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Endocrine Post Clinic ConferenceSeptember 2, 2011
24 yo man with hypertension
Case presentation CT results Imaging algorithms Role of MIBG Other diagnostic tools Genetics Case discussion
Outline: Pheochromocytoma
Case : H&P
Asymptomatic SBP 230s in 2009 on routine post-deployment exam
Reports elevated catecholamines but no findings on CT / MR / MIBG
Tried on beta-blockers which made him feel lousy. + orthostasis
Now with sweats, palpitations, panic attacks, occasional left sided chest pain. +history of migraines, unchanged
Currently without treatment, has tried to avoid physical stresses
Case 1: H&P
Past Medical History:• Hypertension first noted 2009• Migraines since age 12• PTSD• no prior surgeries
Case 1: H&P
Medications: xanax prn anxiety
Social: smokes <1 ppd occ EtOH no cocaine/meth or other illicits
Family: Immediate family healthy without
hypertension
Case 1: H&P
Physical Exam:T 99.3, HR 86, 180/132, 100% on room air
Well-developed, well-nourished male, NAD Eyes: PERRL, EOMI, no lid lag, no stare, vision full to confrontation Neck: no thyromegaly, no masses, no nodules RRR without m/r/g CTAB
Abd benign Skin warm and mildly diaphoretic
Livedo reticularis?
Livedo reticularis?
Case: Laboratory Data
139
3.7
104
19
28 1.0
127
16
489 220
Calcium 9.4Calcitonin < 0.2
LFTs normalAlbumin: 4.4
Thyroid function tests normal
Urinenormeta 9125
(<600)metaneph 9256
(<900)Plasma
normeta 4258 (<149)
metaneph 4302 (<206)
catechol 12182 (<504)
(plt giant and clumped)
CT“Heterogeneously enhancing, partially necrotic right adrenal mass measuring 3.8 cm . . .”
Next step?
Ilias I , Pacak K JCEM 2004;89:479-491
MIBG
Controversy: All cases or only when unable to find a typical tumor or when high suspicion for malignancy?
Malignancy associated with extra-adrenal location, larger tumor size and +SDHB mutation
Metastatic tumors larger, present at younger age and more likely necrotic on path
Metastatic pheochromocytoma: Does the size and age matter?. European Journal of Clinical Investigation. doi: 10.1111/j.1365-2362.2011.02518.x
MIBG
Recent meta-analysis placed sens/spec at 94% and 92% for pheo
In a study of 32 patients, able to reach 100% sensitivity and ppv with MRI+MIBG. Only false negatives (three) with MIBG alone were either small or necrotic intraadrenal masses
The Journal of Clinical Endocrinology & Metabolism June 1, 2010 vol. 95 no. 6 2596-2606Nuclear Medicine Communications July 2006 vol. 27 no. 7 583-587
Patient’s MIBG
Right Left
Inverted MIBG image
Other diagnostic tools
PET > MIBG?A 2009 prospective observational study of 52 patients found greater sensitivity for metastatic disease with PET/CT than MIBG
The Journal of Clinical Endocrinology & Metabolism December 1, 2009 vol. 94 no. 12 4757-4767
Other diagnostic tools
Adrenal vein samplingHigh variability even in normal patients increases risk of false positives
Adrenal venous sampling for catecholamines: a normal value study. Clin Endocrinol Metab. 2010;95(3):1328
Syndromes
MEN 2 vonHippel Landau Neurofibromatosis Familial paragangliomas
When to test genetics?
1/3 pheos carry germline mutation In one study, of 989 apparently non-
syndromic patients, 342 with mutation. Only 8 of these missed if ignored
age>45, only single adrenal pheos and those without prior head/neck paraganglionomas
1Clin Cancer Res October 15, 2009 15; 6378
Plan for this patient
Continue alpha blockade in prep for OR
MRI to better visualize left adrenal If no clear mass in left adrenal, adrenal
venous sampling? Just leave left side alone? Take out whole left adrenal?
Consider genetic testing