Severe gastrointestinal endometriosis and low-grade appendiceal mucinous neoplasm: Is there a link?

Abstract

Significance:
Low-grade appendiceal mucinous neoplasms (LAMN) are welldifferentiated tumors that can cause dilation of the appendix leading to rupture, or can progress outside the appendix in a malignant process. These are rare conditions, seen only in 0.2% of appendectomy specimens and are often incidentally detected in asymptomatic patients. Endometriosis of the gastrointestinal tract, largely of the small intestine and mesentery, may be an associated condition. Combination of both conditions in young females is remarkably rare.

Case Presentation:
A 32-year-old woman presented with acute abdominal pain and vomiting with tenderness over the right lower quadrant. She had no fever, weight loss or altered bowel habits. CEA was normal while CA125 was significantly elevated.

Management:
Imaging studies revealed an ileocecal mass causing partial small intestinal obstruction and a dilated appendix. She underwent right hemicolectomy and appendectomy. Pathology revealed endometriosis of the onemtum, ileum, appendix, ascending and transverse colon. LAMN with extension to the mesoappendix was also reported.

Recommendation:
Endometriosis of the gastrointestinal tract may cause worsening gut obstruction and appendiceal neoplasms. Diagnosis and resection must be done early due to the risk of perforation as well as underlying malignancy. Appendiceal LAMN should be considered a differential diagnosis in cases of severe abdominal pain in a young woman with an appendiceal mass on CT scan.