adrenal insufficiency same as above ? ACTH Thin cortex w/ lymph infiltrates unaffected medulla (Addison’s Disease) AIDS, Amyloidosis, Sarcoidosis 2) Autoimmune thyroid disease & Type I DM Subtype II Schmidt’s Syndrome Hemochromatosis

    Cortical Hypofunction Congenital Adrenal Hyperplasia (Primary Chronic Insufficiency) ? ACTH Enzyme Inhibitor (ex. = ketoconazole) (Failure of Cortisol Production)

    Carcinoma, Infection, Irradiation, No aldosterone or ACTH deficiency Cortical Hypofunction Fatigue, muscle weakness, weight loss, GI upset Infarction Features of panhypopituitarism (Chronic 2º/3º Insufficiency) Hypoglycemia, Salt craving, Prolonged use of steroids?? ACTH Delayed response to prolonged ACTH stimulation test

    HT with or without paroxysmal attacks Most are solitary & in medulla; pink colored; large = encapsulated Headaches, sweating, fever, GI upset, anxiety Diagnosis = Normetanephrine & metanephrine in plasma Pheochromocytoma MEN Type II or III Adrenomedullary Chromafin cells Palpitations orthostatic HT, numbness Clonidine useful in exclusion; Paroxysms can be provoked Cardiac manifestations Rule of 10’s = 10% extrarenal, bilateral, malignant, familial

    Parathyroid hyperplasia MEN Syndrome Type I Pituitary adenomas (Wermer Syndrome) Pancreatic islet tumors

    Pheochromocytoma MEN Syndrome Type II MEN IIA = Parathyroid Adenoma Medullary Thyroid carcinoma (Sipple Syndrome) MEN IIB = Mucocutaneous ganglioneuromas & Marfanoid habitus Parathyroid hyperplasia

    Pheochromocytoma MEN Syndrome Type III Medullary Thyroid carcinoma Mucosal neuromas/Marfanoid features

    Endocrine Pancreas Pathology

    Disease Cause/Risk Factors Symptoms Buzzwords Other

    Blindness (Lens, Retina, Optic Nerve) Genetic (HLA-DR3 &4) MI is #1 cause of death; Diabetic ketoacidosis Primary Diabetes Mellitus CV disease (MI, stroke, gangrene) HLA-DR3&4 Autoimmune Antibodies Glycosylated collagens ?Advanced Glycosylation End Products Type I Nephropathy & Infections (Mucomycosis) Diabetic ketoacidosis Environment (Viral Infection) Intracellular hyperglycemia?Sorbitol (lens & Schwann Cell) Peripheral Neuropathy, Polydipsia & Polyuria

    Genetic (not HLA related) Blindness (Lens, Retina, Optic Nerve) MI is #1 cause of death; Hyperosmolar Coma Primary Diabetes Mellitus Obesity CV disease (MI, stroke, gangrene) Hyperosmolar Coma Glycosylated collagens ?Advanced Glycosylation End Products Type II Insulin Deficiency Nephropathy & Infections (Mucomycosis) Intracellular hyperglycemia?Sorbitol (lens & Schwann Cell) Insulin Resistance (receptor & GLUT) Peripheral Neuropathy, Polydipsia & Polyuria

    Pancreatitis Secondary Diabetes Mellitus Pancreatic Cancer Destruction of islets after disease Drugs

    Thyroid Pathology

    Disease Cause/Risk Factors Symptoms Buzzwords Other Dietary Hyperplastic enlargement of thyroid Diffuse Non-Toxic Goiter ? iodine Involves whole gland ? T3/T4 (Endemic) goitrogens More common ? TSH

    Hyperplastic enlargement of thyroid Diffuse Non-Toxic Goiter Substance interfering with synthesis ? T3/T4 Young females Typically young females (Sporadic) Hereditary enzyme defect ? TSH

