Background: Neuroendocrine tumors are a rare and heterogenous group of malignancies that arise from the neural crest cells with neuroendocrine differentiation. They account for only 0.5% of all malignancies with an incidence of 5-6 new cases per 100,000 inhabitants. While they most commonly arise from the gastro-intestinal tract and respiratory system, NETs likewise infrequently arise from the head and neck.
Aims: To present a rare case of a maxillary mass revealing to be a neuroendocrine tumor by histopathology. Methods/design: Case report.
Results: A case of a 64 year old male with a 5 month history of a slowly enlarging mass on the medial aspect of the left cheek bone. This was accompanied by facial erythema and numbness of the affected area. Due to the progressive enlargement of the mass, there was eventual extension to the orbital cavity resulting to compromised vision. As the metastatic work up was unremarkable, the patient underwent a subtotal maxillectomy with orbital exenteration. Histopathology revealed a malignant round cell tumor, 3.3cm in widest diameter, involving the posterior maxilla, lateral nasal mucosa, the orbital floor, lateral pterygoid and ethmoid mucosae, and the temporalis muscle. Immunohistochemistry studies reveal strong and diffusely positive for synaptophysin, chromogranin, and vimentin, while negative for CK and LCA, compatible with a neuroendocrine carcinoma. The patient underwent 6 cycles of adjuvant chemotherapy with cisplatin and etoposide. Evaluation scans were clear for residual or recurrent tumor, and the patient remains to be asymptomatic almost one year post treatment.
Summary/Conclusion: Neuroendocrine tumors rarely involve the head and neck but are usually aggressive with high rates of recurrence. Best outcomes are achieved using a multimodality approach using surgery, radiation, and a platinum-based chemotherapy. The importance for close surveillance should likewise be implemented due to frequent recurrence and metastases