Primary signet ring cell carcinoma of the appendix: A rare case report

Abstract

Primary adenocarcinoma of the appendix is a rare malignancythat constitutes < 0.5% of all gastrointestinalneoplasms. Moreover, primary signet ring cell carcinomaof the appendix is an exceedingly rare entity. In the present report, we describe a rare case of primary signet ring cell carcinoma of the appendix with ovarian metastasesand unresectable peritoneal dissemination occurring in a 45-year-old female patient. She was clinically misdiagnosed as torsion of ovarian cyst. She underwent appendicectomy and unilateral salpingo-oophorectomy.Histopathology revealed signet ring cell carcinoma and a right hemicolectomy was done. She then received palliative systemic chemotherapy with 12 cycles of oxaliplatin, 5-fluorouracil, and leucovorin (FOLFOX-4). The patient is doing well till today on follow up without progression of disease 10 mo after beginning chemotherapy.

Keywords: Appendix; Primary; Signet ring cell carcinoma; Ovarian metastasis

Core tip: Meticulous histopathological evaluation along with clinical correlation is strongly recommended in such unusual circumstances. The clinician and surgical pathologist should keep in mind this rare entity as a differential diagnosis.