    Hyperplastic enlargement of thyroid ? T3/T4 & ? TSH Nodules/Cysts Multinodal Goiter Hemorrhage, scarring & calcification Recurrent hyperplasia & involution Irregular Irregular with Nodules & Cysts

    Toxic multinodal goiter Plummer’s Syndrome Hyperfunctioning Goiter ? T3/T4 No skin/eye changes of Grave’s Disease

    Thyrotoxicosis (with diffuse hyperplasia) Typically females Autoimmune (LATS/TSI & TGI) Graves Disease Ophthalmopathy (Paralysis & exopthalmos) Treatment: PTU, methimazole, radioiodine & surgery Genetic? (HLA-B8 & DR3) Dermopathy (Pretibial myxedema) Eye Muscle Paralysis & Exopthalmos NOT seen in Thyrotoxicosis

    Low TSH (suggestive) & High T4 (confirms) Cardiac: ?HR,A-fib,Cardiomegaly, CHF Hyperfunctioning Thyroid Heat Intolerance Hyperpigmentation over extensor surfaces Neuromuscular: Hyperreflexia,tremor,wasting Thyrotoxicosis Leakage from Thyroid Lid Lag Uncommon causes: Hydatiform mole, Struma ovarii, carcinomas Skin: Heat Intolerance, ? Sweat & Pigmentation Ingestion of iodide or Synthroid ? Cholesterol ? Appetite, GI motility, Osteoporosis, Eyes Treatment: ? Blockers, Propylthiuracil (PTU) , Iodide, Ablation

    Insufficient Parenchyma Anemia, ? Cholesterol, ? NaHashimoto Thyroiditis (Autoimmune) Primary Hypothyroidism Developmental, Radiation Ablation , Myxedema +High TSH (suggestive) & Low Free T4 (confirms) Cold Intolerance, Fatigue, Depression, Lethargy ? Cholesterol Therapy = T4 replacement (TSH takes 6-8 weeks to normalize) Weight ?, Edema, Constipation, Cardiomyopathy Myxedema Low TSH after Tx ? osteoporosis & arrythmias Delayed reflexes (achilles) & Coma (If severe)

    TSH is unreliable; Use T4 to make diagnosis Seondary Hypothyroidism Pituitary Lesion TRH stimulation Test = no response

    TSH is unreliable; Use T4 to make diagnosis Tertiary Hypothyroidism Hypothalamic Lesion TRH stimulation Test = delayed response (60 min)

    ? Synthesis Idiopathic (block of TSH receptors Hypothyroidism - Other Use increased dose of T4 during pregnancy Heriditary, Hashimoto Thyroiditis Lithium, Iodides, p-aminosalicylate

    Mental Retardation Infant Hypothyroidism Short Stature, Coarse Facial Features Protruding Tongue Cretinism Early Childhood Hypothyroidism Umbilical Hernia Umbilical Hernia Protruding Tongue

    Delayed Brain Development Thyroid Agenesis Deafness & Mutism Fetal Hypothyroidism Iodine Deficiency Deaf & Mute Spasticity Congenital Synthetic Defect Severe Mental Retardation

    Thyroid Pathology (contd)

    Disease Cause/Risk Factors Symptoms Buzzwords Other

    TPO Antibodies (99% sensitive) Female Predominance Diffusely but painlessly enlarged Thyroid Chronic Autoimmune Thyroiditis Deficiency of Suppressor T Cells Early transient Thyrotoxicosis Early transient Thyrotoxicosis, Later Hypothyroidism Lymph infiltrates & follicles (Hashimoto Thyroiditis) Genetic? (HLA-DR3 &5) Later Hypothyroidism Risk for B Cell Lymphomas Hurthle Cells (granular cytoplasm) TSH antibodies blocking receptor Other antibodies: Thyroglobulin, Thyroid Peroxidase, I transporter

    Subacute Thyroiditis Painful, diffusely enlarged Thyroid Viral (Mumps, Influenza, Coxsackie) Painful Self Limited (DeQuervain’s/Granulomatous) Giant Cells & Macrophages (like granuloma)

