ANCIENT SCHWANNOMA OF CERVICAL

SYMPATHETIC CHAIN - A RARE ENTITY

*Tanvi Anoop Lohiya, **Kshitij Dhaval Shah, ***Ashutosh Vishwasrao Patil, ****Renuka Anil Bradoo
ABSTRACT
Schwannomas arising from the cervical sympathetic chain in the parapharyngeal region, are a rare entity. Among
the various histological types of schwannomas reported to date, the ancient (degenerative) variant is the most
rare. The treatment of choice is excision of the tumour. We describe a case of a schwannoma arising from the
cervical sympathetic chain, with the rare histopathology of ancient schwannoma.
Keywords: Schwannoma; ancient schwannoma; cervical sympathetic chain.

Cervical sympathetic schwannoma is a rare ,

benign, slowly growing tumour which develops from
the schwann sheath of peripheral nervous system nerve
fibres. Schwannomas in the parapharyngeal space may
arise from cranial nerves 9th, 10th, 11th and 12th or
the autonomic nerves, the vagus being the most common
site2. Schwannomas have been classified histologically
into five types: common, plexiform, cellular, epithelioid
and ancient. Among these, the ancient schwannoma is
the most rare3. The term ancient is given to cases
showing typical neurilemmoma features with
hyperchromatic areas and bizarre nuclei. Due to their
longstanding course, these tumours develop
degenerative (ancient) changes in the form of cystic or
myxoid degeneration.
CASE REPORT:
A 40 year old female presented with complaints
of painless swelling on the left side of the neck since 3
months. The swelling was insidious in onset and
gradually progressed to its current size. There were no
complaints of dysphagia, dysphonia or neurologic
deficit. On examination, there was a 4 x 3 cm firm,
non tender mobile swelling with normal overlying skin
in the left carotid triangle.An MRI with gadolinium
contrast revealed a 5 x 2.9 x 3.2 cm sized well defined
T2 STIR hyperintense oval lesion in the left carotid
space posteromedial to the carotid bulb, ICA and ECA
extending from C2 vertebra superiorly to superior
endplate of C5, showing intense heterogenous post

contrast enhancement in venous and delayed phases with

multiple non enhancing foci within, suggestive of a
nerve sheath tumour like a schwannoma/neurofibroma
(figure 1 a b & c)
. A USG confirmed no vascularity, hence
ruling out the possibility of a paraganglioma. The
patient was counselled and advised surgical excision of
the tumor. After obtaining valid informed consent, the
surgery was performed under general anaesthesia. A
transverse skin crease neck incision was used and
subplatysmal flaps were elevated. The anterior border
of sternocleidomastoid was delineated and the internal
jugular vein and carotid artery were identified and
delineated. Carotid artery bifurcation was exposed and
retracted by umbilical tape slings to expose the
tumour(fig 2a). The tumour was visualised arising from a
nerve trunk, viz., the sympathetic chain. Decision was
taken to sacrifice the nerve trunk as the tumour was
intracapsular. Tumour was dissected and delineated
from all sides. Nerve trunk was cut and the specimen

Vol.-9, Issue-II, July-Dec. - 2015

INTRODUCTION

Affiliations:
*Senior Registrar,**Assistant Professor,
***Registrar,****Professor & Head
Department of E.N.T,
Lokmanya Tilak Municipal Medical College
and General Hospital, Sion, Mumbai - 22
Address of Correspondence:
Dr Tanvi Lohiya
Senior Registrar,
Department of E.N.T,
Lokmanya Tilak Municipal Medical College
and General Hospital, Sion, Mumbai - 22
Row House no. 4, Kanakia Spaces, Eternity,
Teen Haath Naka, Thane, Maharashtra, 400604

37

was delivered(fig 2b). A suction drain was placed and the

wound was closed in layers. Patient recovered well from
the surgery.
The excised specimen was sent for histopathological
assessment which was reported as spindle cells with
elongated nucleus in a prominent myxoid extracellular
matrix along with Verocay bodies, suggestive of
Ancient schwanomma arising from the sympathetic
trunk(fig 3).

may represent degenerated Antoni A tissue are the main

features of Type B. Occasionally schwannomas may
present with hyperchromatic areas and bizarre nuclei.
These cellular changes reflecting degeneration are
classical of the so-called ancient schwannoma. Sparse
mitotic hyperchromatic nuclei and degenerative
changes, such as cyst formation, calcification, with only
occasional sites of hemorrhage are the major
histopathological characteristics of the ancient forms

The patient developed Horners syndrome post

surgery, in the form of ptosis of the left eyelid(fig 4) which
recovered gradually.
DISCUSSION:
Cervical schwannoma was first reported by Ritter
in 1899. They are typically solitary, well-encapsulated,
benign tumours characteristically running along the
course of a nerve or attached to peripheral, cranial, or
sympathetic nerves. Although the head and neck region
accounts for 2540% of the schwannomas, it is
extremely rare to develop an ancient schwannoma in
the parapharyngeal region. The term ancient
schwannoma was first coined by Ackerman and Taylor
in their review of 48 neurogenic tumours of the thoracic
region5.

