Appendiceal neoplasms;

management
 Outline

• Introduction
• Neuroendocrine tumors
• Epithelial tumors
• Lymphoma
 Introduction
• Appendix cancer is rare and most commonly found incident
ally in an appendectomy specimen (incidence ~1%) that wa
s obtained for an unrelated condition.
• The main histologic types of appendiceal neoplasms are;
• Gastroenteropancreatic neuroendocrine tumors (GEP-NETs, previou
sly called carcinoids). The predominant (50%) histologic type
… !
• Appendiceal mucoceles and mucinous neoplasms of the appendix (C
ystadenomas, Cystadenocarcinomas)
• Adenocarcinomas; The most common appendiceal tumor … !
 Presentation
• Patients with appendiceal neoplasms may present with;
• The majority of cases discovered incidentally during a
n operative procedure for an unrelated cause.
• Almost 1/3rd of cases present with features of acute a
ppendicitis.
• In contrast to other appendiceal tumors, adenocarcinomas mor
e often present with a clinical picture of acute appendiciti
s. Appendiceal adenocarcinomas have a propensity for early p
erforation as well.
• Some cases detected after regional spread of the disea
se with ascites or a palpable mass.
• Imaging; CT scan features of mucinous neoplasms
• Benign; low attenuation, round, well encapsulated cystic mass
 Gastroenteropancreatic Neuroendocrine
Tumors (GEP-NETs or Carcinoid)
• Appendiceal carcinoid tumors are submucosal rubbery masses t
hat are detected incidentally on the appendix. They are rela
tively indolent but can develop nodal or hepatic metastases.
• Most patients have localized disease, and the prognosis is e
xcellent
• Infrequently, these can be associated with a carcinoid syndr
ome if there are hepatic metastases (2.9%).
• Upon incidental findings of a suspected carcinoid, the surge
on must evaluate the nodal basin along the ileocolic pedicle
and also examine the liver for any signs of metastases.
 Management

• For lesions < 1 cm (95% of cases);

• A negative margin appendectomy alone suffice in the absence of
mesoappendiceal invasion.
• For lesions 1-1.9 cm; completion colectomy…?
• A negative margin appendectomy alone may suffice in the absence
of mesoappendiceal invasion, enlarged nodes and adverse histolo
gic features (+ve or unclear margins or mixed histology (e.g, a
denocarcinoid)). But in the presence of such features right hem
icolectomy is recommended
• For tumors  2 cm; right hemicolectomy
• Right hemicolectomy is also recommended for well-differentiated
NETs
 Posttreatment surveillance

• For appendiceal NETs ≤2 cm confined to the appen

dix and treated by simple appendectomy
• No follow-up is required.
• For larger or node +ve tumors treated by right he
micolectomy, post-Rx surveillance is recommended;
• Hx & PE, serum chromogranin A, and CT scan at 3-12
months postresection; then beyond one year Hx & PE Q
6-12 months and chromogranin A testing and CT scan Q 1
year.
 Epithelial tumors
• The spectrum of the appendix epithelial tumors ra
nges from the benign mucocele to an aggressive ad
enocarcinoma.
• Appendiceal mucocele; the term broadly describes a mucu
s-filled appendix that could be secondary to neoplastic
or nonneoplastic pathologies.
• Mucocele; simple or retention cysts,
• Mucosal hyperplasia,
• Mucinous neoplasms
 Mucinous neoplasms

• Mucinous neoplasms has

3 main categories. WHO
2010
• Mucinous adenoma
• LAMN
• Adenocarcinoma
• It avoids the term cyst
adenomas, cystadenocarc
inoma.
 Management

• Surgical resection should be pursued, even for a benign-a

ppearing appendiceal mucocele, as these may harbor a cyst
adenocarcinoma.
• Standard appendectomy; for retention cysts, mucosal hyperplasia
, and mucinous adenomas (cystadenomas) confined to appendix.
• Appendectomy with resection of mesoappendix; for cystadenocarci
noma without mesenteric, adjacent organ, or peritoneal involvem
ent.
• Right hemicolectomy; for complicated mucocele (involvement of t
he terminal ileum or cecum) and cystadenocarcinomas with mesent
eric or adjacent organ involvement.
 Operative issues
• Laparoscopic removal; if there is a homogeneous "cyst" i
nvolving the appendix, no wall irregularity and hint of
spread
• The chance of nodal spread is quite small relative to th
e typical appendiceal adenocarcinoma, and an acceptable
approach (assuming negative resection margins) is to do
nothing further.
• Careful handling and resection of neoplastic mucocele; t
o avoid rupture and peritoneal contamination and dissemi
nation of neoplastic cells. Subsequent dev’t of PMP are
nearly certain in cases of adenocarcinoma.
• Careful examination of intra-abdominal structures;
• To rule out concurrent malignancies. Appendiceal mucoceles m
ay appear associated with colorectal (concurrent CRC in ~ 20
% of patients), ovarian, endometrial, breast, and kidney tum
ors.
• To assess the presence of ascites, peritoneal disease, and l
iver metastasis
• Obtain biopsies; When there is discordance between the primary
lesion histology and the peritoneum, the peritoneal histology i
s usually given priority.
• Adjuvant CRT in this setting is not well defined.
 LAMN

• Low-grade appendiceal mucinous neoplasm (LAMN)

• Confined to appendix; Negative margin appendectomy, ileocececto
my rarely
• Peri-appendiceal acellular mucin dissecting through the wall (t
4a) or adjacent organs (t4b); Negative margin appendectomy, res
ection of acellular mucin
• Peri-appendiceal Epithelial cells dissecting through the wall o
r adjacent organs; Negative margin appendectomy, laparoscopic p
eritoneal surveillance Vs HIPEC
• Distant epithelial cells or acellular mucin (M1a) and Low grade
mucinous carcinoma peritonei; Negative margin appendectomy, ome
ntectomy, HIPEC
• High-grade appendiceal mucinous neoplasm (HAMN-rare); Sim
 Adenocarcinoma
• It has 3 major histologic subtypes: mucinous adenocarcin
oma, colonic adenocarcinoma (nonmucinous), goblet cell histo
logy (adenocarcinoid).
• Confined to appendix; right hemicolectomy, a standard Rx for m
ost adenocarcinoma of appendix, and for tumors that are more deepl
y invasive.
• Peritoneal Dissemination (PMP) and High grade mucinous c
arcinoma peritonei; Surgical debulking (repeated), Cytor
eductive surgery and HIPEC, adjuvant CT … !
• Routine oophorectomy at the time of colectomy because the ovari
es are a common organ for metastases. Resection of ovaries that
are involved with metastatic spread is clearly beneficial.
 Lymphomas
• Appendiceal lymphomas are rare (1-3% of lymphomas, usual
ly non-Hodgkin’s) and difficult to diagnose preoperativ
ely (appendiceal diameter can be  2.5 cm).
• Management
• Appendectomy in most cases.
 References

• Schwartz, 11th edition

• UpToDate, 2018
Thanks ... !