2. Syndromes Flashcards
- Parathyroid Hyperplasia (90%),
- Pituitary Adenoma,
- Pancreatic Tumor (Gastrinoma most commonly)
Multiple Endocrine Neoplasia (MEN) 1
1 Letter of the alphabet which all three have in common is “P” so there are 3 P’s: Pi Para Pane (pituitary, parathyroid, and pancreas)
- Medullary Thyroid Cancer (100%)
- Parathyroid hyperplasia,
- Pheochromocytoma (33%)
Multiple Endocrine Neoplasia (MEN) IIa
MEN 2 A
The A’s :
Adrenal (pheochromocytoma).
Aggressive thyroid CA (medullary)
Alot of Calcium (parathyroid)
- Medullary Thyroid Cancer (80%)
- Pheochromocytoma (50%)
- Marfanoid Body HabitusMucosal Neuroma,
Multiple Endocrine Neoplasia (MEN) IIb
it is a wannaBe (it wants to be like A).
So, it is the same (adrenal pheo, medullary CA)
BUT it has a beef with calcium, so instead of parathyroid, it decided to B a Gangster (gangster for Ganglioneuroma).
Flushing, diarrhea, pain, right heart failure from serotonin by the tumor
Carcinoid Syndrome
Carcinoid Syndrome does not occure until the tumor mets to what organ?
Liver (normally the liver metabolizes the serotonin)
The typical primary location for the carcinoid tumor
GI Tract (70%)
The most common primary location of carcinoid tumor
is the distal ileum
Carcinoid -
Classic Mesenteric Involvement
GI carcinoids are associated with other tumors:
GI tumors (adenoCA)
Urine Test for Carcinoid
5-HIAA (5-hydroxyindoleacetic acid)
In carcinoids, Systemic serotonin degrades what structure of the heart?
valves (right sided), and classically causes tricuspid regurgitation
Most common tumor in Von-Hippen-Lindau
Hemangioblastoma (seen in the retina, cerebellum, spinal cord)
Multiple head and neck paragangliomas (risk ~ 70%)
PGL Syndrome (Paraganglioma & Pheochromocytoma)
Clinical Triad of Tuberus Sclerosis
Facial Angiofibroma
Seizures
Retard Brain
Where is Subependymal Giant Cell Astrocytoma (SEGA) found?
caudothalamic groove adjacent to the foramen of monro
the most common pituitary tumor
Prolactinoma (MEN1)
VHL assoc.
Bilateral clear cell RCC
Papillary Cystadenoma of the epididymis
Endolymphatic Sac Tumor
Pheochromocytoma
Pancreatic Cysts and serous cystadeomas
Tuberous Sclerosis (Bourneville Disease) assoc
Cortical Tubers
Subependymal Nodules
SEGA
Renal AMLs
Cardiac Rhabdomyoma
Pulmonary Cysts
LAM
MEN 1 assoc.
Primary Hyperparathyroidism
Prolactinoma
Gastrinoma
MEN 2A Assoc
Pheochromocytoma
Primary Hyperparathyroidism
Medullary Thyroid Cancer
MEN2B
Marfanoid Appearance
Mucosal Neuromas
Intestinal Ganglioneuromas
These patients fart alot (seriously)
