IOSR Journal Of Pharmacy

(e)-ISSN: 2250-3013, (p)-ISSN: 2319-4219

www.iosrphr.org Volume 5, Issue 5 (May 2015), PP. 08-11

Cervical Sympathetic chain ganglioneuroma : case report and

review of literature
Dr. Ashish Gopal 1 , Dr. Sangeeta Singh 2 , Dr. Meenakshi Sridhar3
Dr. Nita Khurana 4
1

Department of ENT, Aruna Asaf Ali Govt Hospital .Rajpur Road,Tis Hazari, Govt. of N.C.T, New Delhi110054,India
2
Department of ENT, Aruna Asaf Ali Govt Hospital .Rajpur Road,Tis Hazari, Govt. of N.C.T, New Delhi110054, India
3
Department of Pathology , B.S.A. Hospital. Sect 6, Rohini, Govt. of N.C.T, New Delhi, India
4
Department of Pathology , Maulana Azad Medical College ( MAMC), Delhi-110085,INDIA

ABSTRACT: Ganglioneuroms are benign tumor of the autonomic nerve fibers, arising from neural crest and
the reported incidence of ganglioneuroma is one per million population. They usually present in patients under
20 years of age with a slight female predominance .The most common localization is the posterior medistinum
followed by the adrenal gland ,retroperitoneum (sympathetic ganglia) ,and head& neck.In the neck cervical
sympathetic chain is the most frequent site of origin . Cervical Sympathetic chain ganglioneuromas are usually
asymptomatic neck masses and complete surgical resection is the treatment of choice. Surgical excision via a
cervical approach offers definitive therapy but may be associated with an iatrogenic Horner's syndrome for
which the patients should be counseled prior to operative procedure . Being quite rare among other neurogenic
tumors, A case of cervical Sympathetic chain ganglioneuroma is presented and the literature is reviewed.

KEY WORDS- Cervical ganglioneuroma, Head and neck tumors, neurogenic neck tumours , sympathetic
chain.

I. INTRODUCTION:
Ganglionueroma occurring in the neck are uncommon. Shumacker and Lawrence ( 1939) state that
Cervical Sympathetic chain ganglioneuromas is one of the rarest of neck tumors. Cervical sympathetic chain is
the most frequent structure of origin in the neck. Other sites of origin include the larynx, pharynx and ganglion
nodosum of the vagus nervei. Ganglioneuromas can be found in the central nervous system or peripherally in
the sympathetic system Unusual sites include the spermatic cord, heart, bone, and intestine [2]It is a slow
growing tumour with so far no recorded metastatic potential. Surgical excision is the treatment of choice. We
present a case of cervical sympathetic chain ganglioneuroma discussing the diagnosis, treatment protocols and
postoperative complications of this benign tumor.
CASE REPORT : A 38 years old female presentd with diffuse fullness on the right side neck for last 5 years.
(Fig 1). Examination revealed a lobulated, painless, immobile mass of 10cm x 5 cm size with normal overlying
skin . The displacement of the carotids was so anterior that it can be seen pulsating and appeared quite sinister.
Fine needle aspiration cytology (FNAC) showed a preponderance cluster of mature ganglion cells and mixture
of spindle Schwann cells. A diagnosis of ganglioneuroma was suggested Ultrasonography was done to note a
round, homogeneous, mixed lesion . Magnetic resonance imaging (MRI) depicted a well defined spindle mass
lesion of 9 cm x 4 cm originating from the carotid sheath, encroaching upon the right parapharyngeal spaces,
displacing the common carotid and the external carotid anteriorly and causing compression of adjacent soft
tissue and vascular structure suggested a neurogenic tumor. Abdominal ultrasonography (USG) showed no
abnormality and urinary vanillylmandelic acid (VMA) levels were normal Patient was planned for surgical
excision. Neck exploration was carried out, The dissection was rendered tedious due to the superficial lying
great vessels and a compressed internal jugular veins. The vagus nerve identified and preserved along with the
vessels and it was retracted anterior and medially to define a fusiform lesion arising from the sympathetic chain
, when traced upward towards the neck a stump of 3 cm of the nerve was identified (Fig 2). The surgical
removal of the lesion was however done with ease, owing to the well encapsulated nature of the lesions, an
intracapsular dissection could not be feasible and wound was closed after provision of surgical drain . On
macroscopy, it was a thick-walled, tangled, pedicled mass 12 cm 15 cm in sizeSubmitted histopathology,
showed numerous mature ganglion cells with eccentrically placed nucleus in background of Schwann cell
proliferation .Histological appearance of more than 50% presence of Schwann cell population along with

