An Abnormal Case of an Abnormal Headache
Peter Stempniewicz, MD and Alex Tatusov, MD
Hahnemann University Hospital-Drexel University College of Medicine Philadelphia, PA
Objectives
• To help diagnose and treat paragangliomas
• To differentiate paragangliomas from pheochromocytomas
Background
• Paragangliomas (PG) are rare neuroendocrine tumors that arise from
the extra-adrenal autonomic paraganglia.
• PGs can be parasympathetic and thus non-secretory, or they can be
sympathetic and have the ability to secrete catecholamines.
• Parasympathetic PGs are usually found in the neck, while the
sympathetic PG are usually anywhere along the sympathetic trunk
below the neck, most commonly in the abdomen.
Case Presentation
CC: Headache, palpitations, feeling of impending doom
HPI: The patient was a 76 year old female who originally presented to
the ER with complaints of palpitations, headache, high blood pressure
and a sensation of impending doom. She said that she was in her usual
state of health until the week prior where she had an episode of chest
wall pounding that was associated with a sensation of “impending
doom.” She was able to measure her blood pressure at that time, and
it was measured to be around 180/100. She never had any history of
hypertension before, so she went to an urgent care clinic, where she
was prescribed a low dose of amlodipine. Since then, she still had
several similar episodes in the past week, and then was told to go to
the emergency room by her cardiologist, who she had called. These
symptoms had no triggers or particular time of onset. She denied any
associated diaphoresis, nausea, vomiting, cough, shortness of breath,
fevers, chills, lightheadedness, or seizures.
PMH: Hypothyroidism, hyperlipidemia, and a benign non-secretory
paraganglioma that was resected from her chest 5 years prior.
PE: Unremarkable
Initial Labs: CBC, CMP, TSH were unremarkable.
However, given her symptoms there was concern for a
pheochromocytoma, so plasma fractionated catecholamines were
checked.
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Case Presentation Continued
She was feeling better the next day, so she was discharged home, but
then later came back 5 days later with similar complaints, with the
addition of left facial and left leg parasthesias. The results of her
previously ordered plasma fractionated catecholamines had come back
by this time, and they were found to be elevated. The lab results
revealed an elevated norepinephrine of 845 pg/ml (NL<420) and an
elevated total catecholamines of 863 pg/ml (NL<504). Chromogranin A
was also elevated at 6 nmol/L (NL<5). She was started on an alpha
receptor antagonist, and had a CT scan of the abdomen and chest to
look for a pheochromocytoma or recurrent paraganglioma in her chest.
CT scan of the abdomen was negative for pheochromocytoma and the
CT scan of the chest was negative for recurrent paraganglioma. This
lead to some head scratching, so a 24 hour urine metanephrine was
also checked, but came back within normal limits, most likely since it
was measured after the episodic event. A CT scan of the neck was then
decided upon, which showed an enhancing mass splaying the left
carotid bulb measuring approximately 17x12x14 mm. The mass had
asymmetric enhancement on the right side and somewhat
heterogeneous internal enhancement characteristic of a
paraganglioma. Afterwards, she decided that she wanted to have the
surgery at another hospital that was closer to where she lived, and was
shortly discharged.
Figure 1. CT of the neck showing an enhancing mass splaying the left carotid
bulb.
Figure 2. There are several staging systems used to classify carotid body
tumors, with the Shamblin criteria being the most used:
• Type I tumors are localized with splaying of the carotid bifurcation but little
attachment to the carotid vessels
• Type II tumors partially surround the carotid vessels
• Type III tumors intimately surround the carotids
• Type IIIB tumors are of any size that are intimately adherent to the carotid
vessels
Discussion
Paraganliomas are neuroendocrine tumors that arise from embryonic
neural crest cells and are found at autonomic paraganglia. They are
cellularly identically to the more known pheochromocytomas, with the
main difference being location. The two terms have changed in
definition over time. In the 2004 WHO classification, the term
pheochromocytoma is reserved for adrenal catecholamine secreting
tumors, while paraganglioma is the term used to describe extraadrenal tumors of sympathetic or parasympathetic paraganglia origin
(regardless of secretory status). Catecholamine-secreting
paragangliomas often present clinically with hypertension, episodic
headache, sweating, and tachycardia. Secretory paragangliomas are
found in the sympathetic paraverterbral ganglia of the thorax,
abdomen, and pelvis, with around 75% being located in the abdomen.
Only about 5% of paragangliomas arising within the skull base and neck
region are secretory. Resection is the definitive treatment for
secretory paragangliomas. Alpha blockade is usually given 10-14 days
prior to resection.
References
1. The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and
management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary
thyroid cancer. Chen H, Sippel RS, O'Dorisio MS, Vinik AI, Lloyd RV, Pacak K, North American
Neuroendocrine Tumor Society (NANETS) Pancreas. 2010 Aug;39(6):775-83.
2. S. Arya, V. Rao, S. Juvekar, and A.K. Dcruz.Carotid Body Tumors: Objective Criteria to Predict
the 4. Shamblin Group on MR ImagingAJNR Am J Neuroradiol August 2008 29: 13493. http://www.uptodate.com/contents/ paragangliomas-epidemiology-clinical-presentationdiagnosis-and-histology
4. http://www.uptodate.com/contents/ paragangliomas-treatment-of-locoregional-disease