    Vigorous Palpation Palpation Thyroiditis Giant Cells & Macrophages (like granuloma) Disuption of follicles

    Subacute Lymphocyte Self Limited Idiopathic Painless Goitrous Enlargement Post-Partum Thyroiditis Often seen in post-partum women

    Causes Laryngeal Nerve Paralysis (SOB & difficulty swallowing) Fibrosing Thyroiditis - Painless Riedel’s Thyroiditis Simulates malignancy Hard gland May be associated with fibrosis elsewhere

    Majority of Thyroid neoplasms Benign, solitary, encapsulated Follicular Adenoma Not likely to cause thyrotoxicosis Doesn’t take up iodine May make more T3 than T4

    Encapsulated or Infiltrating Most common thyroid cancer ? incidence in Gardner Syndrome RET gene mutation (MEN II) Radiation Complex branching papillae Optically clear nuclei in “Little Orphan Annie Cells” Papillary Carcinoma RAS gene mutation Little Orphan Annie Optically Clear nuclei & Psammoma bodies Metastisize to regional nodes; Usually indolent growth Radiation exposure (most common) Nodal Metastasis Intranuclear inclusions, grooves Treatment = Thyroidectomy & radioiodine therapy, Survival 98%

    2Invasion Follicular Carcinoma RAS gene mutation Vascular or Capsular Invasion Blood Metastasis

    nd most common - Survival 92% Hematogenously spread to bone, lungs & brain Treatment lobectomy or thyroidectomy, radioiodine (if invasive)

    Hurthle Cell Neoplasm RAS gene mutation Abundent granular cytoplasm Behave the same as follicular cell carcinoma/adenoma (Adenoma or Carcinoma)

    Parafollicular C Cells (neuroendocrine) Rare most sporadic, 20% as MEN II or familial Amyloid Stroma RET gene mutation (MEN II) Nodule or multifocal May be paraneoplastic (CEA, somatostatin, serotonin & VIP) Medullary Carcinoma Calcitonin RAS gene mutation Not encapsulated, round or spindle cells Metastasize in blood to lung, liver, bone & nodes Not encapsulated Amyloid( Calcitonin) tumor stroma Treatment = Thyroidectomy

    Poor prognosis uniformly fatal RAS gene mutation Undifferentiated neoplasms Elderly Loss of I uptake & ? Thyroglobulin Anaplastic Carcinomas P53 gene mutation Large, locally invasive, rapidly growing Fatal Survival less than 1 year from diagnosis Possibly derived from Papillary Carcinoma

    Leukemia & Lymphoma

    Disease Cause/Risk Factors Symptoms Buzzwords Other Trisomy 21, Bloom’s, Fanconi’s Rapid Onset, > 20% Blasts Cells similar to lymphoblastic lymphoma Children Acute Lymphoblastic Leukemia Radiation Bone Pain, CNS & Testes Involvement Generally good prognosis (70%) = ? WBC, t12:21, < 9yo, diploidy TdT+, (ALL) Alkylating Agents, Benzene HSmegaly, Anemia & Thrombocytopenia Bad = WBC (>10K), T-Type, t9:22, 11q23, organomegaly Scant agranular cytoplasm Viral T Type = lymphadenopathy & mediastinal mass 3 types: Pre B (85%), Pre T (15%), B “like Burkitts” (2%) Trisomy 21, Bloom’s, Fanconi’s Rapid Onset, > 20% Blasts Fair prognosis (40%), Usually adults Auer rods Acute Myeloid Leukemia Radiation Anemia & Thrombocytopenia Frequent infections & mucosal bleeding CD13, CD15, CD33 (AML) Alkylating Agents, Benzene Monocytic = gum hyperplasia Good = t8:21, t15:17 (treat w/ RA) & Chrom 16 abnormalities Granular Cytoplasm Viral Promyelocytic (M3) = DIC, ?WBC Bad = t9:22, 11q23, origin from myelodysplasia or treatment Trisomy 21, Bloom’s, Fanconi’s Acute Leukemia Radiation Myeloid & Lymphoid features Poor prognosis of Ambiguous Lineage Alkylating Agents, Benzene Viral

