Appendiceal Cancer
• 1% of appendectomies contain cancer
– 2/3 carcinoid (50% all GI carcinoids are appendiceal)
• Tan-yellow mass, surrounding desmoplastic reaction
• < 2 cm no further treatment
• > 2 cm R hemicolectomy
– Remainder: mucinous cystadenocarcinoma,
adenocarcinoma, adenocarcinoid
– Adenocarcinoma associated with metastatic disease
50% of time (likely 2nd appendiceal rupture, tumor
spread)
Appendiceal mucocele
• Dilated, mucin-filled appendix
• 0.3% of all appendectomies
• 4 histologic categories:
1) Retention cyst = simple mucocele (normal
mucosa)
2) Mucosal hyperplasia
3) Mucinous cystadenoma (~ papillary adenoma
of colon)
4) Mucinous cystadenocarcinoma
Mucoceles: pathophysiology
• Obstruction of appendiceal lumen
dilation with mucin
– Obstruction 2nd hyperplasia or fecalith
• Presentation depends on nature/degree of
obstruction:
– Slow, chronic large mucocele formation
– Fecalith acute inflammation, with mucinous
content
Mucocele:
• Lead to progressive enlargement of the appendix.
• 4 histological types: retention cysts, mucosal hyperplasia, cystadenoma,
cystadenocarcinoma.
• Benign etiology: simple appendectomy.
• Pseudomyxoma peritonei:
*diffuse collections of gelatinous fluid are associated with mucinous
implants on peritoneal surfaces and omentum.
*caused by neoplastic mucous-secreting cells within the peritoneum with
the appendix being the site of origin for most cases.
*CT is the preferred imaging modality.
*surgical debulking is the mainstay of treatment and appendectomy
routinely performed. Hysterectomy and bilateral salpingio-oopheorectomy
is performed in women.
Mucoceles: natural history
• Asymptomatic (25%), vs. chronic RLQ
pain vs. acute appendicitis
• Mucocele can also lead to:
– Bowel obstruction 2nd intussusception cecum
– Torsion gangrenous appendix
– Rupture mucinous ascites, pseudomyxoma
peritonei
– Risk factor for colon cancer (synchronous,
metachronous)
Differential Diagnosis
• Duplication cyst
• Mesenteric/omental cyst
• Ovarian cyst
• Periappendiceal abscess
Mucoceles: treatment
• Hyperplasia, mucinous cystadenoma
treated by simple appendectomy
• Malignant mucocele:
– Preop/intraop suspicion R hemicolectomy,
or appendectomy and frozen section
– Post-op diagnosis return for definitive
procedure
Pseudomyxoma Peritonei
• Extremely rare; It is a rare form of cancer that is unexpectantly diagnosed in
2 of 10,000 laparotomies with an incidence rate of one case per million per
year
• More commen in female, Most common 6th decade of life
• Diffuse, intraperitoneal collection of gelatinous fluid with mucinous tumor
implants on peritoneal surfaces and omentum. . A slang term for the large
amounts of this thick mucous in the abdomen is "jelly belly".
• Strictly, etiology is secondry to grade I mucinous cystadenocarcinoma of the
appendix
• Unlike most cancers, PMP rarely spreadsthrough the lymphatic system or
throughthe bloodstream.
• Therefore PMP is characterized by mucin and scattered cancer cells in the
abdominal cavity but not with liver or other sites of metastasis.
• If left untreated, mucin will eventually build up to the point where it
compresses vital structures: the colon, the liver, kidneys, etc. can become
"squashed" by this mucous, and death can eventually result.
• In this circumstance the benign cells cause damage in much the same way
as cancerous tumors that produce mucous, so are considered in the medical
community to be a low-grade cancer.
• Ovarian, pancreatic cancer similar picture
Pathophysiology
• Mucocele rupture dissemination of mucin-
producing tumor cells throughout peritoneal
cavity
• Characteristic and predictable pattern of tumor
progression:
1) Gravity dependent collection of tumor
(pelvis, retrohepatic space, paracolic gutters, Treitz)
2) Resorption of peritoneal fluid accumulation of
tumor cells to distinct sites:
Deposit Sites 2nd Fluid Resorption
1) Between liver, R hemidiaphragm
-2nd lymphatics within undersurface of
hemidiaphragm
2) Greater, lesser omentum
-lymphatics draw fluid, attracting tumor
cells to their surface omental caking
Sugarbaker Protocol
• Radical debulking of tumor load:
– appendix, peritoneum, omentum;
– additional viscera as indicated
– Curative therapy = all nodules > 2.5 mm
• Intraoperative heated mitomycin
• Post-operative 5-FU
• Reports of 80% 10 yr survival
Rationale for Radical Surgery
1) Low aggressiveness of tumor; rare LN or
liver involvement
2) Peritoneal dissemination occurs early
3) Areas of spread are treatable by
peritonectomy/omentectomy
4) Redistribution phenomenon: small bowel
is largely spared (2nd motility?)
5) Regional chemotherapy can attack all
surfaces exposed to tumor
