St. Luke's University Health Network Bethlehem, PA, United States
Award: Presidential Poster Award
Brittney Shupp, DO1, Nishit Patel, MD2, Anthony Cianciolo, DO3, Hammad Liaquat, MD3, Lisa Stoll, MD4, Ayaz Matin, MD4, Chatargy Kaza, MD3 1St. Luke's University Health Network, Bethlehem, PA; 2St. Luke's University Hospital, Easton, PA; 3St. Luke's University Health Network, Fountain Hill, PA; 4St. Luke's University Hospital, Bethlehem, PA
Introduction: Neuroendocrine esophageal carcinomas are rare and account for 1.26% of esophageal malignancies. They present similar to other forms of esophageal cancer with complaints of dysphagia and weight loss and uncommonly secrete hormones. We present a case of undifferentiated neuroendocrine esophageal carcinoma with severe hypercalcemia.
Case Description/Methods: A 54-year-old male with Barrett’s esophagus with low grade dysplasia presented with dysphagia and weight loss. Outpatient endoscopy was performed and showed a semi circumferential, friable mass in the distal esophagus (Image A, B). Biopsies were taken and the patient was sent for CT imaging revealing diffuse thickening of the esophagus with hepatic masses and lung nodules concerning for metastasis (Image C, D). Shortly after, the patient presented to the hospital with failure to thrive. Vital signs were stable and initial labs demonstrated a WBC of 14.92 thousand/uL, and hemoglobin of 13.3 g/dL. AST/AST 76/68 U/L and alkaline phosphatase was 1,181 U/L. The patient’s albumin was 2.8 g/dL with a corrected calcium of 19.36 mg/dL that trended up to 22.10 mg/dL. Additional lab work demonstrated a PTH of 619 pg/mL but undetectable PTH-related protein. Urine calcium and vitamin D studies were normal. At that time, pathology results were available and revealed poorly differentiated carcinoma with neuroendocrine features (Image E). Liver biopsy was also performed and consistent with undifferentiated neuroendocrine carcinoma (Image F). Sestamibi scan showed a small subtle parathyroid adenoma, but hypercalcemia was resistant to sensipar. The hypercalcemia was treated aggressively with intravenous fluids, calcitonin, sensipar, and bisphosphonates with transient improvement in calcium levels. Following discharge and initiation of chemotherapy, the patient had multiple readmissions for resistant hypercalcemia with wide-spread metastasis. Given his poor long-term prognosis, the patient elected hospice and passed away 4 months after initial diagnosis.
Discussion: Serum calcium greater than 11.5 mg/dL is often due to malignancy secondary to secretion of PTH-related protein as opposed to PTH. Not only is neuroendocrine esophageal carcinoma not associated with hormone production, there are only a few cases in which ectopic neuroendocrine tumors have been found to secrete intact PTH mimicking primary hyperparathyroidism. To our knowledge, this is the first documentation of severe hypercalcemia due to production of PTH by a neuroendocrine esophageal carcinoma.
Figure: Upper endoscopy showing a semi-circumferential, friable, irregular mass in the distal esophagus (A,B). CT imaging showing extensive and diffuse thickening of the esophagus (see arrows) concerning for malignancy (C) along with multiple lung nodules and innumerable large hepatic masses (see arrows) concerning for metastasis (D). Esophageal biopsy demonstrating benign squamous mucosa with underlying sheets and islands of poorly differentiated carcinoma with neuroendocrine features (20X magnification) (E). Liver pathology with nests of tumor showing high nuclear-to-cytoplasmic ratio, prominent nucleoli (40x magnification). No keratinization or gland formation is present (F).
Disclosures: Brittney Shupp indicated no relevant financial relationships. Nishit Patel indicated no relevant financial relationships. Anthony Cianciolo indicated no relevant financial relationships. Hammad Liaquat indicated no relevant financial relationships. Lisa Stoll indicated no relevant financial relationships. Ayaz Matin indicated no relevant financial relationships. Chatargy Kaza indicated no relevant financial relationships.
Brittney Shupp, DO1, Nishit Patel, MD2, Anthony Cianciolo, DO3, Hammad Liaquat, MD3, Lisa Stoll, MD4, Ayaz Matin, MD4, Chatargy Kaza, MD3. P2433 - A Rare Case of Undifferentiated Neuroendocrine Esophageal Carcinoma With Severe Hypercalcemia, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.