3. 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.
4. 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.
8. 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.
9. 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.
11. 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.
12. 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.
