Editing Adrenal: Pheochromocytoma (section)

== Hereditary forms of pheochromocytoma ==

* In cases of VHL the risk of malignancy is low but pheochromocytoma is characterized as producing norepinephrine.
* Unlike pheochromocytomas in patients with VHL, MEN2 and NF1 predominantly produce epinephrine.
* Compared to sporadic cases, '''in familial syndromes, the pheochromocytomas are almost always bilateral and more frequently malignant.''' Clinical manifestations are similar.

{| class="wikitable"
|'''Syndrome'''
|'''Clinical characteristics'''
|'''Risk of pheochromocytoma'''
|'''Risk of malignant disease'''
|-
|'''Multiple endocrine neoplasia type 2A'''

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|
# '''Medullary cancer of thyroid'''
# '''Hyperparathyroidism'''
# '''Cutaneous lichen'''
# '''Amyloidosis'''
|'''50%'''
|'''3%'''
|-
|'''Multiple endocrine neoplasia type 2B'''
|
# '''Medullary cancer of thyroid'''
# '''Hyperparathyroidism''' (rare)
# '''Multiple neuromas'''
# '''Marfanoid body habitus'''
|'''50%'''
|'''3%'''
|-
|'''<span style="color:#ff0000">Von Hippel-Lindau (VHL), type 2</span>'''
|'''<span style="color:#0000ff">HIPPPEEL</span>'''
#'''<span style="color:#ff0000">CNS and/or retinal </span><span style="color:#0000ff">H</span><span style="color:#ff0000">emangioblastomas</span>'''
# '''<span style="color:#ff0000">ccRCC (</span><span style="color:#0000ff">I</span><span style="color:#ff0000">ncreased risk) and renal cysts</span>'''
#'''<span style="color:#0000ff">P</span><span style="color:#ff0000">heochromocytoma</span>'''
#'''<span style="color:#0000ff">P</span><span style="color:#ff0000">araganglioma</span>'''
#'''<span style="color:#0000ff">P</span><span style="color:#ff0000">ancreatic neuroendocrine tumours and cysts</span>'''
#'''<span style="color:#0000ff">E</span><span style="color:#ff0000">pididymal cystadenoma</span>'''
#'''<span style="color:#ff0000">Ear </span><span style="color:#0000ff">E</span><span style="color:#ff0000">ndolymphatic sac tumour</span>'''
#'''<span style="color:#ff0000">Broad </span><span style="color:#0000ff">L</span><span style="color:#ff0000">igament tumours</span>'''
|10-20%
|5%
|-
|'''Neurofibromatosis type 1'''
|
# '''Neurofibromas'''
# '''Café-au-lait skin spots'''
|1%
|11%
|-
|'''Familial paraganglioma syndrome type 4'''
|Carotid body tumors (chemodectomas)

Vagal, jugular, tympanic, abdominal, thoracic paragangliomas
|20%
|30-50%
|-
|'''Familial paraganglioma syndrome type 1'''
|Carotid body tumors (chemodectomas)

Vagal, jugular, tympanic, abdominal, thoracic paragangliomas
|20%
|<3%
|}