Case Report
Open Access
Rare Presentation of Cervical Vagal
Schwannoma in Adolescence: A Case Report
Demah Alayadhi1, Abdullah Arafat2*, Mohammed Elkarim2, Mohammed Alessa2, Ali Alassiri3
1College of Medicine, King Saud Ben Abdulaziz University of Health Sciences.
2Department of ORL-H&N Surgery, King Abdulaziz Medical City, National Guard Health Affairs.
3Department of Pathology and Lab Medicine, King Abdulaziz Medical City, National Guard Health Affairs.
2Department of ORL-H&N Surgery, King Abdulaziz Medical City, National Guard Health Affairs.
3Department of Pathology and Lab Medicine, King Abdulaziz Medical City, National Guard Health Affairs.
*Corresponding author:
Abdullah Arafat, Department of ORL-H&N Surgery, King Abdulaziz Medical City, National Guard Health Affairs; Saudi Arabia. E-mail:-
Received: December 06, 2017; Accepted: December 13, 2017; Published: December 21, 2017
Citation: Alayadhi D, Abdullah A, Elkarim M, Alessa M, Ali A (2017) Rare Presentation of Cervical Vagal Schwannoma in Adolescence: A Case Report. SOJ Surgery 4(3): 1-3. DOI: http://dx.doi.org/10.15226/2376-4570/4/3/00149
Abstract
Schwannoma is a rare benign tumor arising from the Schwann
cells that produce myelin sheath that cover cranial, peripheral and
autonomic nerves. Cervical vagal schwannomas are slow growing
asymptomatic solitary benign neck tumors. It affects females and
males equally, and it is most common between third and fifth decades
of age. Cervical vagal schwannoma requires high clinical suspicion to
be diagnosed because of its rarity. Imaging and FNA are a valuable
investigation to diagnose and differentiate cervical vagal schwannoma
from other diseases. The treatment of choice is complete surgical
resection of the mass and histopathology of the excised mass is
obligatory for a definitive diagnosis. We report a case of a 17-year-old
male patient presented with left neck mass who was diagnosed with
cervical schwannoma of vagal nerve origin, which is rare.
Introduction
Schwannomas, also known as neuromas, neurinomas, or
neurilemmomasis a rare benign tumor arising from the Schwann
cells that produce myelin sheath which covers nerve fibers [1,2].
It can originate from any cranial, peripheral and autonomic
nerves of the body except olfactory and optic nerves [13]. It has
been stated that schwannoma represents 5% of all soft tissue
tumors [4]. About one third of schwannoma is diagnosed in the
head and neck region [3]. Of the reported cases, approximately
half of the parapharyngeal schwannomas arise from the vagus
nerve [8]. Cervical vagal schwannomas are slow growing benign
tumors. Almost always, manifest as asymptomatic solitary
lateral neck mass that can be palpated along the medial border
of sternocleidomastoid muscle [8]. It affects females and males
equally, and it is most common between third and fifth decades
[5]. Cervical vagal schwannoma requires high clinical suspicion
to be diagnosed because of its rarity [8]. In addition, imaging is
important to diagnose and differentiate vagal schwannoma from
other disease [8]. The treatment of choice is complete surgical
resection of the mass and histopathology of the excised mass is
obligatory for a definitive diagnosis [5].
Case Report
A 17-yearsold male presented to our center with
progressive, slow growing left neck mass which was noticed
by his family 4 years ago. He had no obstructive symptoms,
dyspnea, dysphagia, or other associated symptoms nor was
there a history of radiation or family history of neck tumors.
Examination revealed hard, pulsatile, nontender mass placed
in the carotid space measured 3 x 4 cm with normal overlying
skin and no discharge. It was movable in horizontal but not in
vertical plane. There were no palpable lymph nodes and the
facial nerve was intact. Examination of the ear, nose and throat
was unremarkable. Flexible nasopharyngolaryngoscopy showed
normal bilateral vocal cord mobility. Ultrasound guided biopsy
of the mass showed benign spindle cell tumor consistent with
schwannoma, which was confirmed by immunochemistry and
was negative for malignancy. On MRI, there was a well-defined
homogenous enhanced mass displacing common carotid artery
and internal jugular vein posteriorly and laterally demonstrating
is intense signal on T1 WI, Hyper intense signal on T2 WI [Figures
1,2]. Subsequent contrasted CT revealed a well-defined oval
mass involving the left carotids sheath showing heterogeneous
faint enhancement with mild displacement of adjacent vascular
structures [Figure 3].
Based on patient’s presentation, examination and investigations, the patient was operated by transverse left cervical skin incision. Dissection of the all muscles was done. A yellowish mass measuring 3 x 4 cm was seen in the area between the carotid artery and internal jugular vein. Tumor was excised completely from the left vagus nerve, leaving the nerve intact. The diagnosis of schwannoma was confirmed by histopathology which reveals a schwannoma composed of fascicles of plump spindle cells with elongated tapering nuclei and moderately abundant, eosinophilic cytoplasm with indistinct cell borders. The cellularity is variable due to the characteristic zonation of alternating hypercellular areas (Antoni A) figure 4a, with distinctive focal nuclear palisading surrounding aggregates of cellular processes (Verocaybodies)
Based on patient’s presentation, examination and investigations, the patient was operated by transverse left cervical skin incision. Dissection of the all muscles was done. A yellowish mass measuring 3 x 4 cm was seen in the area between the carotid artery and internal jugular vein. Tumor was excised completely from the left vagus nerve, leaving the nerve intact. The diagnosis of schwannoma was confirmed by histopathology which reveals a schwannoma composed of fascicles of plump spindle cells with elongated tapering nuclei and moderately abundant, eosinophilic cytoplasm with indistinct cell borders. The cellularity is variable due to the characteristic zonation of alternating hypercellular areas (Antoni A) figure 4a, with distinctive focal nuclear palisading surrounding aggregates of cellular processes (Verocaybodies)
Figure 1: MRI of the neck, coronal section, T2WI: Well-circumscribed
oval-shaped Hyperintensemass.
