Adrenal Medulla and Paraganglia Flashcards
What are the primary cells of the adrenal medulla?
The chromaffin cells
What hormones are secreted by the medulla?
Epinephrine and Norepinephrine
What is pheochromocytoma?
Catecholamine-secreting tumors of chromaffin cells of the adrenal medulla
What is the key symptom of a person with pheochromocytoma?
Hypertension
Photomicrograof the tumor shows polyhedral tumor cells with ample finely granular cytoplasm. Dx?
Pheochromocytoma
Pheochromocytoma is shown. What IHC markers are used to identify this tumor?
Chromogranin and synaptophysin
What can episodic catechol release associated with pheochromocytoma precipitate?
Paroxysms/Crises of headache, sweating, palpitations, tachycardia, abdominal pain, and vomiting
What is catecholamie cardiomyopathy?
Myocardial necrosis caused by elevated catecholamine levels
What is Dx of pheochromocytoma
Elevated urinary levels of catechol metabolites (vanillylmandelic acid, metanephrine, unconjugated catecholamines)
What are paragangliomas?
Pheochromocytomas arising at extra-adrenal sites
What are carotid body tumors?
Paragangliomas arising at the carotid bifurcation
What gene is associated with Paragangliomas?
SDHD gene
What is Multiple Endocrine Addenomatosis?
Inherited disorders in which multiple endocrine organs are affected by diverese cellular proliferations
What is another name for MEN type 1? What complications are typically seen?
Wermer Syndrome
- Pituitary Adenoma
- Parathhyroid Hyperplasia/Adenoma
- Islet cell tumor of the pancreas
What mutation causes Wermer Syndrome?
Mutation in the MEN1 tumor suppressor gene that encodes protein termed menin
What condition is observed in almost all patients with MEN1? MEN2?
Primary Hyperparathyroidism; Medullary Thryroid Carcinoma
What gene is responsible for MET2 syndromes?
RET protooncogene
What mutation is responsible for most patients with MEN 2B?
Point mutation at codon 918 of the tyrosine kinase domain of RET
What mutation has been identified in most patients with MEN2A?
Mutations in the cysteine-rich extracellular domain of RET
What is used to Dx MEN2?
ID RET mutations
What is a neuroblastoma?
An embryonal malignancy of neural crest origin composed of neoplastic neuroblasts
What is the most common tumor of childhood?
Neuroblastoma
What are the four categories of neuroblastomas?
- Neuroblastoma
- Ganglioneuroblastoma, intermixed
- Ganglioneuroma
- Ganglioneuroblastoma, nodular
Neuroblastoma is shown. What morphologies are characteristic of this tumor?
Homer Wright Rosettes
What metabolites are found in the urine of a person with Neuroblastoma?
Norepinephrine, VMA, homovanillic acid, and dopamine
What is a ganglioneuroma?
Mature variant of neuroblastic tumors
Histology shows well-differentiated, amture ganglion cells, associated with spindle cells in a loose, abundant fibrillar stroma. Dx?
Ganglioneuroma
