Paraneoplastic Syndrome, GN Neoplasms, and MEN Flashcards
recognize these at part of a differential
65% of gastroenteropancreatic neuroendocrine neoplasms are _____ or _____ at time of diagnosis
unresectable
metastatic
which genetic disorder are gastroenteropancreatic neuroendocrine neoplasms associated with?
MEN 1
what do B cells secrete?
insulin
what do D cells secrete? what effect does it have?
somatostatin
decreases rate of gastric emptying, inhibits release of glucagon and GH
what do G cells secrete?
gastrin
what are B cell adenomas also called?
insulinomas
continued insulin secretion, despite hypoglycemic state, and is benign in 90% of cases
B cell adenoma
what are the lab results in B cell adenomas?
glucose, insulin, C peptide
plasma glucose <45
serum insulin > 6
elevated C peptide
what should we rule out in a case of B cell adenoma?
medication cause with sulfonylureas
what diagnostic can we get done for a B cell adenoma?
FNA
what is the treatment of choice for a B cell adenoma?
surgical resection
excessive gastric causing the stomach to secrete excessive acid
G cell gastrinoma
severe acid-related peptic disease and diarrhea associated with G cell gastrinomas
Zollinger-Ellison syndrome
what is rare, but one of the more common functional pancreatic neuroendocrine tumors?
G cell gastrinoma
what percentage of G cell gastrinomas are malignant?
50%
a G cell gastrinoma can be in the _____ or _____
pancreas
duodenum
in which patients should we consider a G cell gastrinoma?
patients with history of peptic ulcers
which condition are G cell gastrinomas associated with?
MEN 1
secrete glucagon, usually malignant with metastases, and cause weight loss, diarrhea, nausea, and DM
A cell glucagonomas
do not produce hormones, but grow large prior to detection; causing symptoms due to mass effect
nonfunctional tumors
what is the treatment for a G cell gastrinoma?
surgical resection
why is genetic testing in MEN recommended?
it is inherited as autosomal dominant
causes predisposition to the development of tumors in at least 2 endocrine glands
multiple endocrine neoplasia (MEN)
what is the life expectancy of MEN 1?
less than 55 yo
what tumors are common in MEN 1? (3)
parathyroid
pancreatic
pituitary
what tumor is common in MEN 2A?
medullary thyroid carcinoma
what tumors are common in MEN 2B? (2)
medullary thyroid carcinoma
pheochromocytoma
what is MEN 1 also known as?
Wermer Syndrome
a patient presents with hypercalcemia and hyperparathyroidism. what could they be experiencing?
MEN 1
Wermer Syndrome
patients with MEN 1 (Werner Syndrome) can present with what 2 other tumors?
enteropancreatic tumor
gastrinoma
if a patient has MEN 1 (Werner Syndrome) + pituitary adenoma, they could have an elevated _______
serum prolactin
what 3 types of non-endocrine tumors can be present in a patient with MEN 1 (Werner Syndrome)?
facial angiofibroma
lipoma
collagenoma
what is MEN S2 also known as?
Sipple Syndrome
what becomes a significant possibility and concern in patients with MEN 2A?
medullary thyroid cancer
what is our recommended prophylactic treatment for MEN 2A?
total thyroidectomy by 6 yo in children with gene mutation
what 4 conditions are common in MEN 2B?
pheochromocytoma
medullary thyroid cancer
mucosal neuroma
marfan like habitus
what is our recommended prophylactic treatment for MEN 2B?
total thyroidectomy by 6 months in babies with gene mutation
symptoms and physical findings due to the effects of a neoplasm that is mediated by hormones and cytokines either secreted by the tumor or an immune response from the body to the tumor
paraneoplastic syndrome
what is the common initial presentation of paraneoplastic syndrome?
fever
in which cancers is paraneoplastic syndrome most common in? (4)
lung
breast
ovarian
lymphatic (hodgkins/non-hodgkin)
what is the most common cause of SIADH in paraneoplastic syndromes? why?
small cell lung cancer
secretes ADH
what does SIADH in paraneoplastic syndromes lead to?
hyponatremia
what is the most common cause of hypercalcemia in paraneoplastic syndromes? why?
bony metastasis from lung cancer
increased osteoclast bone resorption put into blood
what is a DDx for hypercalcemia in paraneoplastic syndromes?
hyperparathyroidism
what are 2 causes of hypoglycemia in paraneoplastic syndromes?
insulinoma
hepatocellular carcinoma
why does hepatocellular carcinoma cause hypoglycemia in paraneoplastic syndromes?
damaged liver cannot meet body’s glucose demand
what are 2 causes of cushing syndrome in paraneoplastic syndromes?
small cell lung cancer
pancreatic cancer
SCLC with associated cushing syndrome has a _____ prognosis than patients without cushing syndrome
worse
