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482
G. T. Ault and K. G. Cologne
process of neoadjuvant chemoradiotherapy
can potentially take up to 3months.
– This may allow surgery on the metastatic
disease while treatment for the primary
lesion is still ongoing.
– Alternatively, radiotherapy on rectal lesions
can be reserved for overall disease that
responds favorably to initial treatments–
particularly the short-course variety.
• The data for the liver-rst approach are limited, and largely based on non-randomized
data, but seem to remain a viable method with
reasonable long-term results.
Colon-First Strategy
• Proponents of the colon-rst strategy hypothesize that the colon or rectum acts as an ongoing source of seeding metastatic disease.
• Additionally, the primary tumor represents a
potential source of bowel-related morbidity in
the form of bleeding, obstruction, or
perforation.
• Some authors suggest the rates of this can be
as high as 20%, but others suggest it is much
lower than this.
• Additionally, the risk of morbidity associated
with a colorectal anastomosis may be
increased by addition of a liver procedure,
where anesthesia and surgical techniques may
include a low-ow state or temporary alteration in portal blood circulation (e.g., a Pringle
maneuver), which may affect blood ow to the
bowel.
• Resection of the colon rst may allow detection of new or occult liver metastases which
can then be removed with denitive surgery.
Margin Status
• Excision to negative margins in hepatic resection results in improved disease-free and overall survival.
• A consensus statement from the Society of
Surgical Oncology concluded that while wide
margins of >1cm are desirable, a close margin
should not preclude resection. Another study
suggested that a margin <5mm is a risk factor
for local recurrence.
Ablation ofLiver Metastases
• There are a variety of techniques for ablation
of liver metastases that do not require tissue
resection: percutaneous ethanol ablation,
radiofrequency ablation, microwave ablation,
cryoablation, and irreversible electroporation
(or nanoknife).
• Radiofrequency ablation is the most commonly employed technique, though all can be
used successfully and also used in conjunction
with other forms of therapy (e.g., surgical
resection).
• Several different treatments may be used
simultaneously or in a staged approach.
Other Liver Metastasis Strategies: Hepatic Intra-arterial Chemotherapy/Chemoembolization
• Regional chemotherapy through the hepatic
artery is a therapeutic option for patients with
isolated liver metastasis that are not amenable
to surgical resection or local ablation.
• This method can also be combined with other
forms of treatment.
• This mode of therapy is based upon the fact
that liver macrometastases derive more than
80% of their blood supply from the hepatic
arterial circulation, while normal hepatocytes
are supplied primarily by the portal circulation. This allows selective delivery of drug to
the tumor with relative sparing of the normal
hepatocytes.
• There is also a marked increase in the local
concentration of the chemotherapy that is
achieved by injection into the hepatic artery.
Regional administration of agents that are rapidly metabolized in the liver by a rst-pass
effect leads to higher levels of drug exposure
and minimizes side effects.
Pulmonary Metastasis
• Approximately 10% of patients with colorectal cancer develop pulmonary metastasis.
• The vast majority of patients with metastatic
colorectal cancer to the lungs have advanced
36 Colorectal Cancer: Management ofStage IV Disease
483
disease and are therefore treated with systemic
chemotherapy or best supportive care.
• Due to differences in blood supply, pulmonary
metastases may be more common after rectal
(vs. colon) cancer due to the dual blood supply
of the rectum (portal and systemic).
• Due to the retrospective nature of the reported
information in the literature, clinical outcome
data after metastasectomy for colorectal lung
metastases must be interpreted with caution.
• Improved clinical outcome and survival data
is more likely due to ideal patient selection
and tumor biology rather than the surgical
intervention in and of itself. In addition, there
are no adequate control groups in these
reports; therefore, survival statistics are difcult to interpret.
• However, there are patients who undergo pulmonary metastasectomy with no evidence of
disease after long-term follow-up. In addition,
long-term survival without complete resection
is very rare, suggesting that select patients do
occasionally benet from pulmonary metastasectomy. Input on the resectability of lung
lesions by a thoracic surgeon at multidisciplinary evaluation is essential.
