Primitive neuroectodermal tumor of the maxillary sinus in an elderly male: A case report and literature review

Abstract

Primitive neuroectodermal tumor (PNET), which belongs to the Ewing's sarcoma (ES) family of tumors, is mainly seen in children and young adults. PNETs are extremely rare in the maxilla. Here, we report a case of PNET of the left maxillary sinus in an elderly male. Magnetic resonance imaging (MRI) revealed a slightly enhanced solid mass occupying the left maxillary sinus and infiltrating into the retroantral space. A partial maxillectomy was performed. Despite postoperative chemotherapy, follow-up computed tomography (CT) and MRI revealed a nodal metastasis in the submandibular space. Neck dissection was performed. However, the patient died 10 months after the second surgery because of distant metastasis to the liver. MRI and CT were particularly useful in detecting the extent of the tumor, recurrence, and metastasis. Further, a literature review of the previously reported PNET cases of the maxilla was carried out. In this paper, we also discuss the current approach for the diagnosis and management of these tumors.

Imaging Features of Primitive Neuroectodermal Tumor in the Maxilla: A Case Report and Literature Review

“…In general, the gender distribution of patients with PNET varies among different studies. 1,3,6 We found that women slightly predominate, as distinct from the previously maxillary PNET cases reported by Shah et al in 2014 (male/female ratio of 9:10) ( Table 1). 1 As in our case, there was either as a soft tissue mass or swelling in the most of the reported cases of PNETs of the maxilla in Table 1.…”

“…1,3,6 We found that women slightly predominate, as distinct from the previously maxillary PNET cases reported by Shah et al in 2014 (male/female ratio of 9:10) ( Table 1). 1 As in our case, there was either as a soft tissue mass or swelling in the most of the reported cases of PNETs of the maxilla in Table 1. The patient was admitted to our clinic with progressively increasing, painful swelling on the right side of the face that had been ongoing for 2 months.…”

“…1,6 Eighteen cases of PNETs of the maxilla have been reported in the literature according to the MEDLINE/PubMed database. The clinical presentations and imaging features of these cases are presented in Table 1.…”

“…Therefore MRI can be considered as the imaging modality more effective than CT for preand post-surgical assessments of PNET. 1 However, the combination of PET or PET/CT with conventional imaging has demonstrated sensitivity and specificity > 90% in the preoperative staging of Ewing sarcoma family of tumors. 23 Cone beam computed tomography in head and neck cancer can use as imaging modality, especially as alternative to CT because it needed the lesser radiation dose, the short scan time and avoids soft tissue distortions caused by gravity and showed reliable negative predictive value in application to cortical bone invasion, high resolution.…”

“…PNET has a poor prognosis and is commonly seen in children and young adults. 1 Because this tumor presents aggressive biological behavior and tends toward loco-regional and systemic metastases, it is a tumor with an extremely poor survival rate. 2 The incidence of head and neck PNET Imaging Features of Primitive Neuroectodermal Tumor in the Maxilla: A Case Report and Literature Review A AB BS S T TR RA AC CT T Primitive neuroectodermal tumor (PNET) is a term used to describe a group of highly malignant neoplasms composed of undifferentiated round cell tumors of neuroectodermal origin with variable cell differentiation.…”