Chirurgie du cancer

Abstrait

Cervical Ganglioneuroma in a Six Year Old Boy

Piyush Ranjan and Manish Kumar

Ganglioneuromas are benign neoplasm of neuroblastic origin which arises from central or peripheral parts of the autonomic nervous system. They are normally found at posterior mediastinum, retroperitoneum, and the adrenal gland but ganglioneuromas are rarely found in the cervical region. Here, we present a case of cervical ganglioneuroma in a six year old male child who presented with slow growing painless lateral neck swelling since birth.

Introduction

GanglioNeuromas (GN) are benign, neoplasm of neuroblastic origin which arises from central or peripheral parts of the autonomic nervous system. Besides, common sites such as posterior mediastinum, retroperitoneum, and the adrenal gland, GN are rarely demonstrated in the cervical region. As reported in literatures [1,2] only 1% to 5% of the patients present with neck masses.They are usually seen in children and young adults of age less than 20 years with a slight female predominance.

Case Report

A 6 year old boy presented with a slow growing mass in the left lateral side of the neck since birth slowly increasing in size especially in last two years.Mass was 7×5×3 cm,firm in consistency and was non tender.It was not associated with any systemic or compression related symptoms. There was no relevant medical,family and personal history.With provisional diagnosis of tubercular lymphadenitis/ lymphoma,two cervical lymph nodes were excised and sent for excisional biopsy to render the diagnosis,largest measuring 3×2×2 cm and smallest being 3×1.5×1 cm.On cut surface,they were pale white and firm in consistency.Onmicroscopy,it showed well encapsulated tissue bit containing few scattered mature ganglion cells surrounded by schwanian rich stroma suggesting Ganglioneuroma-Schwanian dominant neuroblastictumor. Further on IHC(ImmunoHisto Chemistry),the section was positive for various IHC markers like S100,Calretinin,Synaptophysin,CGA,Ki-67 suggesting mature ganglioneuroma.Radiologically,a contrast enhanced MRI was done which showed a large 6.5×4.5 cm T1W hypo and T2W hyperintense mass lesion seen at left carotid space region and extending superiorly in to parapharyngeal space and inferiorly upto cricoid cartilage displacing the neck vessels(carotid and jugular vein) antero laterally.Lesion showed intense enhancement after contrast administration.In addition multiple variable sized cervical lymph nodes were seen on left side.Features were suggestive of Ganglioneuroma with cervical lymphadenopathy.Then surgical excision was planned. Under general anaesthesia,a transverse incision was given over the swelling for the removal of tumor. The internal jugular vein and thecommon carotid artery which were displaced by the tumor were retracted and preserved. The excised lesion was well circumscribed, making an intact removal possible. The patient was extubated immediately after the surgery with no clinical signs of respiratory distress.The patient was well and without any complaints in the postoperative period.present in the sympathetic ganglia and adrenal medulla, indicating that these tumors may grow nearly anywhere along their distribution. Ganglioneuroma usually occurs in the abdomen or thorax, but it can also occur in the head and neck region, with the latter being it’s least common location. Actually, this is one of the rarest neck tumors. The first report of a ganglioneuroma located in the neck was made in 1899 by de Quervain [4] .The most frequent origin in the neck region is the cervical sympathetic chain, but it can also be located in the larynx, pharynx, and ganglion nodosum of the vagus nerve [5].

present in the sympathetic ganglia and adrenal medulla, indicating that these tumors may grow nearly anywhere along their distribution. Ganglioneuroma usually occurs in the abdomen or thorax, but it can also occur in the head and neck region, with the latter being it’s least common location. Actually, this is one of the rarest neck tumors. The first report of a ganglioneuroma located in the neck was made in 1899 by de Quervain [4] .The most frequent origin in the neck region is the cervical sympathetic chain, but it can also be located in the larynx, pharynx, and ganglion nodosum of the vagus nerve [5]. These types of tumors are more common at a young age, as 60% of patients with ganglioneuroma are ​