Adrenal Guidelines- CUA Flashcards
What are classic symptoms of Pheo?
Headache, episodic prespiration, tachycardia, flushing, nasuea, HTN. Many tumors are asymptomatic.
what is a pheo called that happens outside of adrenal gland?
Paraganglioma
Can you differentiate malignancy by histology? if not then how?
not for pheo, the only way you can say it is malignant is by presence of mets.
what are risk factors for recurrence of pheo?
right sided, bilateral tumors, extra-adrenal disease, hereditary disease, large tumors
what are hereditary diseases associated with pheo?
NF-1, MEN2(RET mutation), VHL, Hereditary paragangliomas( SDHA,B,C, D, SDHAF2), familial pheo(TMEM127, MAX), polycythemia paraganglioma syndrome(EPAS1, HIF2A), hereditary leiomyomatosis(FH mutation)
what genetic mutation is associated with aggressive rapidly progressing pheo/paraganglioma?
SDHB
IMPORTANT, REMEMBER this lol
What is CUA BPR 2019 recommendations on genetic screening?
We recommend all patients with
PPGLs(pheochromocytoma/paragangliomas) be considered for referral for genetic testing
what are the best two initial tests to diagnose pheo?
Plasma free metanephrines and urine fractionated metanephrines
what are CUA BPR recommendations regarding follow up testing for PPGLs?
We suggest repeating plasma and/or
24-hour urinary metanephrines at first postoperative followup
to ensure complete resection (
We suggest monitoring for PPGL
recurrence by annually measuring plasma-free metanephrines
and/or 24-hour urinary fractionated metanephrines
what are recommendations regarding duration of FU for Pheo?
We suggest annual followup for at
least 10 years following complete resection to monitor for
local or metastatic recurrences or new tumors
We suggest high-risk patients (young,
genetic disease, larger tumor and/or a paraganglioma) be
offered lifelong annual follow-up
what is recommended for patients with pheo with borderline results? (<4fold increase)
We suggest using annual clonidine suppression
or chromogranin A testing for followup of patients
with positive preoperative results
what is recommended for patients with bichemically inactive pheo tumors for FU?
We suggest imaging tests be obtained every 1–2 years, in addition to yearly metanephrines for
patients with biochemically negative disease( sometimes they can become active)
what is recommended as imaging modality of choice in case of biochemical recurrence?
For biochemical recurrence, we suggest CT/MRI as first-line imaging modalities, and
123I-metaiodobenzylguanidine (MIBG) scintigraphy as secondline. (F-FDG PET is better but it is not widely available in Canada)
What is recommended regarding malignant pheo for FU and management?
Malignant pheochromocytoma treatment
should be discussed in a multidisciplinary setting that
includes surgeons, interventional radiologists, endocrinologists,
oncologists, and nuclear medicine physicians
what kind of laboratory work up does CUA 2011 recommend for Adrenal incidentalomas?
- 24 hr Urinary Free Cortisol or 1mg DST
- 24 hr Urinary metanephrines and catecholamines
- If hypertensive, plasma aldosterone and plasma renin
