Novel use of a Weerda laryngoscope for transoral excision of a cervical ganglioneuroma: a case report
© Yokoi et al; licensee BioMed Central Ltd. 2012
Received: 30 August 2011
Accepted: 26 March 2012
Published: 26 March 2012
A ganglioneuroma is a benign neoplasm arising from neural crest cells of the sympathetic nerve fibers and is most commonly seen in the posterior mediastinum or retroperitoneum. Although very uncommon, ganglioneuromas must be included in the differential diagnosis of neck masses. In young adult women, neck incisions made for excision of these benign tumors should be avoided whenever possible.
We herein describe the case of a 19-year-old Japanese woman with a ganglioneuroma. The tumor was found in the parapharyngeal space, an unusual location. A fine-needle aspiration biopsy was performed but was considered inadequate to make a definitive diagnosis, so the asymptomatic lesion was surgically excised using a Weerda laryngoscope. The lesion measured 4 × 3 cm in size and was encapsulated. A pathological analysis showed the presence of two distinct cell types, ganglion cells and Schwann cells, embedded in a loose myxoid stroma. The final diagnosis was a ganglioneuroma.
A complete excision was made possible by using a transoral approach with a novel use of the Weerda laryngoscope. Although its applicability to specific cases depends on the location, size and nature of the tumor, we believe that the Weerda laryngoscope will continue to be useful for performing transoral surgery for cervical tumors.
Ganglioneuroma is the most differentiated benign counterpart of neuroblastoma and originates similarly from neural crest cells that normally migrate into the adrenal medulla and sympathetic ganglia [1, 2]. Ganglioneuroma can be found anywhere along the sympathetic chain, but is most commonly located in the posterior mediastinum and retroperitoneum [3, 4]. Clinically, the signs and symptoms of cervical ganglioneuromas are usually related to the mass effect and nerve dysfunction, but these tumors often present as swelling with no specific symptomatology , as in the present case.
We herein describe a case of ganglioneuroma that was found in the parapharyngeal space, an unusual location [3, 5]. Complete excision was made possible by using a transoral approach with a novel use of the Weerda laryngoscope. We also review the pertinent recent literature and discuss our findings.
After surgery, the patient exhibited left palpebral ptosis and anisocoria with ipsilateral mydriasis typical of Horner's syndrome. These symptoms resolved completely within three months.
Ultrasonography of the abdomen was performed to exclude any visceral involvement. The patient's postoperative course was satisfactory, and she was discharged with no difficulties. No local recurrence or distant metastases have been observed during the five years since her surgery.
Prognostic evaluation of neuroblastic tumorsa
International Neuroblastoma Pathology Classification
< 0.5 years
Poorly differentiating or differentiating and low or intermediate MKI tumor
1.5 to 5 years
Differentiating and low MKI tumor
< 1.5 years
High MKI tumor
1.5 to 5 years
Undifferentiated or poorly differentiated tumor
Intermediate or high MKI tumor
≥ 5 years
Stroma-rich intermixed (favorable)
Composite Schwannian stroma-rich/stroma-dominant and stroma-poor
Ganglioneuromas are usually found in the posterior mediastinum or in the retroperitoneum [3, 4]. A report in which tumors were classified according to their anatomical distributions showed that only one of 88 patients had a tumor in the parapharyngeal region. The most common sites were the mediastinum (34 cases) and the retroperitoneum (27 cases) .
Although very uncommon, ganglioneuromas must be included in the differential diagnosis of neck masses, along with infectious cervical adenitis, branchial cleft cyst and some other malignancies, such as other variants of neuroblastic tumors, sarcoma and malignant lymphoma . Surgical excision is the treatment of choice in these cases, both to confirm the diagnosis and to prevent any further tumor growth and consequent compression of the adjacent structures.
Numerous approaches for resection of parapharyngeal space neoplasms have been described, including the transcervical approach, the transparotid approach, the transcervical-transpharyngeal approach, the infratemporal fossa approach and combinations of the these [8, 9]. The location, size and pathological type determine the choice of surgical approach . Transoral resection of a superomedial parapharyngeal benign neoplasm, with decreased morbidity compared with that of traditional approaches, has also been reported . In contrast to external approaches, the transoral approach does not require dissection in proximity to facial nerve branches; furthermore, the potential for postoperative salivary fistula present with transparotid techniques is avoided. In addition to these advantages, neck wounds can be avoided and patients can resume oral intake after surgery, making short hospitalizations possible . However, before surgery, it is important to communicate with the patient about the relative advantages and disadvantages of external and transoral approaches, as the latter allow less exposure and carry risks of hemorrhage, damage to cranial nerves (including Horner's syndrome and first-bite syndrome) and tumor spillage.
In our present case of a 19-year-old woman, a tumor was observed in the neck and was found to be prominent on the right side of the oropharynx when her mouth was open. To avoid performing an excision through an incision if possible, we attempted an excision using a transoral approach with a Weerda laryngoscope.
The Weerda laryngoscope has recently been reported to be useful for the removal of early esophageal lesions  and embedded esophageal foreign bodies . In this study, we report the first case of a patient from whom a tumor of the parapharyngeal space was excised using a transoral approach with a Weerda laryngoscope.
Although very uncommon, ganglioneuromas must be included in the differential diagnosis of neck masses. Although its applicability to individual cases may depend on the location, size and nature of the tumor, we believe that the Weerda laryngoscope will continue to be useful for transoral surgery for cervical tumors.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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