Nitish Kumar Baisakhiya  ( Department of ENT, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe) Wardha, India. )
S. Mukundan  ( Department of Surgery, Mahatma Gandhi Institute Medical Sciences, Sevagram, Wardha, India. )

Ganglioneuroma (GN) has a neuroectodermic origin and is localized along the sympathetic trunk. GN of the neck is a rare tumour and due to proximity to the thyroid gland, clinically and radiologically, these lesions can be mistaken as thyroid swellings. Definite diagnosis only can be suspected after surgical exploration and complete surgical excision is the treatment of choice, as it will ensure thorough sampling of the tumour and cure.

Ganglioneuroma (GN) of the neck is a rare tumour that most commonly presents as an enlarging neck mass.^1,2 In the neck due to proximity to the thyroid gland, clinically and radiologically, these lesions can be mistaken as thyroid swellings (i.e. goiter)³ and diagnosis only can be suspected after surgical exploration as in the present case.^1,3

A 22-year male presented with a 10-year history of a gradually increasing, painless mass in the left side of his neck without any other associated symptoms (Fig. 1). On examination the mass was situated laterally in the neck in the carotid triangle and was moving with deglutition. Fine needle aspirate cytology revealed clusters of colloid cells. Ultrasound detected a hypoechoic hypovascular mass situated near the upper pole of the thyroid. With all these findings a diagnosis of non-functioning thyroid goiter was made. During surgery there was a firm capsulated mass measuring 3 x 2.5 x 3 cm present in the left carotid space separate from the thyroid gland and it could be totally excised. The cut surface was whitish and had a whorled pattern (Fig. 2). Histopathology revealed an encapsulated tumour with low cellularity and a variably loose stroma showing degenerative changes. Two distinct cellular subgroups were identified microscopically: large ganglion cells and Schwann cells. A diagnosis of ganglioneuroma with degenerative changes was made. Post operatively the patient developed Horner's syndrome (Left ptosis, miosis, enophthalmos and absence of ciliospinal reflex) (Fig. 3). He underwent a Fasanella Savant procedure for correction of left ptosis and is doing well at follow up.[(f1)] [(f2)]

Tumours of sympathetic nervous system (neuroblastoma, ganglioneuroblastoma and ganglioneuroma) are derived from neural crest cells. Histological differentiation of these tumours are directly related to their clinical behaviour. Ganglioneuroma consists of mature Schwan cells and ganglion cells. Neuroblastoma and ganglioblastoma are malignant and the proportion of neuroblastoma element determines the histological grading and prognosis.^4,5 Ganglioneuroma is a benign tumour. Ganglioneuroma usually occurs in older population and in literature contrary to our case there is a slight female predominance.⁶ The most common locations for GN to occur are the posterior mediastinum, retro peritoneum, adrenal gland, and neck (8%) respectively. Unusual sites include the spermatic cord, heart, bone, and intestine.^7-9 In the neck it is sometime confused with other neck swellings i.e. thyroid swellings. Complete surgical excision is the treatment of choice, as it will ensure thorough sampling of the tumor and cure.^2,3,6,9

1. Barthelemy I, Belveze P, Emering C, Reynaud P, Beaujard H, Peri G, et al. [Cervical ganglioneuroma. Review, apropos of a case] Rev Stomatol Chir Maxillofac 1998; 99:210-3.
2. Cannady SB, Chung BJ, Hirose K, Garabedian N, Van Den Abbeele T, Koltai PJ. Surgical management of cervical ganglioneuromas in children. Int J Pediatr Otorhinolaryngol 2006; 70:287-94.
3. Leonardis M, Sperb D, Alster C, Campisi C, Herter NT. Ganglioneuroma of the neck, masquerading as a goiter. Eur J Surg Oncol 2003; 29:929-30. 
4. Askin FB, Perlman EJ. Neuroblastoma and peripheral neuroectodermal tumors.       Am J Clin Pathol 1998; 109:S23-30.
5. Joshi VV, Cantor AB, Altshuler G, Larkin EW, Neill JS, Shuster JJ, et al. Age-linked prognostic categorization based on a new histologic grading system of neuroblastomas: a clinicopathologic study of 211 cases from the Pediatric Oncology Group. Cancer 1992; 69:2197-211.
6. Geoerger B, Hero B, Harms D, Grebe J, Scheidhauer K, Berthold F. Metabolic activity and clinical features of primary ganglioneuromas. Cancer 2001; 91:1905-13.
7. Jain M, Shubha BS, Sethi S, Banga V, Bagga D. Retroperitoneal ganglioneuroma: report of a case diagnosed by fine-needle aspiration cytology, with review of the literature. Diagn Cytopathol 1999; 21:194-6.
8. Mithofer K, Grabowski EF, Rosenberg AE, Ryan DP, Mankin HJ. Symptomatic ganglioneuroma of bone: a case report. J Bone Joint Surg 1999; 81:1589-95.
9. Goldman R, Winterling AN, Winterling CC. Maturation of tumor of the sympathetic nervous system.Report of long-term survival in 2 patients, one with disseminated osseous metartases, and review of cases from literature. Cancer 1965; 18:1510-6.