A 38-year-old female with systemic lupus erythematosus presented with abdom
inal pain, diarrhea and iron-deficient anemia. Computed tomogram showed a 2
x4 cm inhomogeneous lesion of the right adnexa. An unusual mass was identif
ied extending from the appendiceal orifice at colonoscopy, and an 8 cm tubu
lar appendix,apparently prolapsed into the cecum,was identified at celiotom
y. An appendectomy with cecectomy was performed. On cut section, mucin was
extruded from the lumen of the appendix. A mucinous neoplasm of the appendi
x with mucinous dissection to the serosal surface was reported at the time
of frozen section. No gross ovarian pathology or peritoneal implants were n
oted. Cystadenoma with associated mucocele formation was verified by perman
ent histology. Mucocele of the vermiform appendix is a rare condition assoc
iated with neoplastic transformation in approximately 75% of all cases. Ben
ign mucinous cystadenoma of the appendix should be differentiated from cyst
adenocarcinoma by frozen section at the time of celiotomy to ensure appropr
iate treatment. While systemic lupus erythematosus can lead to cutaneous mu
cinosis, an association with mucinous cystadenoma of the appendix has not b
een previously reported. Surveillance for metachronous colonic neoplasms is
warranted in patients diagnosed with a mucinous neoplasm of the appendix.