Cervical Sympathetic chain ganglioneuroma ..

neuroblastic cells confirms the diagnosis ( Fig3) . Within the first day of the operation, the patient developed
Horner syndrome, characterized by myosis and ptosis. She had also difficulty in swallowing her saliva. One
month after the operation, ptosis and difficulty in swallowing recovered, but myosis persisted. The tumor did not
show any signs of regrowth or recurrance within 30 months after the operation.

II. DISCUSSION AND REVIEW OF LITERATURE

Medical literature is replete with noting on rarity of ganglioneuroma. In 1939, Schumacker and
Lawrence1 commented that cervical sympathetic chain ganglioneroma was indeed the rarest neck tumours.
Elsewhere in the neck region they arise from the larynx, pharynx, and the hypoglossal nerve, along the length of
the vagus and the intervertebral foramina and thereby extending to even the spinal cord. It can also manifest in
the thoracic cavity (posterior madiastinum), abdominal cavity (retro-peritoneum, adrenals and pelvic
sympathetic ganglia) as well as in the orbit. The primordial tissue of sympathetic nervous system, is the seat of
origin of neuroblastic neoplasm which is neural crest and neural tube derived. as per the Shimada classification
,The family of neuroblastic neoplasm includes three histological variants; neuroblastoma, ganglioneuroblastoma and ganglioneuroma, During the pathological progression , this neoplasm shows different
degree of maturation with complete immature cells in neuroblastoma, the ganglio-neuroblastoma and
ganglioneuroma follows next in line, depending on degree of maturation2. The first two are the malignant
spectrum of this neoplasm. However the recent International Neuroblastoma Pathology Committee3 has in
histological detail to classify them in four categories viz the
i.

neuroblastoma,

ii.

neuroblastoma intermixed with ganglio-neuroblastoma,

iii.

nodular ganglio-neuroblastoma and

iv.

ganglioneuroma.

This is the modification of Shimadas classification. The same classification further sub-divides
ganglioneuroma into matured and immature types depending upon the percentage of presence of Schwann cells
and neuroblastic cells. Thus the ganglioneuroma is a well differentiated neoplasm of the younger aged
population between 2-15 years, with more neural tissue and scanty Schwann element and no chromaffin
element. It is confusing to know the differences in neurogenic tumours till we understand them according to
their origin. Nerves consist of neural tissue with supporting tissue like schwann and closely connected
paraganglia. Neurogenic tumours may arise from neural cells itself as in the case of ganglioneuroma and its
subtypes, or from paraganglion cells, like carotid body tumours and paragangliomas, and lastly from the
supporting tissue like Schwann cells which comprises of schwannoma.. The pathology may not ring a bell in the
doctors mind due to its rarity in presentation. The gross suspicion of this neoplasm is made when it presents
with either compression symptoms or a large mass , which are generally late. Autonomic dysfunction like
diarrhoea, profuse sweating, virilisation, hypertension and alopecia are attributed to immature neuroblastic
tumours. These autonomic dysfunctions as a result of catecholamine secretion occur in 37% of these cases. They
are slow growing tumour with so far no recorded metastatic potential and complete excision being suffice to
bring about a cure. Ganglioneuroma should be distinguished from the immature forms like neuroblastomas and
neurofibroma. Histological appearance of more than 50% presence of Schwann cell population along with
neuroblastic cells confirms the diagnosis. The metaiodobenzylguanidine scan (MIBG) is trusted to show 88%
sensitivity and 99% specificity for
these tumours along with carcinoid and pheochromacytoma.
Immunohistochemistry, in which ganglion cells stain for neuron specific enolase (NSE) and Schwann cells
stain for S-100 protein5, Useful to identify the biochemical and immunology of cell by using antigen and
visible labelled tagged antibodies. Computed tomography (CT) scanning is a preferred methods for imaging
ganglioneuromas and ganglioneuroblastoma6,7,8. MRI is the modality of choice for evaluating the extension of
tumors.9. and provides important pre-operative information for planning optimal surgical treatment.
Immunohistiochemistry is important to identify the biochemical and immunology of cell by using antigen and
visible labelled tagged antibodies, to differentiate specific cellular components occur in 37% of these cases
which secrete vanillymandelic acid and homavanillic acid and the presence of tumour on sites as ganglia and
retro-peritoneal adrenals4