    Trisomy 21, Bloom’s, Fanconi’s Gradual onset, Mature Cells Usually adults, Philadelphia Chromosome (t 9:22) ? LAP Chronic Myeloid Leukemia Radiation 100% cellular Bone Marrow (no fat) bcr-abl ? Tyrosine Kinase activity ( Treat with Gleevec) t9:22 or bcr-abl (CML) Alkylating Agents, Benzene Pluripotent Stem Cells?Granulocytes ? Leukocyte Alkaline Phosphatase (LAP) Pluripotent Stem Cells Viral Splenomegaly = Draggin Sensation Poor Prognosis - Always goes to blast crisis (AML or ALL)

    Gradual onset, Mature Cells, Hepatosplenomegaly Not radiation Usually adults (most common adult leukemia in west) Trisomy 21, Bloom’s, Fanconi’s Chronic Lymphocytic Leukemia Clonal B Cell Disorder (small lymphocytes) CD5, CD20, CD23 Same as CLL; indolent, not affected by therapy Alkylating Agents, Benzene (CLL) ? Infections Del 13q Some have large cell transformation (Richter Syndrome) Viral Hypogammoglobulinemia RichterSyndrome Bad = trisomy 12, CD38 & de1 11q

    Gradual onset Adult Men Usually adult men (2% of leukemias) Trisomy 21, Bloom’s, Fanconi’s Hairy Cell Leukemia Bacterial & Mycobacterial Infections Not radiation Fair prognosis (40%) Alkylating Agents, Benzene (HCL) Beefy Red Splenomegaly, ? Reticulin in BM “Fried Egg appearance” Pancytopenia (70%), Neutropenia & Monocytopenia (90-100%) Viral TRAP+ B Cells TRAP+ Good Treatment - 2-CDA, pentostatin & interferon

    Many subtypes based on ringed sideroblasts & blast counts BM = Hypercellular, Periphery = Pancytopenia Idiopathic Ringed Sideroblasts May progress to AML Myelodysplastic Syndrome <20% blasts in BM, Megaloblastic appearance Therapy related (ex. Radiation) Chromosome 5,7,8 Very poor prognosis Anemia, Infection, Hemorrhage Chromosome 5,7,8 abnormalities

    ? erythropoietin Early organomegaly due to congestion ? RBC, Platelets & Granulocytes ? Hematocrit Late organomegaly due to extramedullary erythropoiesis Polycythemia Vera Growth Factor Mutations Fibrotic Marrow, Hypertension, Pruritis, Gout Hypertension & Hemorrhage Spent Phase = Fibrotic Marrow DVT, MI, Stroke ? Viscosity Fair to good prognosis (depends on treatment-phlebotomy)

    Early progression to myelofibrosis (spent phase) Dacrocytes (teardrop cells) & enlarged platelets in periphery PDGF & TGF-??Chronic Idiopathic Myelofibrosis Pancytopenia & Etramedullar hematopoiesis “Sensation of fullness” Dacrocytes (Agnogenic Myeloid Metaplasia) Excess collagen from fibroblasts Prognosis is difficult to predict Reticulin Markedly enlarged spleen, Gout Transformation ot AML (20%)

    Marked Thrombocytosis (>600,000) Least common of myeloproliferative disorders Large Platelets Essential Thrombocythemia Idiopathic Large Platelets Diagnosis by exclusion No myelofibrosis Thrombosis & Hemorrhage Good prognosis (>10 year survival)

    No Typical Reed Sternberg Cells Predominance of lymphocytes/hystiocytes Men more common Hodgkin’s Disease Lymphohystocytic Cells EBV Typically a cervical node Best Prognosis (Lymphocyte Predominance) B Cell Lymphoma Cd20 & CD45 Can turn into Diffuse Large B Cell Lymphoma CD20 & CD45

    Hodgkin’s Disease Predominance of lymphocytes/hystiocytes No Typical Reed Sternberg Cells Men more common EBV (Lymphocyte Rich) CD15 & CD30 Lymphohystocytic Cells Can turn into Diffuse Large B Cell Lymphoma

    Leukemia & Lymphoma (Contd.)

