• Shuffle
    Toggle On
    Toggle Off
  • Alphabetize
    Toggle On
    Toggle Off
  • Front First
    Toggle On
    Toggle Off
  • Both Sides
    Toggle On
    Toggle Off
  • Read
    Toggle On
    Toggle Off
Reading...
Front

Card Range To Study

through

image

Play button

image

Play button

image

Progress

1/28

Click to flip

Use LEFT and RIGHT arrow keys to navigate between flashcards;

Use UP and DOWN arrow keys to flip the card;

H to show hint;

A reads text to speech;

28 Cards in this Set

  • Front
  • Back

MEN1 inheritance?

AD ; germline mutation at chromosome 11q13

familial MEN1 defined as....

one MEN1 case plus one first-degree relative who has at least one of the 3 tumors

usual disorders associated with MEN1

primary hyperparathyroidism (parathyroid adenomas)




pituitary adenomas -> prolactinoma




enteropancreatic -> gastrinoma (zollinger-Ellison syndrome)

how are MEN1 tumors different from comparable tumors without the mutation

earlier age, greater tendency to recur

what is commonly associated with MEN1 mutation?

cutaneous features such as angiofibromas, collagenomas, and lipomas

most common manifestation of MEN1 mutation?

hyperparathyroidism

characteristic skin rash

glucagonoma

associated with watery diarrhea and weakness

VIPoma

combination of multiple angiofibromas (more than three) and any collagenomas indicates MEN1 in...

75% of cases

familial MEN1

at least one typicalMEN1 tumor in a first-degree relative of an index case of familial MEN1.

non-familial MEN1

combinations of tumors similar to those seen in familial MEN1 but with no involvement of first degree relatives, no MEN1 mutations, and lack of typical MEN1 features such as early onset of disease or multiple tumors

familial MEN1 w/ only one MEN1 condition

new index cases maypresent with only one MEN1-related tumor, usually primary hyperparathyroidismbut occasionally other tumors that tend to produce more symptoms, e.g.gastrinoma. The presence of early age onset, multiple tumors, or specifictumors is an indication to consider the diagnosis of MEN1. Children inparticular may present with tumors in only one major gland

more than 95% of MEN1 patients develop_______ by age 45

primary hyperparathyroidism

most common pituitary adenoma in MEN1 patients

prolactinoma; may be less responsive to therapy and more likely to recur

which MEN1 tumors are frequently malignant and represent major source of mortality due to MEN1

gastrinomas and carcinoids

what treatment is rarely curative for gastrinomas?

surgery; in non MEN1 tumors the success rate is higher

carcinoids in MEN1 patients are usually located where?

malignant thymic (70%) and bronchial (20%)


gastric carcinoids have low malignancy

What should you not miss in these patients?

neuroendocrine tumor

diagnostic procedure for MEN1

initial goal is to confirm the presence or absence of the three main MEN1 related endocrine tumors; the order of the test should be determined by the current symptoms; the presence of first degree relatives with similar findings and/or the presence of typical MEN1 gene abnormalities confirms the diagnosis of familial MEN1

imaging test preference for pituitary adenoma

MRI

primary test for diagnosis of gastrinoma

fastin gastrin measurement and the measure of gastric acid secretion w/ secretin stimulation

gastrinomas are likely to metastasize to...

the liver; use MRI to determine if there has been metastasis

enteropancreatic tumor imaging

CT

newly diagnosed MEN1 patients procedure

endoscopy -> look for carcinoid tumors



after 5 -> look for insulinomas, anterior pituitary tumor, and PTH adenoma




at age 20 -> look for gastrinoma, thymus or bronchial carcinoid, and enteropancreatic tumors

treatment of choice in Cushions pituitary ACTH secreting tumor

trans-sphenoidal surgery

treatment for gastrinoma

proton pump inhibitors; octreotide has been show to effectively reduce hormone secretion and shrink both benign and malignant tumors

treatment for insulinoma

surgery

treatment for carcinoid tumors

prophylactic thymectomy recommended due to the potential risk of malignant thymic carcinoid; management of malignant gastric carcinoids w/ surgery or by treatment with octreotide