Cervical Sympathetic chain ganglioneuroma ..

.
Fig 1:

Fig 2- Histopathology of ganglioneuroma composed of mature ganglion cells in Schwannian stroma (H.E.
440).

Fig 3 - Peroperative view of the mass with its distal edematous end continuous with neural components of the
neck.

III.

CONCLUSION

Neuroblastic tumors of the head and neck, namely, neuroblastoma, ganglioneuroblastoma and
ganglioneuroma are rare entities, accounting for 6% of the tumors of childhood. Ganglioneuromas are most
frequently diagnosed in patients between the ages of 10 and 29 years. and are most commonly located in the
posterior mediastinum followed by the retroperitoneum.2They are usually located in the abdomen (6580%), or
thorax (1015%), and rarely in the neck (5%) [3]. These tumors define a spectrum of sympathetic
neuroectodermal tumors, ranging from the undifferentiated neuroblastoma to the mature ganglioneuroma. The
presence of immature tissue in neuroblastoma and ganglioneuroblastoma indicates malignant or potentially
malignant behavior; ganglioneuroma is composed entirely of ganglion cells and Schwannian stroma, which is
considered benign [4]. A case of ganglioneuroma was first reported by Loretz in 1870 and ganglioneuroma of
the neck was first reported by De Quervain in 1899 [1]. Ganglioneuroma arises under the age of 20 years in 60%
of the cases, the median age of presentation is approximately 7 years with a slight preponderance of cases in

10

Cervical Sympathetic chain ganglioneuroma ..

females [1], [4]. Compared to other neuroblastic tumors, ganglioneuroma occurs in older patients. The ratio of
neuroblastoma to ganglioneuroma is approximately 6:1 to 10:1 [2]. Ganglioneuroma most often manifests as an
asymptomatic mass. Clinically, signs and symptoms of cervical ganglionueromas are usually related to
compression of vital structures such as nerves or vessels of the neck. The tumors involving the cervical
sympathetic chain causes Horner syndrome characterized by ptosis, myosis, ipsilateral facial anhydrosis and
flushing. In 37% of cases, catecholamines secreted by the tumor may increase levels of VMA or homovanillic
acid (HMA) in the plasma and urine, which may lead to hypertension, diarrhea, sweating, flushing and renal
acidosis [1], [5]. We did not detect an elevation of hormonal levels in our patient. In I131-MIBG scintigraphy an
increased uptake in the tumor tissue can be detected [4]. Most characteristic histologic feature of
ganglioneuromas is the presence of mature ganglion cells. However in 25% of ganglioneuromas elements of
immature neurogenic tumors can be seen. It is hypothesized that neuroblastic tumors undergo a maturational
process or spontaneous regression of neuroblastoma to ganglioneuroma [1]. Macroscopically, ganglioneuroma
may appear encapsulated, although a true capsule is infrequent [2]. The cervical sympathetic chain is the most
frequent structure of origin in the neck. Other sites of origin are larynx, pharynx and ganglion nodosum of the
vagus nerve [2]. Apart from neuroblastic tumors, there are other rare tumors of the neck. Ganglioneuroma may
be mistaken for such tumors as paraganglioma, oncocytoma, lipoma, plasmocytoma, embryonic
rhabdomyosarcoma, Ewing's sarcoma and non-Hodgkin lymphoma and such infections as actinomycosis,
toxoplasmosis, tuberculosis, infectious cervical lymphadenitis and branchial cleft cyst [3], [6]. USG reveals a
homogeneous, hypoechoic mass with well-defined borders however MRI and computerized tomography (CT)
are more valuable imaging tecniques in the diagnosis of ganglioneuroma. MRI is superior to CT in the diagnosis
of intraspinal tumors [7]. The tumor has been shown to have a capsule and calcifications both radiologically and
histologically [5], [8]. Although ganglioneuroma tends to be a more homogeneous tumor than neuroblastoma or
ganglioneuroblastoma, it is not possible at imaging evaluation to discriminate among these three tumors [2].
There are rare reports of metastatic ganglioneuroma. It is believed that these tumors represent metastases of
neuroblastoma or ganglioneuroblastoma that have subsequently matured to ganglioneuroma; these patients have
an excellent prognosis [4].
Surgical excision is the treatment of choice to confirm the diagnosis and prevent further growth and
compression of neighboring structures. These tumors are not aggressive, and even in the case of incomplete
resection, residual ganglioneuroma will not regrow or produce symptoms [3]. Significant morbidity may occur
as a result of intraoperative sacrifice of neural structures and vasculature associated with the tumor. Horner
Syndrome and significant aspiration of thin liquids are frequent complications. However these symptoms
usually resolve in the postoperative follow-up [1]. Chemotherapy and bone marrow transplantation are also used
in the treatment [2]. Radiotherapy should be avoided in children as it increases the risk of growth retardation and
tumor development secondary to radiation [3]. Atives in this above mentioned case report. 2They are usually
located in the abdomen (6580%), or thorax (1015%),.

