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