Primary Retropharyngeal Neuroblastoma

Abstract

Neuroblastoma is the most frequent extracerebral tumor in infants younger than 1 year. It is most often localized in the abdomen or thorax. A cervical localization is rare, and in the majority of cases, the tumor is located in the lateral region of the neck, lateral to the carotid artery. 1 We describe a newborn boy who presented with difficulty in breathing associated with a retropharyngeal neuroblastoma. A retropharyngeal localization is exceptional, and, to our knowledge, there have been no previous reports in which retropharyngeal neuroblastomas were differentiated from lateral cervical neuroblastomas. Our goal is to emphasize the surgical importance of differentiating retropharyngeal cervical neuroblastomas from laterocervical neuroblastomas and to present their clinical and therapeutic characteristics. REPORT OF A CASEA 6-week-old boy was hospitalized with a 1-week history of progressively worsening inspiratory dyspnea and stridor, without any associated feeding problems. There was no family history of neurocristopathy. The clinical examination and endoscopy with the patient under general anesthesia showed a swelling of the left side of the pharynx, covered with normal mucosa, forcing the larynx forward and descending down into the hypopharynx. The mobility of the larynx was normal. Cervical palpation revealed no abnormalities. A left Horner syndrome and a heterochromia iridis were noted. A lateral cervical radiograph showed a pharyngeal tumor forcing the larynx forward (Figure 1). A cervical computed tomographic (CT) scan revealed a welldefined heterogeneous mass, with a few calcifications, that was in contact with the internal carotid artery and the prevertebral area and was reducing the airway (Figure 2). Blood tests were negative for chorionic gonadotropin ␤, carcinoembryonic antigen, ␣-fetoprotein, neuronspecific enolase, and urinary catecholamines. At the age of 8 weeks, the patient was surgically treated because of increasing difficulty in breathing. A transoral approach was used, and a L-shaped incision in the pharyngeal mucosa was performed. The tumor was well defined. The vascular pedicles of the tumor were electrocoagulated. The excision was macroscopically complete. The pharyngeal mucosa was sutured. The patient underwent extubation on awakening, and no dyspnea was observed.Microscopically, the tumor consisted of small, round and fusiform cells, which were only slightly larger than lymphocytes (Figure 3). They were arranged in irregular sheets separated by fibrovascular strands. In well-preserved areas, the cells were larger, with fewer chromatic nuclei, and arranged in round aggregates. There were no true rosettes. The background was loosely fibrillar. On immunohistochemical studies, the tumor cells expressed neuron-specific enolase and synaptophysin. The histologic diagnosis was neuroblastoma.For 8 days, the patient was fed with a nasogastric tube. A control fiberoscopy at day 8 showed complete healing. Oral feeding was resumed without any problems. Iobenguane sulfate I 123 scintigraphy perfor...

Retropharyngeal Neuroblastoma in an Infant

“…1,3,7-10 However, cases among infants more than 1 month of age are limited and we have identified only 3 documented observations in the literature (Table 1). 1,4,5 Cervical NBLs have a good prognosis in children below 1 year of age. 1 Resectable localized tumors can be cured by complete excision, but caution is required because of the risk of complications such as damage to the sympathetic cervical nerves, cranial nerves IX and X, and vascular structures.…”

“…1 Resectable localized tumors can be cured by complete excision, but caution is required because of the risk of complications such as damage to the sympathetic cervical nerves, cranial nerves IX and X, and vascular structures. 2,4,5 For unresectable tumors, especially for emergency cases with airway obstruction, prompt chemotherapy should be initiated after biopsy for accurate diagnosis. Concurrent steroid use might be beneficial to some extent for reduction of edema surrounding tumor.…”

“…2,3 Complete excision is often difficult because of localization near vital vasculature and, when surgery is possible, there is the risk of complications such as swallowing disturbances and Horner syndrome. 2,4,5 We report a 3-month-old boy with retropharyngeal NBL who was successfully treated with chemotherapy alone.…”