Paracoccidioidomycosis is a systemic fungal disease caused by the dimorphic Paracoccidioides species endemic to South America. Infection classically presents with pulmonary, mucosal, or reticuloendothelial involvement, though other organs can be involved.Central nervous system involvement is rare, and almost universally reported within the endemic area for the fungus. We present a 60-year-old Brazilian male who complained of occipital headache, ataxia, dysmetria, and dysarthria for two months, diagnosed with neuroparacoccidioidomycosis in Houston, Texas. The patient had a cerebellar mass and a left pulmonary spiculated apical mass suspicious for a lung metastatic malignancy and a preliminary histological report consistent with invasive cryptococcosis. The patient's work and travel history were paramount in achieving the final diagnosis.
Background
Neuroblastic tumors arise from primitive sympathetic ganglion cells and are the most common extracranial solid tumor of childhood. Ganglioneuroma is the most well-differentiated, benign subtype and comprises less than 1% of all soft tissue tumors. One to 5% of these occur in the head and neck, including the parapharyngeal space. It is uncommon to find a physically detectable, neoplastic parapharyngeal space mass in a child.
Case report
A 7-year-old boy presented with a painless left neck mass. Imaging revealed a 2.6 × 2.7 × 6.1 cm left parapharyngeal space mass. Fine-needle aspiration suggested a ganglioneuroma. The patient developed compressive symptoms, notably pain, thus the decision was made to surgically resect. The tumor was excised transcervically, preserving the great vessels, phrenic and cranial nerves. Postoperatively, the patient developed a mild ipsilateral Horner’s syndrome, suggesting the mass to be a ganglioneuroma of the sympathetic chain.
Conclusions
Cervical ganglioneuromas typically present as slow-growing masses that cause compressive symptoms or are found incidentally. Resection is reserved for those with significant symptoms and is often complicated by Horner’s syndrome. In the review of literature, there are 23 reported cases of ganglioneuroma in the head/neck with a median age of 17 years. Eight of these occurred in the para/retropharyngeal spaces. Of these, five presented as an asymptomatic neck mass, two presented with compressive symptoms, and one was discovered incidentally. It is important to consider ganglioneuroma in the workup of pediatric neck masses, even in young children. Asymptomatic masses should be monitored for symptomatic transformation, and excision should be considered.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.