Figure 2: MRI of the neck, axial section: Well-circumscribed oval-shaped
mass, Hyperintensity in T2WI (on the top) and hypointensityT1WI (on
the bottom).
Figure 3: Contrast Computed Tomography of the neck, coronal section:
Well-circumscribed oval-shaped which displace the CA and IJV posteriorly
and laterally
Figure 4A: Clow power view showing alternating areas of Antoni A (hypercellular;
blue star) and Antoni B (hypocelluar; black star)
Figure 4B: Verocay body (circles), a distinctive feature of schwannoma
resulting from regimentation of Schwan cell nuclei with nuclear free
zone on either side, also seen hyalinized vasculature which is a common
feature (arrows).
figure 4b, and looser hypocellular areas (Antoni B) figure 4b
[Figure 4a, Figure 4b]. Thick-walled, hyalinized blood vessels are
also present figure 4b.Patient was followed as an outpatient at
2 weeks, 6 weeks, 3 months, 4 months, 8 months and 1 years.
Patient developed mild left ptosis 2 weeks postoperatively, which
was recovered spontaneously after 3 months and both vocal
cords were intact in post-operative follow up.
Discussion
Schwannoma is a benign tumor arising from the Schwann
cells that produce myelin sheath that cover cranial, peripheral
and autonomic nerves [1,2]. It presents as encapsulated, well
circumscribed, firm, nodular lesions [2]. It has been stated
that schwannoma represent 5% of all soft tissue tumors [4].
About one third of schwannoma is diagnosed in the head and
neck region, and the most common involves cranial nerve is
the eighth nerve, called vestibular schwannoma (3). Compared
to vestibular schwannoma, cervical vagal schwannoma is a
rare tumor [5].Cervical vagal schwannomas are slow growing
tumors that adhere to their nerve of origin [6]. Majority of vagal
nerve schwannomas are benign, and malignant transformation
is rare [6]. Most of patients with cervical vagal schwannoma
manifest as asymptomatic solitary lateral neck mass [8]. The
most common presenting symptom is hoarseness, but the most
specific sign for cervical vagal schwannoma is paroxysmal cough
[8]. Paroxysmal cough is elicited by stimulation of the vagal
nerve during mass palpation [4].Carotid Artery (CA) is displaced
anteriorly and medially by cervical vagal schwannomas while
Internal Jugular Vein (IJV) is displaced posteriorly and laterally
[6]. The mass is almost always mobile in the horizontal but not
in vertical direction [6]. The differential diagnoses of the mass
could be paragangiloma, branchial cleft cyst, carotid body tumor,
malignant lymphoma, metastatic cervical lymphadenopathy,
and cervical vagal schwannoma [9]. Preoperative diagnosis of
cervical vagal schwannoma is difficult because of its rarity [3].
FNA and imaging can be helpful preoperatively to diagnose and
differentiate cervical vagal schwannoma from the other diseases
[3]. FNA value is questionable since the treatment of choice is a
complete surgical resection of the mass [9]. MRI is considered
the gold standard for cervical vagal schwannoma, whereas CT
findings usually nonspecific and not helpful [1,9]. On MRI, the
lesion is heterogeneously hyper intense in T2WI and hypointene
in T1WI [10]. After gadolinium administration, the lesion showed
heterogeneous hyperintesity [10]. MRI findings are important to
differentiate schwannomas by its origin in relation to vascular
displacement. The vagus nerve schwannomas displace CA
medially and IJV laterally, where schwannoma of the sympathetic
chain leads to displacement of these vessels without separation
[5,1]. Also, MRI is useful to differentiate tumors of carotid body
which splay the carotid bifurcation; lyre sign [1]. In addition, MRI
is used to exclude paragangliomas which have salt and pepper
sign, from schwannomas. So, salt represents the hyperintense
(white part) of mass due to hemorrhage in the hyper vascular
tumors, where pepper represents hypointense (black part) of the
mass due to increase blood flow in the vessels [11]. A complete
surgical resection of the tumor from the vagus nerve is the goal
of the treatment with attempt to preserve the vagal pathway
[7]. If the complete resection of the tumor with preservation of
the vagus nerve was impossible, the best choice in this case is
to reset the involved segment and end to end anastomosis [8].
The most common complication postoperatively is hoarseness,
so preoperative focal cord mobility assessment is necessary
[5]. In preoperatively assessment, vocal cord paralysis has been
reported in 12% of cases [5,8]. Whereas postoperatively, vocal
cord paralysis was reported in 85% of cases [8]. In case of vocal
cord paralysis postoperatively, aggressive voice therapy should
start as soon as possible after the surgery to compensate the vocal
cord [8]. Other possible complications are dysphagia, dysphonia,
Horner’s syndrome, facial myotonia, hypoglossal palsy, and facial
palsy [8]. We have presented a rare case of vagal schwannoma in
an adolescent male with a few previously published cases in the
literature [5].
Competing Interests
The authors declare that there is no conflict of interests
regarding the publication of this paper
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