A 46-year-old Caucasian woman with no significant past medical or family history presented to her primary care provider with a one-year history of sore throat and dysphagia to solid foods. She reported a remote smoking history in her teens and rare alcohol consumption. Workup included a barium swallow study with a persistent filling defect in the upper esophagus measuring 5
cm × 1.8
cm originating from the posterior right wall of the esophagus. Differential diagnosis included both benign and malignant lesions of the upper esophagus. Upper endoscopy with endoscopic ultrasound revealed an ulcerated, friable mass extending from 15 to 19
cm from the incisors with evidence of invasion into the muscularis propria and suspicion for regional node involvement (Figures and ). Biopsies from the upper endoscopy were positive for moderately differentiated invasive adenocarcinoma (Figures and ).
(a) Upper endoscopy highlights the close proximity of the tumor to the upper esophageal sphincter (UES). (b) Endoscopic ultrasound shows invasion of the tumor into the muscularis propria and suspicion for regional node involvement.
Figure 2 (a) H&E stain (×40 magnification) shows moderately differentiated adenocarcinoma in the deep mucosa beneath intact squamous epithelium. (b) H&E stain (×100 magnification) shows cribriform glands and micropapillae infiltrating (more ...)
The patient was staged as T3N1M0 after a PET/CT of the neck demonstrated a hypermetabolic esophageal mass with adjacent right paratracheal and superior mediastinal lymphadenopathy. CT scan of the chest, abdomen, and pelvis were negative for distant metastases. Six weeks of definitive/neoadjuvant chemoradiotherapy were delivered according to the Ilsen regimen (cisplatin 30
and irinotecan 65
on weeks 1, 2, 4, and 5 of radiation). Radiotherapy consisted of a total of 64.8
Gy to the primary esophageal tumor and adjacent nodes plus 39.6
Gy to superior mediastinal nodes. Follow-up PET/CT 14 weeks after completion of definitive chemoradiation therapy revealed continued hypermetabolic activity in the proximal esophagus and repeat upper endoscopy revealed residual tumor. Otolaryngology performed a cervical esophagectomy, lymph node dissection, and left radial forearm microvascular free tissue transfer reconstruction of the cervical esophagus. The pathologic specimen contained moderately differentiated adenocarcinoma of the esophagus undermining the squamous epithelium, with metastasis to two of six paratracheal lymph nodes, but no evidence of Barrett's esophagus or identifiable vestiges of heterotopic gastric mucosa.
Five months after surgery, she developed difficulty with an anastomotic stricture that was dilated endoscopically and determined to be a result of local tumor recurrence. Nine months after the initial surgery, salvage laryngopharyngectomy with a right radial forearm flap reconstruction was performed. The patient's course continued with a second locoregional recurrence five months after salvage surgery, necessitating further chemotherapy/limited field radiotherapy (first low-dose cisplatin, then capecitabine due to an urticarial rash, and 50.4
Gy). A third recurrence five months later in a right subclavicular node was treated with resection and concurrent capecitabine/limited field radiation. Most recently, she had evidence of multifocal recurrence nearly two years after the initial chemoradiation. She is currently participating in a phase II clinical trial with an oral Aurora kinase inhibitor and has stable disease based on radiographic imaging after six treatment cycles.