1565 Goblet Cell Carcinoid: An Unusual Presentation of Acute Abdominal Pain

Abstract

INTRODUCTION: Goblet Cell Carcinoid (GCC) is a rare neoplasm comprising 0.3-0.9% of appendectomy specimens and less than 14% of all malignant masses of the appendix. While abdominal pain is a common presenting complaint of GCC, rarely does it manifest as an acute perforated appendicitis, as depicted in the case below. CASE DESCRIPTION/METHODS: A 66 year old male with a medical history of hypertension, heart failure, endocarditis, chronic alcoholic pancreatitis presented with RLQ pain 3 days prior with associated fevers, chills, nausea, vomiting and maroon colored stools. His vitals were stable with exam notable for diffuse lower abdominal tenderness and no rebound tenderness. Labs demonstrated a mild leukocytosis of 11.7 and negative blood cultures. CT abdomen and pelvis revealed a small appendiceal abscess and acute appendicitis for which he was treated with intravenous fluids and antibiotics, then was discharged on day 3 with oral antibiotics. 7 days later, he was readmitted with intractable nausea and vomiting. Repeat CT revealed mild improvement in acute appendicitis with labs notable for a leukocytosis of 14 for which he was restarted on antibiotics. Six days later, he underwent a laparoscopic appendectomy as repeat CT was concerning for persistent appendicitis with necrosis (Figure 1). Intra-operatively, the appendix was found to be ruptured. Pathology of appendiceal specimen revealed GCC (Figure 2). His hospital course was also complicated by septic shock and respiratory failure requiring intubation, and acute tubular necrosis requiring hemodialysis. The patient eventually improved and underwent a staging right hemicolectomy which identified lymphovascular invasion and node positivity (Figure 3). DISCUSSION: GCC, a rare appendiceal malignancy of both neuroendocrine and glandular histological features, accounts for less than 5% of all primary tumors of the appendix. Presentation varies from no symptoms to overt gastrointestinal bleeding, with acute appendicitis as the most common initial manifestation. While GCC is of a more aggressive phenotype compared to its benign carcinoid tumor counterpart, prognosis is generally favorable. However in 15-30% of cases, metastasis to the ovaries, pelvis, abdominal cavity, ribs, vertebra and lymph nodes has been observed. Surgery is typically the treatment of choice with tumors <2 cm confined to the appendix requiring only an appendectomy. For larger tumors with nodal involvement, right hemicolectomy within 3 months after appendectomy followed by chemotherapy is warranted.