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Medicine (MD/DO)
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Neuroendocrine Disorders

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Pathology | Medicine (MD/DO) School Study Aid

Neuroendocrine Disorders
20 Picmonics to Learn | 50 mins
Pheochromocytoma
Cause/Mechanism
Adrenal Medulla Tumor
Increased Catecholamines
Assessment
Episodic Hypertension
Diaphoresis
Abdominal or Chest Pain
Interventions
Surgery
Antihypertensives
Phenoxybenzamine
Metyrosine (Demser)
Considerations
Do Not Palpate Abdomen
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3 mins
MEN 1 (Multiple Endocrine Neoplasia)
Pathophysiology
Autosomal Dominant
Menin Mutation (Tumor Suppression Gene)
Chromosome 11
Signs and Symptoms
Pancreatic Endocrine Tumors
Pituitary Tumors
Parathyroid Adenomas
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2 mins
MEN 2A (Multiple Endocrine Neoplasia)
Pathophysiology
Autosomal Dominant
RET Gene
Signs and Symptoms
Medullary Thyroid carcinoma
Calcitonin
Parathyroid
Pheochromocytoma
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45 secs
MEN 2B (Multiple Endocrine Neoplasia)
Pathophysiology
RET Gene
Autosomal Dominant
Signs and Symptoms
Medullary Thyroid Carcinoma
Pheochromocytoma
Mucosal Neuromas
Marfanoid Body Habitus
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2 mins
Zollinger-Ellison Syndrome Disease
Pathophysiology
Gastrin-Secreting Tumor
Increased Gastric Acid
Recurrent Peptic Ulcers
MEN Type 1
Symptoms
Abdominal Pain
Heartburn (Pyrosis)
Diarrhea
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3 mins
Zollinger-Ellison Syndrome Diagnosis and Treatment
Diagnosis
Gastrin > 1000 pg/mL
Positive Secretin Stimulation Test
Treatment
High-Dose PPI
Octreotide
Surgical Resection
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3 mins
Carcinoid Syndrome
Pathophysiology
Neuroendocrine Cells
Serotonin
Appendix
Diagnosis
Chromogranin A
Neuron Specific Enolase
Dense Core Bodies on EM
Signs and Symptoms
Symptoms When Metastasize to Liver
Flushing
Wheezing
Right Heart Murmur
Diarrhea
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4 mins
Bronchial Carcinoid Tumor
Characteristics
Excellent Prognosis
Signs and Symptoms
Symptoms of Bronchial Obstruction
Often Asymptomatic
Carcinoid Syndrome
Diarrhea
Wheezing
Flushing
Diagnosis
5-HIAA in Urine
Nests of Neuroendocrine Cells
Chromogranin A Positive
Treatment
Surgical Resection
Octreotide for Symptomatic Relief
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2 mins
Medullary Thyroid Carcinoma
Sporadic and Familial Types
Arises from Parafollicular C cells
Produces Calcitonin
Hypocalcemia
May Produce ACTH
Diagnosis
Sheets of Cells With Amyloid Deposition
Increased Calcitonin with Pentagastrin Infusion
Risk Factors
MEN 2A and 2B
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2 mins
Small Cell (Oat Cell) Carcinoma
Characteristics
Aggressively Malignant
Strong Correlation with Smoking
Central Location
Amplification of Myc Oncogenes
Signs and Symptoms
Narrowing of Bronchial Airways
Cushing's Syndrome
Ectopic ACTH
Syndrome Of Inappropriate Antidiuretic Hormone (SIADH)
Lambert-Eaton Syndrome
Diagnosis
Kulchitsky Cells
Neuron-Specific Enolase Positive
Chromogranin A Positive
Treatment
Chemotherapy and Radiation
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3 mins
Lambert-Eaton Syndrome
Mechanism
Autoantibody to the Presynaptic Calcium Channel on Motor Neurons
Inhibits Acetylcholine (ACh) Release at Neuromuscular Junction
Paraneoplastic Syndrome
Small Cell Lung Cancer
Symptoms
Proximal Muscle Weakness
Improves with Muscle Use
Autonomic Symptoms
Extraocular Muscle Sparing
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2 mins
Schwannoma
Characteristics
Acoustic Schwannoma
Cranial Nerve VIII
Cerebellopontine Angle
S100 Positive
Bilateral Schwannoma in Neurofibromatosis Type 2
Tinnitus
Cranial Nerve V
Affects Corneal Reflex
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2 mins
Von Hippel-Lindau Disease
Pathophysiology
Chromosome 3
Presentation
Hemangioblastomas
Medulla, Retina, Cerebellum
Cysts
Kidney, Liver, Pancreas
Develop Bilateral Renal Carcinomas
Pheochromocytomas
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2 mins
Neurofibromatosis Type 1
Pathophysiology
Autosomal Dominant
Chromosome 17
Signs and Symptoms
Neurofibromas
Optic Glioma
Lisch Nodules
Hamartomas
Cafe Au Lait Spots
Scoliosis
Pheochromocytoma
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4 mins
Neuroblastoma Diagnosis and Management
Diagnosis
24-hour Urine Test
Increased Catecholamines
Homovanillic and Vanillylmandelic Acid
Ultrasound
Magnetic Resonance Imaging (MRI)
Biopsy
Pathology
Small Round Blue Cells
Homer-Wright Rosettes
Bombesin Positive
Neuron Specific Enolase Positive
Interventions
Local Excision
Chemotherapy
Radiation
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4 mins
Neuroblastoma Characteristics and Presentation
Characteristics
Malignant Neuroendocrine Tumor
Neural Crest Cells Origin
Sympathetic Ganglia
Adrenal Glands
Amplification of N-myc Oncogene
Most Common Cancer In Infancy
Clinical Presentation
Abdominal Mass That Can Cross Midline
Gastrointestinal Distress
Metastasis
Raccoon Eyes
Opsoclonus-Myoclonus Syndrome
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4 mins
VIPoma
Characteristics
Pancreatic Neuroendocrine Tumor
Secretes Vasoactive Intestinal Polypeptide (VIP)
Clinical Features
GI Distress
Watery Diarrhea
Hypokalemia
Achlorhydria
Management
Octreotide
Surgical Resection
Associations
Multiple Endocrine Neoplasia Type 1 (MEN 1)
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2 mins
Somatostatinoma
Characteristics
Pancreatic Neuroendocrine Tumor
Originates from Delta Cells
Clinical Features
Steatorrhea
Gallstones
Hyperglycemia
Diagnosis
Increased Somatostatin
Abdominal CT or MRI
Management
Octreotide
Surgical Resection
Chemotherapy
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2 mins
Glucagonoma
Characteristics
Pancreatic Neuroendocine Tumor
Originates from A Cells
Clinical Features
Weight Loss
Necrolytic Migratory Erythema
Depression
Diarrhea
Deep Vein Thrombosis
Diabetes Mellitus
Diagnosis
Increased Glucagon
Abdominal CT
Management
Octreotide
Surgical Resection
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2 mins
Insulinoma
Characteristics
Pancreatic Neuroendocrine Tumor
Benign
Associated with MEN-1
Clinical Features
Whipple Triad
Symptoms of Hypoglycemia
Hypoglycemia During Episode
Relief with Glucose
Diagnosis
Increased C-peptide
Management
Surgery
Octreotide
Diazoxide
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2 mins

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