Multi-origin mucinous neoplasm: Should we prophylactically remove the appendix in the setting of mucinous ovarian tumors?

INTRODUCTION: Tumors of the ovary and appendix have been well documented in the setting of pseudomyxoma peritonei (PMP) with constant debate over tumor origin. Generally, these tumors are found to have a single primary origin, most commonly the appendix, with metastatic spread to the ovaries. CARE PRESENTATION: Here we present a 61-year-old female who underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH-BSO) for a primary mucinous ovarian carcinoma. She presented to our institution one year later with abdominal pain and a palpable right lower quadrant mass, which on histopathologic exam was found to be a primary low grade mucinous appendiceal neoplasm (LAMN), alluding to the potential of two separate primary disease processes. DISCUSSION/CONCLUSION: With two primary, non-synchronous lesions, a thorough literature review suggests that during the patient's initial TAH-BSO, she could have additionally undergone an appendectomy. In doing so, this would provide accurate, complete staging and determine if the two neoplasms were truly primary in origin or metastatic. In addition, new genetic markers are being discovered, such as the Special AT-rich sequence-binding protein 2 (SATB2) marker, which has been found to be positive in those with a LAMN and negative in those with a primary mucinous ovarian carcinoma. By acquiring appropriate and complete staging we can better diagnose and treat these neoplasms.