    Disease Cause/Risk Factors Symptoms Buzzwords Other

    Cervical, Supraclavicular or mediastinal nodes Lacunar Reed Sternberg Cells Hodgkin’s Disease Most common type; affects adolescent or young adult women EBV Variable cell composition & necrosis Collagen Bands (Nodular Sclerosis) Staging (usually I or II) determines treatment; Good prognosis CD15 & CD30 Women

    Variable cell composition & focal necrosis Hodgkin’s Disease Disordered fibrosis 2ndEBV Classic Reed Sternberg Cells most common type; men more common (Mixed Cellularity) Eosinophils, Plasma & T Cells Staging determines treatment; Guarded prognosis CD15 & CD30

    1) Diffuse Fibrosis RS Cells embedded in Fairly common usually in older patients Poor Prognosis Hodgkin’s Disease fibrous stroma Often Bone Marrow Involvement (Stage III or IV) EBV Many Reed Sternberg Cells (Lymphocyte Depletion) 2) Reticular RS Cells in sheets Poor prognosis Bone Marrow ? #s of Reed Sternberg Cells Usually not a true Hodgkin’s Lymphoma?

    Gradual onset, Mature Cells, Hepatosplenomegaly Not radiation Usually adults (most common adult leukemia in west) Trisomy 21, Bloom’s, Fanconi’s Clonal B Cell Disorder (small lymphocytes) CD5, CD20, CD23 Same as CLL; indolent, not affected by therapy Small Lymphocytic Lymphoma Alkylating Agents, Benzene ? Infections Del 13q Some have large cell transformation (Richter Syndrome) Viral Hypogammoglobulinemia RichterSyndrome Bad = trisomy 12, CD38 & de1 11q

    Nodular Growth Pattern with 2 cell types 1) Centrocytes = cleaved nuclear contours CD10,CD20,CD23 Fairly Common; indolent, not affected by therapy Follicular Lymphoma 2) Centroblasts = clear chromatin & multinucleate BCL-2, t14:18 May transform to diffuse large B cell lymphoma Bone Marrow frequently involved

    Atrophied germinal center CD5 & CD20 Mantle Cell Lymphoma Not indolent or treatable Small cells with cleaved nuclear contour BCL-1, t11:14

    Marginal Zone B-Cell Lymphoma H. Pylori Large B Cells, some plasma cells CD20 Common in GI Tract, Indolent, not dependent on treatment (MALT Type) Autoimmune (Sjogren, Hashimoto)

    Very Common, Very Aggressive EBV T14:18, t8:14, t13:14 Prognosis depends on treatment, BCL-6 = good prognosis Diffuse Large B-Cell Lymphoma Large cell size, diffuse growth pattern HSV8 BCL2,C-Myc,BCL6

    One of three bad types in kids “Starry Sky picture” Highest growth fraction of all tumors Burkitt’s Lymphoma EBV “Starry Sky picture” CD10,CD19,CD20 Most manifest as extra-nodal sites C-Myc Responsive to treatment, but poor prognosis Lymphoblastic Lymphoma One of three bad types in kids Mediastinal Mass like Hodgkins CD3, TdT+, TCL-1 (T Type) (T/B) Responsive to treatment, but poor prognosis

    One of three bad types in kids Anaplastic Large T/Null Cell CD3, CD30 Uncommon in adults, Common in children Lymphoma ALK, EMA t2:5 Presents in extra-nodal tissue Usually T, not B cell

