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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