• Modern series of lung resection for metastatic
colorectal cancer report operative mortalities
of less than 2% (Table36.1).
• Radiation therapy for colorectal cancer pulmonary metastasis has been of limited utility
in the past due to radiation-induced pneumonitis, rib and spinal fractures, and skin toxicities. However, these toxicities can be
minimized with the advent of roboticassisted Gamma Knife radiotherapy or
“Cyberknife.”
• While it appears that certain colorectal cancer
patients would benet from pulmonary metastasectomy even in the presence of liver metastases, no randomized controlled trials have
been conducted and reported, and the effectiveness of pulmonary metastasectomy has
been suggested mostly by results of retrospective analyses.
Peritoneal Metastasis
• The peritoneal surface is involved in approximately 10–15% of colorectal cancer patients
at time of initial presentation (synchronous
metastases) and in 20–50% of patients who
develop recurrence (metachronous
metastases).
• As a site of colorectal cancer metastasis, the
peritoneal surface ranks second only to the
liver.
• It is characterized by intraperitoneal spread of
metastatic nodules.
• The most frequent signs/symptoms are ascites
and bowel obstruction.
Table 36.1 Outcome of patients undergoing pulmonary metastasectomy for colorectal cancer
Study n Operative mortality (%) 5-yr survival (%) Signicant risk factors
Mori etal. 35 – 38 None found
McCormack etal. 144 0 44 Margin
McAfee etal. 139 1 31 Number of lesions, CEA
Yano etal. 27 – 41 Number of lesions
Saclarides etal. 23 – 16 Number of lesions
van Halteren etal. 38 – 43 DFI
Shirouzu etal. 22 – 37 Number of lesions, size
Girard etal. 86 1 24 CEA, margin
Okumura etal. 159 2 41 Number of lesions, LN status
Zanella etal. 22 0 62 None found
Zink 110 0 33 Size, CEA
Dahabre etal. 52 – 33 None found
n number of patients, yr year, LN lymph nodes, DFI disease-free interval
Adapted from Rizk NP, Downey RJ. Resection of pulmonary metastases from colorectal cancer. Semin Thorac
Cardiovasc Surg. 2002;14(1):29–34
484
G. T. Ault and K. G. Cologne
• Peritoneal metastasis occurs by direct
implantation of cancer cells via one of four
mechanisms: (1) spontaneous intraperitoneal seeding from a T4 colorectal cancer that
has penetrated the serosal surface of the
colon, (2) extravasation of tumor cells at the
time of colon perforation from an obstructing cancer, (3) iatrogenic tumor perforation
through an area of serosal injury or enterotomy at the time of colon resection, and (4)
leakage of tumor cells from transected lymphatics or veins at the time of colon
resection.
• The risk of peritoneal metastasis is therefore
highest in the setting of locally advanced cancers, and until recently, most oncologists
viewed peritoneal carcinomatosis as a terminal condition, to be palliated only with systemic chemotherapy.
• However, in a small set of cases, the peritoneal
cavity is determined to be the only site of metastatic disease after a detailed workup of the
lungs and liver. This has led some to hypothesize that in some cases, peritoneal carcinomatosis may represent a rst site of dissemination,
and therefore, not necessarily indicative of
generalized disease.
• Preoperative detection of peritoneal metastases is not reliable. Noninvasive imaging frequently misses small peritoneal lesions, even
when these are widely disseminated.
• The extent of carcinomatosis is a major prognostic factor and is best assessed by either
laparoscopic or open exploration.
– Two different peritoneal carcinomatosis
staging systems (Gilly’s classication and
Peritoneal Cancer Index of Sugarbaker)
can be used to assess the extent of carcinomatosis. These staging systems have both
shown utility in determining the prognosis
and treatment of patients with peritoneal
carcinomatosis.
• Standard management of patients known to
have peritoneal metastases at initial presentation (if known preoperatively) is systemic
chemotherapy.
• Colon resection plays an important role for
patients with obstructing primary cancers and
also for patients with occult metastases that
are rst detected in the operating room.
• Historically, the median survival for patients
with unresected peritoneal metastasis treated
with 5-uorouracil-based systemic chemotherapy was very poor (6–8months).