    Extranodal NK/T Cell Lymphoma Peripheral T-Cell Lymphoma EBV CD3 Poor Prognosis; Common in Asia, uncommon in US Nasal Type eats face off

    Leukemia & Lymphoma (Contd. 2)

    Disease Cause/Risk Factors Symptoms Buzzwords Other

    Indolent Cutaneous disorder CD4 Mycosis fungoides Cerebriform nuclei Extracutaneous spread Polyclonal Ig (Leukemia = Sezary Syndrome)

    Painful Bone Destruction, ? Humoral Immunity M Protein Radiation, Chemicals, Asbestos Most common 1º malignant tumor of bone Plasmacytosis with IL-6, Vertebral Fractures Bence Jones Protein Multiple Myeloma Black Race Complications: Renal failure, Amyloidosis, AML (rarely) M Protein, Bence Jones proteins, Hypercalcemia Hypercalcemia HSV8? Fair prognosis (3 yr) Can spread to nodes, skin, etc IL-6

    Plasmocytosis with ? Light Chain Production Radiation, Chemicals, Asbestos Fibrils of ?-pleated sheets that stain Congo Red 1º Amyloidosis Black Race Large Tongue Usually immunoglobulin light chain Large tongue, Neuropathy, GI Multiple Myeloma Arrythmias, CHF

    Fibrils of ?-pleated sheets that stain Congo Red HSmegaly, 2º Amyloidosis Chronic Infection or Inflammation HSmegaly, Proteinuria Proteinuria

    Most common monoclonal gammopathy Monoclonal Gammopathy of Diagnosis of exclusion, IgG < 3.5 Aging M Proteins without other symptoms Undetermined Significance May develop into multiple Myeloma Requires no treatment

    Pituitary Pathology

    Disease Cause/Risk Factors Symptoms Buzzwords Other

    ? Prolactin Most common secretory adenoma of anterior lobe Prolactinoma Amenorrhea, Galactorrhea, Infertility, Effects less obvious in postmenopausal women Loss of Libido Treated with bromocriptine

    Injury to pituitary stalk Compression ? Prolactin Due to decreased dopamine to the anterior lobe Hyperprolactinemia Prolactin Trauma to Hypothalamus, Infection Non-prolactin secreting adenomas may compress stalk Drugs (Phenylthiazines)

    ? Growth Hormone ? ? Hepatic IGF-1 Second most common secretory adenoma of anterior lobe Some produce prolactin as well Somatotroph Adenoma IGF-1 Treatment = transsphenoidal resection, bromocriptine & radiation Acromegaly (adults) & Gigantism (children) Acromegaly = hands, feet, face skin & viscera DM, HT, CHF, arthritis, weakness, GI carcinoma

    ? ACTH? ? Cortisol (Cushing’s Disease) ACTH 90% are microadenomas Corticotroph Adenoma Moon facies, central obesity, striae, bruising Microadenoma Treatment = transsphenoidal resection Osteoporosis, DM & HT

    Gonadotroph Adenoma No clinical syndrome Secrete hormones inefficiently and variably

    Thyrotroph Adenoma Rarest

    Pregnancy & Lactotroph Hypertrophy Sheehan’s Syndrome Hypotension, DIC Hypopituitarism Most common cause of anterior lobe ischemic necrosis Sickle Cell Anemia, ? ICP

    Maldevelopment of diaphragma sella Depends on number of residual cells Pituitary Apoplexy Empty Sella Syndrome Hypopituitarism Maldevelopment = Arachnoid Herniation via enlarged opening Sheehan’s Syndrome No syndrome Ablation

    Interruption of DA to Posterior Lobe Trauma Diabetes Insipidus Large volume of dilute urine ? ADH Treatment = Administer ADH Tumor Inflammation

    Ectopic ADH (Small Cell Carcinoma) Syndrome of Inappropriate ADH Lung Disease (TB) Water Retention, Hyponatremia Treatment = Water Restriction, Slow normalization of Na? ADH (SIADH) Intracranial Lesions (? Inhibition) GI Upset, Cerebral Edema Drugs

