Our patient was a 68-year-old woman with chronic obstructive pulmonary disease.
She had no history of gastroesophageal reflux disease or esophageal disorders but was found to have a progressive solitary pulmonary nodule (1×1.3cm).
A right lower wedge resection was performed in December 2011 and the University of Iowa pathology department identified the tumor as a LCNEC.
Approximately six months after her operation, our patient started having slowly progressing dysphagia for both solids and liquids.
A positron emission tomography (PET) scan performed in 2012, one year post surgery, was unremarkable for recurrence or metastasis.
Repeat computed tomography (CT) in June 2013 (18 months post surgery) did not show any recurrence (Figure 1).
Our patient had an esophagogastroduodenoscopy (EGD) and barium swallow that revealed no stricture of her esophagus but failure of primary and secondary peristaltic waves and reflux.
In July 2013, esophageal manometry found failure of the lower esophageal sphincter to relax, with a residual pressure of 32mmHg.
Our patient was treated with calcium channel blockers with partial relief.
Thus far, up to 18 months post resection, our patient had been able to maintain her weight and her basic laboratory parameters, including hemoglobin, albumin and cholesterol levels.
However, in January 2014, she reported worsening dysphagia and weight loss of 10lbs over a period of one month.
She was referred to our center for further treatment of her achalasia.
A repeat esophageal manometry showed an elevated lower esophageal sphincter pressure of 50mmHg as well non-propagative, non-peristaltic contractions throughout her esophageal musculature (Figure 2).
A repeat EGD with an intention to perform therapeutic pneumatic dilation showed a nearly circumferential ulcerated, fungating mass from approximately 36cm that extended to her gastroesophageal junction at 39cm.
Biopsies revealed a LCNEC with similar features to the primary tumor resected in 2011, and was concluded to be a metastasis.
Subsequently, a repeat CT scan revealed progressive diffuse metastatic disease.
Our patient underwent two courses of chemotherapy but died from the disease after six months.