# A Rare Presentation of a Neuroendocrine Tumor: A Case Report

## TL;DR

This case report describes a rare and aggressive esophageal neuroendocrine tumor that presented with unusual symptoms and led to rapid metastasis.

## Contribution

The novelty lies in highlighting an atypical clinical presentation of a rare esophageal neuroendocrine tumor with paraneoplastic syndrome and rapid progression.

## Key findings

- The patient presented with weakness, hypophonia, and dysphonia, with no prior symptoms of esophageal malignancy.
- The tumor was diagnosed at an advanced stage with liver metastasis and hypercalcemia, leading to a poor prognosis.
- The case emphasizes the importance of early detection and vigilance for rare tumors with non-specific symptoms.

## Abstract

Neuroendocrine tumors (NETs) are slow-growing, rare malignancies that originate from neuroendocrine cells and may be classified as functional, secreting hormones that produce clinical symptoms, or non-functional, often remaining asymptomatic until advanced stages. Primary gastrointestinal NETs in the esophagus (E-NETs) are rare, accounting for only 0.4%-2% of esophageal malignancies. Due to their rarity, often subtle early symptoms, and being mostly non-functional tumors, they are frequently diagnosed at an advanced stage with metastasis. This case report describes a patient who presented to the emergency department (ED) with a three-week history of weakness, hypophonia, and dysphonia, two months after carpal tunnel surgery. The patient had a past medical history (PMH) significant for hypertension and hypothyroidism and had no recent symptoms or medical concerns upon presenting for her preoperative examination for carpal tunnel surgery a few months prior. Before this preoperative appointment, she had an established primary care physician (PCP); however, she never had regular follow-ups or consistent monitoring. The patient’s initial workup in the ED revealed hypercalcemia and elevated liver enzyme levels. In addition to these abnormal lab results, a subsequent right upper quadrant ultrasound showed multiple lesions in the liver, indicative of metastasis. The next day, the patient had an abdominal CT scan performed, which revealed wall thickening in the distal esophagus, suggestive of neoplasm or malignancy. The tumor was thought to have originated at the gastroesophageal junction and metastasized to the liver. The patient had a poor prognosis and passed away from the metastatic tumor within two weeks of diagnosis. This case underscores the aggressive nature and atypical presentations of E-NETs, including rare paraneoplastic syndromes, such as hypercalcemia. It also underscores that serious malignancies can develop in patients without traditional risk factors. While standardized guidelines for E-NET follow-up and management remain limited, this case reinforces the importance of maintaining a high index of suspicion for atypical symptoms and ensuring adherence to routine preventive care, which may facilitate earlier detection of rare but aggressive tumors.