Tumor Board-Appendiceal Adenocarcinoma

Presented By - Ranjita PallaviPGY-3

Department of Internal Medicine

2

Appendiceal Tumor Classification

Appendicular Adenocarcinoma

• Majority of patients with adenocarcinomas present with acute appendicitis.

• Other features include ascites, abdominal mass, or generalized abdominal pain. In less than 20 percent of cases, the cancer is found incidentally at surgery for other reasons.

• Intestinal-type tumors typically manifest as a focal mass without mucocele formation.

• Although they have a poorer prognosis, this is the case only if the disease is locoregionally advanced at presentation.

• The mucinous type spreads more often throughout the peritoneal cavity.

UMP

• Mucinous tumor of uncertain malignant potential (UMP) is used for neoplasms where the histological features do not allow distinction between a lesion that is benign from the one that has the potential to cause metastases.

• There is loss of normal complement of lymphoid tissue in the wall adjacent to the neoplastic epithelium accompanied by fibrosis of submucosa and muscularis propria.

• Appendix may be transformed into a cystic structure composed of a thin fibrous wall lined by neoplastic mucinous epithelium.

• Calcification may occur in the fibrous wall. • Because of a different therapeutic management, confirming the diagnosis

of mucinous tumor of UMP is important.• Right hemicolectomy should be considered for UMP considering the risk

of 10% of residual disease and metastases.

Appendiceal adenocarcinoma

Prognosis

TNM Staging

Long Term Outcomes

• 36 patients were studied.• 88% presented with acute

appendicitis.• 50% underwent curative resection• Overall 5 year survival 46% • 5 year survival after curative

resection 61% vs 32% for palliative surgery.

• Prognostic variables: Histologic type: colonic vs mucinous, T stage (T1,2 vs T3,4) and tumor grade.

• T2 or greater should be treated with

Hemicolectomy.• T1 would benefit from

hemicolectomy.

Treatment – Appendiceal Adenocarcinoma

Recommendations:  • Simple appendectomy for adenocarcinomas that are confined to the

mucosa or well-differentiated lesions that invade no deeper than the submucosa.

• Hemicolectomy for more deeply invasive tumors.• Routine oophorectomy has been proposed at the time of colectomy

because the ovaries are a common organ for metastases.• Resection of ovaries that are involved with metastatic spread is clearly

beneficial. • However, no series has shown an improvement in survival with

prophylactic oophorectomy, and this approach is not recommended.• Role of adjuvant chemotherapy/RT is unclear.

Adjuvant Chemotherapy

Intraperitoneal Hyperthermic Chemotherapy- IPHC

• Ideal candidates: Asymptomatic patients with small volume peritoneal carcinomatosis  who are likely to be successfully cytoreduced (leaving behind deposits <2.5 mm) with surgical debulking

• Rapid recurrence of the peritoneal surface disease despite treatment, combined with progression of nodal or extraperitoneal systemic disease, interferes with long-term benefit.

• CT scans may not distinguish between diffuse peritoneal adenomucinosis (DPAM) from mucinous peritoneal carcinomatosis, features like the presence of tumor implants >5 cm on the jejunum, proximal ileum, or adjacent mesentery is more consistent with mucinous adenocarcinoma with secondary peritoneal carcinomatosis than DPAM.

Cytoreductive Surgery

• Presence of segmental obstruction of the small bowel raises suspicion for peritoneal adenocarcinomatosis.

• It predicts a less favorable outcome from aggressive cytoreduction and intraperitoneal heated chemotherapy.

• Total abdominal colectomy, pelvic peritonectomy, and end-ileostomy is a technically feasible procedure and is advocated for the palliation of patients with peritoneal carcinomatosis of appendiceal origin.

Metastatic Disease

Thank You