ABSTRACT
@#Primary malignant melanoma of the esophagus (PMME) is an exceptionally rare condition, representing a mere 0.1 to 0.2% of esophageal cancers, and accounting for just 0.1 to 0.5% of all melanomas. This case involves a 39 -year-old Filipino male who sought medical attention after an episode of choking. Subsequently, endoscopy with biopsy revealed a mass in the distal third of the esophagus, ultimately diagnosed as PMME based on histopathology and immunohistochemistry. FDG-PET/CT scan revealed a hypermetabolic distal esophageal mass and a confluent upper paratracheal lymphadenopathy. He was initially treated with Pembrolizumab, Nivolumab, and Ipilimumab immunotherapy. However, post-treatment FDG PET/CT scans unveiled metabolic progression of the esophageal mass with new hypermetabolic cervical lymph nodes, necessitating a shift to carboplatin and paclitaxel chemotherapy. After two cycles, there was a notable metabolic regression of the mass and paratracheal node with metabolic resolution of the cervical lymph node. An additional 2 cycles of chemotherapy were given, aimed to further reduce the size of the tumor, however, a succeeding follow-up study revealed metabolic progression of the mass. Surgical resection of both the esophageal mass and paratracheal nodes became imperative. The aggressive characteristics, metastasis at early diagnosis, and lack of effective treatment have contributed to the poor prognosis of PMME. Total esophagectomy is the preferred method of treatment. Chemotherapy and immunotherapy may be used in advanced diseases but with variable efficacy. The utilization of FDG PET/CT scans plays a crucial role in both the initial staging and the ongoing assessment of treatment response in patients diagnosed with PMME. This advanced imaging modality offers valuable insights into the extent of the disease and aids clinicians in evaluating the effectiveness of the chosen therapeutic interventions. Given the rarity and challenges associated with PMME, a multidisciplinary approach integrating surgical, medical, and imaging strategies is essential for comprehensive patient care.