A Rare Case of Primitive Neuroectodermal Tumor of the Amygdala: A Case Report and Literature Review

Abstract

Background: The primitive neuroectodermal tumor (PNET) belongs to the family of malignant small round cell tumors with neuroepithelial differentiation.Isolated cases of PNET have been reported in visceral sites, such as the kidneys, uterus, ovaries, testicles, bladder, and pancreas.The occurrence of these tumors in the head and neck region is very rare.Purpose: We report the case of a 37-year-old patient with a PNET of the amygdala and a favorable outcome after induction chemotherapy followed by exclusive radiotherapy.Results: The patient exhibited a significant clinical and radiological response to the first-line chemotherapy regimen (VAC-IE) followed by radiotherapy.The treatment led to a complete clinical and radiological response.Currently, the patient is alive and in good health, with no signs of local recurrence or metastasis three years post-treatment.Conclusion: PNETs of the amygdala are extremely rare and aggressive.A combined therapeutic approach involving surgery, chemotherapy, and radiotherapy is essential for effective treatment.This case demonstrates that aggressive treatment can lead to favorable outcomes even in rare tumor locations.