REFERENCES
[1]
[2]
[3]

[4]
[5]

[6]
[7]
[8]

[9]

H.B.Schumaker Jr., and E Lawrence, Cervical sympathetic ganglioneuroma: Case report and review of
literature. Surgery 5, 1939,572-81
K.E. Bove , A. J. Mc Adams : Composite ganglioneuroblastoma an assessment of the significance of
histologic maturation in neuroblastoma diagnosed beyond infancy. Arch Path Lab Med ,105,1981, 325
H.Shimada , I.M.Ambros ,L.P. Dehner : Terminology and morphologic criteria of neuroblastic
tumours. Recommendations by the International Neuroblastoma Pathology Committee. Cancer
86,1996 349-363
B.George B.,B.Hero ,D. Harm. : Metabolic activity and clinical features of primary ganglioneuromas.
Cancer 2001 May 15; 91(10): 2001, 1905-1913.
J.Q.Trojanowski ,W.M.Molenar., D.L.Baker D.L: Neural and neuroendocrine phenotype
neuroblastoma, ganglio-neuroblastoma, ganglioneuroma and mature versus embryogenic human
adrenal medullary cells. Prog.Clin. Biol. Res.; 366: 1991 ,335-341,.
R.Rabah ,R. Weber , GKSerhatkulu ,A Cao , H. Dai , A. Pandya . Diagnosis of neuroblastoma and
ganglioneuroma using Raman spectroscopy. J Pediatr Surg., 43(1), Jan 2008, 171-176
Y.K.Guo , Z.G..Yang , Y.Li , .Y.P..Deng , E.S..Ma ,PQ Min . Uncommon adrenal masses: CT and
MRI features with histopathologic correlation. Eur J Radiol Jun 2007; 62(3) :359-370
J Stutterheim , A.Gerritsen , L.Zappeij-Kannegieter , B.Yalcin ,R. Dee , MM.van Noesel . Detecting
Minimal Residual Disease in Neuroblastoma: the Superiority of a Panel of Real-Time Quantitative
PCR Markers. Clin Chem. May 21 2009,
GJ Lonergan , CM Schwab , ES Suarez , CL Carlson . Neuroblastoma, ganglioneuroblastoma, and
ganglioneuroma: radiologic -pathologic correlation. Radiographics; 22(4) Jul-Aug 2002, 911-934

11