    + (Rapid Normalization can cause Central Pontine Myelinolysis)

    Hyperpituitarism, Hypopituitarism Hypothalamic Tumors Glioma & Craniopharyngioma are most common Diabetes Insipidus

    CNS Pathology

    Disease Cause/Risk Factors Symptoms Buzzwords Other

    Reversible Concussion Blunt non-penetrating injury Paralysis Reversible Traumatic Paralysis Ocurring Immediately After Injury Immediate

    Seizures Most common sites: Frontal Pole, Temporal Pole, Medial orbital Stress on parenchymal vessels Cognitive & Personality Changes surface of the temporal lobe Contusion Coup Intact pia-glial membrane Headaches Pia-arachnoid not penetrated Countercoup Dizziness Occur at crests of gyri

    Subarachnoid bleeding Traumatic disruption of pia-arachnoid and brain surface Lacerations Penetrating Wounds Satellite petichial hemorrhages Meningocerebral cicatrix Meningocerebral cicatrix (glial scar) = epileptogenic focus Edema in tissue

    Fracture Immediate Loss of Consciousness Collection of blood between dura and skull Epidural Hemorrhage Most = Arterial (Middle Meningeal) Lucid Interval Lenticular shaped hematoma Rarely = Dural Sinus, Bridging Veins Later Loss of Consciousness

    Superficial Cortical Bridging Veins 48 hours-days; appears as clotted blood More Common than epidural hemorrhage Dural Sinus Laceration Gradual Loss of Consiousness Acute Subdural Hemorrhage Clotted Blood Typically seen in infants, elderly & alcoholics Depressed Fractures Hemiparesis ? Hemiplegia Sheet of blood between dura & arachnoid; forms hyaline sac Bullet Wounds Evidence of Herniation (if > 60 cc)

    Superficial Cortical Bridging Veins Clotted & Fluid Blood More Common than epidural hemorrhage Dural Sinus Laceration Gradual Loss of Consiousness Subacute Subdural Hemorrhage Clotted & Fluid Blood Typically seen in infants, elderly & alcoholics Depressed Fractures Hemiparesis ? Hemiplegia Sheet of blood between dura & arachnoid; forms hyaline sac Bullet Wounds Evidence of Herniation (if > 60 cc)

    Superficial Cortical Bridging Veins > 3 weeks & Liquefied Hematoma More Common than epidural hemorrhage Dural Sinus Laceration Gradual Loss of Consiousness Liquefied Hematoma Typically seen in infants, elderly & alcoholics Chronic Subdural Hemorrhage Depressed Fractures Hemiparesis ? Hemiplegia Minor Precipitating Event Sheet of blood between dura & arachnoid; forms hyaline sac Bullet Wounds Evidence of Herniation (if > 60 cc) Slow Bleeding, No immediate symptoms, Minor precipitating event

    Trauma Superficial Cortical Veins Stiff Neck, Alterations in Consciousness Subarachnoid Hemorrhage Stiff Neck Usually multiple and small Surface cortical lacerations Vasospasm & Hydrocephalus Surface cortical contusions

    Not in contact with the cortical surface, usually solitary Intracerebral Hematoma Rupture of Intrinsic Cerebral Vessels Frontal & Temporal Lobes are most common sites

    Damage usually occurs where nerves exit through foramina Cranial Nerve Damage Cranial Fractures Most Common = CNI, CNII, CN V & CN VI

    Pontomedullary Avulsion Marked Hyperextension of neck Poor prognosis, Immediately fatal if complete, die later if incomplete

    Unconscious from moment of impact Diffuse Axonal Injury No lucid interval Results in disconnection of distal axonal segment Diffuse Shearing of axons Calpain (1º Axotomy) Ca2+ influx & swelling Most common sites = Corpus Callosum & S. Cerebellar Peduncles Microtubule depolymerization, Calpain activation

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