Retropharyngeal Ganglioneuroma Presenting with Neck Stiffness: Report of a Case and Review of Literature

Abstract

Ganglioneuromas rarely occur in the retropharynx with only three cases reported in the current literature. The most common symptom associated with retropharyngeal ganglioneuromas is dysphagia. We report a retropharyngeal ganglioneuroma with an unusual clinical presentation of neck stiffness and pain. A 42-year-old woman presented with incapacitating neck pain, neck stiffness, right upper extremity weakness, as well as dysphagia. Neurological workup was normal. Imaging revealed a hyperdense, ill-defined, diffuse right retropharyngeal mass suggestive of a possible nerve sheath tumor with no communication with the cervical spine. Surgical removal was uneventful and associated with a postoperative Horner's syndrome. In follow-up, dysphagia and neck symptoms improved. Retropharyngeal ganglioneuromas can occur in a wide age range of patients. 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 intervention. Neck pain is an atypical symptom that needs to be worked up to rule out a communication with the spinal column prior to surgical removal. Patients must be counseled that atypical symptoms may not completely resolve with surgical treatment. KEYWORDS: Retropharyngeal, ganglioneuroma, neck pain, dysphagiaGanglioneuromas are benign tumors that originate from primordial neural crest cells which migrate from the mantle layer of the developing spinal cord to the sympathetic ganglia, adrenal medulla, and other sites. These tumors are well-circumscribed and consist of ganglion cells and Schwann cells with few neuroblasts. Ganglioneuroblastomas are malignant variations of these tumors that are characterized by decreased differentiation within ganglion cells in the presence of Schwann cells and are also associated with neuroblasts. Ganglioneuromas occur most commonly in children over 10 years of age.The most frequent sites of presentation include the posterior mediastinum and the retroperitoneum. In general, the neck is a rare site of presentation. 1 In a series of 88 patients with ganglioneuromas, only two occurred in the cervical region. 2 The retropharyngeal area of the neck is an extremely rare site of presentation with only three reported cases in the literature. [3][4][5][6][7] The most common symptom of presentation was dysphagia. We are reporting a retropharyngeal ganglioneuroma presenting with an unusual symptom complex that consisted primarily of neck stiffness, neck pain, and dysphagia.

A huge retropharyngeal bronchogenic cyst displacing the posterior wall of the hypopharynx: Case report and review of the literature

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A Malignant Transformation of a Spinal Epidural Mass from Ganglioneuroblastoma to Neuroblastoma

“…Ganglioneuroma is a slow growing, benign tumor, which can be rarely associated with neurofibromatosis type I and multiple tumor syndromes like multiple endocrine neoplasia type IIB 33 ). These tumors are fully differentiated tumors with mature ganglion cells and schwann cells 16 , 23 ). Spinal ganglioneuromas represent less than 10% of all ganglioneuromas, usually with the involvement of paraspinal region 28 ).…”

Multiple cervical ganglioneuroma: A case report and review of the literature

“…We reviewed cases of GN located in the head and neck region that were reported in the English-language literature during the past 10 years. A total of 16 papers and 26 cases were included (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). The epidemiology and clinical characteristics are summarized in Table I.…”