Fig. 2: (a) Tumour delineated after retraction of IJV Nerve of

Origin sympathetic chain shown by the arrow, (b) Cut ends
of the nerve after excision of the nerve.

Microscopically, schwannomas have a distinctive

pattern of compact cellularity arranged with palisading
nuclei (Antoni A pattern) alternating with a more
loosely arranged hypocellular pattern (Antoni B
pattern). Nuclear palisading and associated Verocay
bodies, which may reflect their prominent extracellular
matrix and secretion of laminin are the dominant
characteristics of Type A tissue, whereas a loose
organization with myxomatous and cystic changes that

Vol.-9, Issue-II, July-Dec. - 2015

Fig. 3: Gross & Microscopic images (H & E stain, 40

Fig. 1: Imaging a) Contrast enhanced CT (b)T1 weighted

MRI axial view (c) T2 weighted MRI sagittal view
38

Fig. 4: Post operative Horners syndrome.

CONCLUSION:
Schwanomma arising from cervical sympathetic
chain in the parapharyngeal region is a rarity. Ancient
schwanomma, a pathological variant, is found rarely.
We have reported this case due to its rarity of

anatomical site of origin and pathology. The

management remains the same as that of a schwanomma
in any other part of the body.
DISCLOSURES
(a)

Competing interests/Interests of Conflict- None

(b) Sponsorships - None

(c)

Funding - None

(d) No financial disclosures

REFERENCES
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Sheridan MF, Yim DWS. Cervical sympathetic

schwannoma:a case report and review of the English
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G. Extracranial head and neck schwannomas. J
Otolaryngol 1992;21:186-8.

2.

Bozec A, Dassonville O, Poissonnet G, et al.

Laryngeal schwannoma: a case report. Ann
Otolaryngol Chir Cervicofac :2003;120:404.

Weiss SW, Goldblum JR. ParotisSchwannoma.

In: Enzinger FM, Weiss SW, eds.Soft Tissue
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4.

Walther LE, Mentzel T, Sens A, Beleites

E.Degenerative changes in parapharyngealschwannoma ancient schwannoma. Case
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5.

Ackerman LV, Taylor FH. Neurogenoustumors

within the thorax; aclinicopathological evaluation
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6.

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Otolaryngol Pol. 2014SepOct; 68(5): 2715.doi:
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13.PubMed PMID: 25283326.

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Vol.-9, Issue-II, July-Dec. - 2015

of schwannomas.One of the most misleading aspects

of this tumor is the degree of nuclear atypia. These
tumors behave as ordinary neurilemomas and therefore
the nuclear atypia can be regarded as a purely
degenerative change.A contrast enhanced CT or MRI
is the investigation of choice. Anteriorly displaced
internal carotid artery is characteristic of neurogenic
and vagal paraganglioma, whereas salivary gland tumors
generally cause posterior displacement. On CT
examination without contrast, a Schwannoma is
generally hypodense compared to the muscle; with
contrast, this lesion shows some degree of enhancement.
MRI reveals low signal intensity on T1 and high signal
intensity on T2-weighted images. Paraganglioma, on
the other hand, is classically isodense when compared
to muscle on pre-contrast CT, with more reliable
homogeneous enhancement post-contrast. The postgadolinium MRI sequences of paraganglioma show
extremely bright contrast enhancement in a
characteristic salt and pepper pattern, representing the
low signal intensity of vascular flow voids which is not,
however, pathognomic for paraganglioma, but may be
found with hypervascular lesions. But rarely, cervical
sympathetic schwannomas can also cause splaying of
the ECA and ICA and mimic the Lyre sign, giving
rise to false-positive Lyre sign6. The definitive treatment
is surgical excision. Horners syndrome is to be expected
following removal of the tumour, due to the necessary
division of the cervical sympathetic chain, but this does
not appear to have an adverse effect on the patient.
The ptosis due to paralysis of Mllers muscle can be
repaired through slight advancement of the levator
aponeurosis, or resection of the conjunctiva and
Mllers muscle.