P0348 - A Rare Case of Esophageal Neuroendocrine Carcinomas

Dong Joo Seo, MD, Praneet Srisailam, MD, Navya Kirla, MD
Roger Williams Medical Center, Providence, RI

Introduction: Esophageal neuroendocrine carcinomas(NECs) are rare lesions with an indolent behavior and neuroendocrine differentiation. NETs account for about 0.5% of all newly diagnosed malignancies. The incidence, which is on the rise, possibly due to improved awareness, is approximately 5.86/100,000 per year with a female preponderance of around 2.5:1. Poor prognosis factors are lesion ≥2 cm; deep submucosa invasion or beyond (at least 24% are metastatic); Ki-67 ≥3%; vascular invasion; low degree of structural differentiation; presence of atypia and/or necrosis.

Case Description/Methods: Patient is a 62 year old female, non-smoker, who presented with neck swelling associated with dysphagia to solids for few months. Antibiotics did not relieve her symptoms and they gradually worsened. A CT scan of chest showed a 2 cm nodule on the right lobe of thyroid. Patient was referred to endocrinologist for fine needle biopsy. Biopsy was unremarkable. Patient was then referred to gastroenterologist after barium swallow demonstrating the narrowing in the proximal esophagus. Upper endoscopy revealed a medium sized partially obstructing, circumferential, submucosal mass with ulceration in the proximal esophagus. Biopsy was consistent with intermediate to high grade neuroendocrine tumor with TTF-1, chromogranin negative and CD56, synaptophysin positive, with a proliferative ratio of 15-20%. CT imaging showed a 4 x 1 x 2 cm intraluminal mass, not associated with metastasis or adenopathy. Endoscopic ultrasound showed the lesion, only extending into submucosal area. Despite 4 cycles of chemotherapy with carboplatin/etoposide concurrent with Radiotherapy, patient still had a smaller but persistent NECs.

Discussion: NECs are rare cancer with aggressive progression, poor prognosis, and resistance to surgical and medical treatment. Most common presenting symptoms are dysphagia, weight loss and anorexia. Often, esophageal NECs can be mixed with squamous carcinoma and/or esophageal adenocarcinoma (mixed type). Typical endoscopic finding of pure NECs is ulcerated tumor upon elevated lesion while mixed type is characterized nodular or polypoid lesion with glistening overlying surface. The median survival of pure NECs and Mixed type is 3.53 year and 7 years, respectively. With multiple biopsies, pathologic distinction between pure NECs and mixed type is critical at the time of the diagnosis.


Figure: circumferential, ulcerated, submucosal mass in the proximal esophagus.


Disclosures:
Dong Joo Seo indicated no relevant financial relationships.
Praneet Srisailam indicated no relevant financial relationships.
Navya Kirla indicated no relevant financial relationships.


Dong Joo Seo, MD, Praneet Srisailam, MD, Navya Kirla, MD. P0348 - A Rare Case of Esophageal Neuroendocrine Carcinomas, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.