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Tumor Board-Appendiceal Adenocarcinoma
Presented By - Ranjita Pallavi
PGY-3
Department of Internal Medicine
Appendiceal Tumor Classification
2
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

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Appendiceal adenocarcinoma

  • 1. Tumor Board-Appendiceal Adenocarcinoma Presented By - Ranjita Pallavi PGY-3 Department of Internal Medicine
  • 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.