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