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Case Report

Primary supratentorial intracerebral malignant

paraganglioma

Ahmed A. Al Jishi, Boleslaw Lach, Ali Elgheriani, Edward Kachur, Aleksa Cenic
Department of Surgery, Division of Neurosurgery, Hamilton General Hospital, McMaster University, Hamilton, Ontario L8L 2X2, Canada.

ABSTRA CT
Paragangliomas are extra-adrenal neuroendocrine tumors that derive from neural crest. In general, they are benign tumors but
few cases had shown a tendency to metastasize. Malignant forms have been reported previously with intracranial metastasis from
duodenal origin, but primary intracranial origin represents a rare and unusual location for such tumors. Here, we report a rare case of
a 48-year-old lady who presented with symptomatic right-sided insular mass with negative metastatic work up. A complete surgical
resection had been done with an unexpected diagnosis of primary gangliocytic paraganglioma with malignant features.

Key words: Brain, chemodectoma, gangliocytic paraganglioma, intracerebral, intracranial

INTRODUCTION intraparenchymal paraganglioma with malignant
features was encountered lately at our center. The
Paragangliomas are rare neuroendocrine tumors. case is considered to be the first of its kind, and it
They arise from the paraganglia distributed along the does reflect the potential risk for malignant primary
paravertebral sympathetic chains and related ganglia, growth of paraganglioma within the brain parenchyma.
as well as from the parasympathetic paraganglia such A review of the literature for intracranial supratentorial
as aortic body, carotid body, and vagal nerve. As a result paragangliomas along with the predisposing genetic
of their neuroectodermal origin, few paragangliomas mutation will be discussed.
can be functional. Hence, autonomic dysfunctional
symptoms may occur such as excessive sweating, CASE REPORT
hypertension, and tachycardia secondary to vasoactive
substances release. They are known to be benign tumors A 48-year-old healthy female presented to the
with WHO Grade I. However, around 5-10% of them may emergency department with 1-week history of
transform malignant along the course of the disease. [1] headache and slurred speech. She noticed that her
balance had got worse 3 days prior to presentation.
Paraganglioma may also arise from intracranial or On examination, she was noticed to be ataxic with left
intra-spinal origin. Several explanations were proposed sided pronator drift and left lower facial asymmetry.
for their existence which include possible growth from The initial computed tomography (CT) scan of her
ganglionic cells, growth from paraganglia associated brain showed an iso-dense mass in the right sub-insular
with blood vessels or growth from embryonic area measuring 3.6 cm × 4.1 cm × 3.7 cm, along
neuroepithelial rest. They have a tendency to with perilesional vasogenic edema and mass effect
[2]
grow in the sellar/parasellar region or cauda equina over adjacent structures [Figure 1]. There was no
region. However, the intraparenchymal growth of evidence of calcification or hemorrhagic foci within
paraganglioma is uncommon with seldom reported the lesion. It carried slightly low intensity signal in
cases in the literature. An unusual case of primary T1-weighted image with heterogenous signal intensity
in T2-weighted image. The lesion was well-demarcated
Access this article online and had homogenous gadolinium uptake with restricted
Quick Response Code: diffusion in diffusion-weighted image/apparent
Website:
www.nnjournal.net diffusion coefficient map [Figure 2].

DOI: The patient was admitted to the hospital for a right-sided
10.4103/2347-8659.154431 fronto-parieto-temporal craniotomy for tumor resection
guided by neuronavigation. Intraoperatively, the tumor

Corresponding Author: Dr. Ahmed A. Al Jishi, Department of Surgery, Division of Neurosurgery, Hamilton General Hospital,
McMaster University, 237 Barton St East, Hamilton, Ontario L8L 2X2, Canada. E‑mail: dr_aljishi@yahoo.com

Neuroimmunol Neuroinflammation | Volume 2 | Issue 2 | April 15, 2015 121