Appendiceal carcinoma: a diagnostic and therapeutic challenge

Appendiceal carcinoma is a very rare clinical entity, constituting 1% of all colorectal malignancies and 1% of all appendectomy specimens. Appendiceal malignancies often present atypically, thus creating diagnostic challenges. We present a patient with mucinous carcinoma of the appendix who presented with hematuria and abdominal pain. Similar case reports are extremely rare in the literature, while typical presentations of appendiceal carcinoma include abdominal pain, abdominal mass, early satiety, nausea, and iron-deficiency anemia. Initially, the diagnostic investigation in our patient was focused on urinary tract disorders, but ultimately resulted in finding a mucinous appendiceal carcinoma. The carcinoma had invaded the urinary bladder and was disseminated in the peritoneal cavity. Aggressive cytoreductive surgery is the most common therapeutic approach for disseminated tumors, often followed by intraperitoneal chemotherapy. However, treatment should be individualized based on patient-specific parameters, such as the presence of comorbidities, performance status, as well as the presence of metastatic disease. Our patient had optimal cytoreduction with subsequent systemic therapy with 5-fluorouracil, leucovorin, oxaliplatin, and bevacizumab, a monoclonal antibody directed against vascular endothelial growth factor. She completed her treatment regimen without complications and is currently being restaged. An integrative approach is required in the diagnostic investigation and management of appendiceal malignancies.