Evaluating the Presence of a Stage IV Low-Grade Well-Differentiated Neuroendocrine Tumor of the Ileocecum: A Case Report with Evaluation of Staging Protocol of Neuroendocrine Tumors and Treatment Options Based on Current Available Evidence

Abstract

Neuroendocrine tumors (NET) are rare neoplasms that can originate throughout the human body. An initial treatment option includes upfront surgical resection of the primary tumor (pT) if the tumor can be localized. Current systemic therapy options following resection of the pT or with evidence of metastatic disease include somatostatin analogs, evorlimus, peptide receptor radionuclide therapy, cytotoxic chemotherapy, and interferon alpha among other less common therapy options. We present a case of a patient with a NET that originated in the ileocecal region. The patient underwent upfront surgical resection with a right hemicolectomy due to the location of the tumor. The pT was notable for extensive invasion into the visceral peritoneum and metastasis to nearby lymph nodes. However, despite being diagnosed as a stage IV NET, the Ki67 index was less than 1%, categorizing it as a low-grade well-differentiated tumor. Following resection of the tumor, there was no evidence of metastasis to the liver on the follow-up magnetic resonance imaging and recurrent somatostatin receptor overexpressing neoplasm on the Gallium-68 DOTATE PET/CT scan. Due to the juxtaposition of the low grade of the tumor and the high staging, several different treatment options were discussed with the main distinction being whether to base these options off of the stage or the grade of the tumor in the case. Low-grade well-differentiated NET have a good prognosis. On the other hand, stage IV NET and tumors that have metastasized to nearby lymph nodes and organs have an increased likelihood to reoccur and worse outcomes. Recommendations for NET based on current evidence have a lack of clarity in terms of when to undergo observation versus systemic therapy.