Multidisciplinary Treatment for Advanced Pancreatic Adenocarcinoma Arising from Esophageal Heterotopic Pancreas in a Young Adult: A Case Report.

INTRODUCTION

Heterotopic pancreas (HP) is a congenital anomaly characterized by pancreatic tissue located outside its normal anatomical site, without anatomical or vascular continuity with the pancreas proper. HP typically occurs in the stomach or duodenum, whereas esophageal HP is extremely rare. Malignant transformation is even rarer, with only a limited number of reported cases. We report a case of adenocarcinoma arising from esophageal HP successfully managed by multidisciplinary treatment.

CASE PRESENTATION: A 32-year-old woman presented with persistent epigastric pain accompanied by severe right-sided posterior thoracic pain that was markedly positional, preventing her from resting in the supine position. Upper gastrointestinal endoscopy revealed ectopic gastric mucosa with a papillary elevation in the lower thoracic esophagus (Lt), and endoscopic US (EUS) demonstrated a homogeneous hypoechoic submucosal mass. Contrast-enhanced CT and esophagography identified a well-defined 30-mm lesion arising from the right wall of the Lt, without distant metastasis or lymphadenopathy. Although EUS-guided fine-needle aspiration (EUS-FNA) and referral for a second opinion were recommended, the patient declined both because of intractable pain and requested immediate surgical management. Thoracoscopic tumor resection was therefore performed for diagnostic and therapeutic purposes. Histopathology confirmed pancreatic-type adenocarcinoma arising from Heinrich type I HP, with lymphatic invasion and a positive resection margin. Accordingly, 3 cycles of adjuvant chemotherapy with gemcitabine (1000 mg/m2) and nab-paclitaxel (125 mg/m2) were administered over 3 months. Follow-up CT and PET-CT showed no recurrence or metastasis, and robot-assisted subtotal esophagectomy was performed 7 months after the initial surgery. Postoperative pathology demonstrated fibrotic scar tissue without residual malignancy. The patient declined further adjuvant therapy and remains free of recurrence 12 months after the second surgery.

CONCLUSIONS

Esophageal HP carcinoma is exceedingly rare. Multidisciplinary treatment following pancreatic cancer protocols enabled curative resection and favorable outcomes. Given the malignant potential, meticulous preoperative evaluation and individualized therapeutic planning are essential.