Lindsay Tse, DO, Joshua Klein, DO, Maurizio Miglietta, DO. Palisades Medical Center
Goblet cell adenocarcinoid tumors are rare neoplasms that are almost exclusively located in the appendix. These neoplasms arise from multipotent stem cells in the intestinal crypts, contain neuroendocrine features, and produce mucin; making these tumors distinct from adenocarcinomas or carcinoid tumors of the appendix.
Goblet cell adenocarcinoids usually present as a typical acute appendicitis and therefore are most commonly diagnosed only after surgical removal and pathologic examination. Currently, controversy exists over whether a simple appendectomy is sufficient treatment or if a subsequent right hemicolectomy is necessary.
This case report describes a patient diagnosed with goblet cell adenocarcinoid of the appendix, and will discuss some of the current literature and guidelines for treating this neoplasm. A 48 year old male initially presented to the emergency department with a three day history of abdominal pain that was localized to the right lower quadrant.
The patient also reported nausea associated with multiple episodes of non-bilious emesis. Past medical history was significant for diabetes and hypertension, and no previous surgeries were reported. On admission the patient was febrile with a temperature of 101.8, a WBC of 16.6 and a CT scan that demonstrated a dilated appendix with periappendiceal inflammatory changes consistent with acute appendicitis.
The patient subsequently underwent an uncomplicated laparoscopic appendectomy and was discharged on post operative day 1. Pathology revealed goblet cell adenocarcinoid tumor of the appendix with transmural invasion involving the periappendiceal fat consistent with a pathologic T3 lesion. Three weeks later the patient returned to the operating room for a laparoscopic hand assisted right hemicolectomy. Final pathology demonstrated a specimen with negative margins and 12 negative lymph nodes.
This case illustrates the current controversy over appropriate operative intervention for goblet cell adenocarinoid tumors. Due to the rarity of these neoplasms, the majority of the existing literature is composed of small retrospective studies or case reports. Some consensus does exist regarding which patient should undergo right hemicolectomy based on lesion staging and other pathologic findings. Currently, long term survival benefits have not been demonstrated with the use of adjuvant chemotherapy or radiation.
Therefore, properly identifying patients who require and would benefit from right hemicolectomy remains a pivotal component in the management and treatment of patients with goblet cell adenocarcinoid of the appendix.
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