– However, patient survival is highly vari-
able, depending on the extent of metastatic
disease and response to chemotherapy.
– Contemporary combination chemotherapy
regimens have signicantly greater efcacy and can produce long periods of disease control in certain patients.
• Despite the grim prognosis for patients with
peritoneal carcinomatosis from colorectal
cancer, a subset of patients once thought
unsalvageable are now being considered for
surgery with curative intent.
– Pioneered by Sugarbaker, the goal of cyto-
reductive surgery and intraperitoneal (IP)
chemotherapy is to remove all macroscopic
disease with peritonectomy procedures and
visceral resections followed by perioperative IP chemotherapy to destroy residual
microscopic disease.
– IP delivery offers pharmacokinetic advan-
tage over standard intravenous delivery by
producing high regional concentrations of
drug while simultaneously minimizing systemic toxicities.
– For patients with isolated peritoneal carci-
nomatosis from colorectal cancer, radical
surgery to achieve an R0 resection (if it can
be accomplished) remains the mainstay of
treatment.
• Benet from cytoreductive surgery with
heated intraperitoneal chemotherapy has been
suggested in several retrospective case series,
a multi-institutional registry review, two randomized trials, and a systematic review.
– These randomized trials must be inter-
preted with caution as neither used modern
combination chemotherapy as the control
arm.
– While patients who undergo complete
cytoreduction followed by HIPEC may
seem to have a more favorable prognosis
than can be achieved with systemic chemo-
36 Colorectal Cancer: Management ofStage IV Disease
485
therapy alone, there remains insufcient
evidence to conclude whether the survival
advantage is due to treatment or to biologic
features that allow these patients to undergo
complete cytoreductive surgery (selection
bias).
• In published series, the overall treatmentrelated morbidity rates range from 23% to
44% and mortality rates from 0% to 12%.
• Many important unanswered questions
remain, including which patients with colorectal cancer peritoneal carcinomatosis have a
higher or considerably lower likelihood of
long-term survival after cytoreductive surgery
and HIPEC (Fig.36.5) and whether results in
any population are better than could be
achieved using modern oxaliplatin and/or irinotecan-based systemic chemotherapy with or
without biologic agents.
– In addition, the quality of the cytoreductive
surgery is dependent upon the skills and
level of experience of the surgeon. The
favorable results (particularly with regard
to treatment-related toxicity achieved by
international experts in the eld) may not
be replicated in routine clinical practice.
• Because of all of these issues, the National
Comprehensive Cancer Network [NCCN]
believes this approach should not be considered standard at present and only pursued in
centers with demonstrated expertise, preferably in the context of a clinical trial.
Fig. 36.5 Hyperthermic intraperitoneal chemotherapy
(HIPEC). (Courtesy of Eric K.Johnson, MD)
Ovarian Metastases
• Approximately 4–30% of ovarian neoplasms
are metastatic cancers with the most common
being colorectal and breast cancer.
• A recent autopsy study demonstrated that of
all women dying with colorectal cancer,
between 6% and 14% were found to have
ovarian metastasis at the time of death.
• For those women with stage IV disease, the
risk of developing ovarian metastases is substantially higher and approaches 90% for
those with established peritoneal metastasis.
• Therefore in a woman with a recent diagnosis
of advanced colorectal cancer, any ovarian
mass should be considered a metastasis from
colorectal cancer until proven otherwise.
• The removal of macroscopically normal ovaries (prophylactic oophorectomy) in women
with colorectal cancer is controversial and
remains the subject of much debate.
– Proponents of removal argue that resection
improves the cure rate by removing potential microscopic “undetectable” synchronous disease, eliminates the risk of ovarian
cancer, and removes the risk of future
metachronous ovarian metastatic disease.
– Others argue that the low incidence of
ovarian metastasis, the small amount of
supportive data, and few clinical correlations with predictive value make the additional resection of the ovaries unnecessary.
• Clinical studies attempting to document the
benet of ovarian metastasectomy in patients
with colorectal cancer are small and
retrospective.
– The majority of studies to date, however,
fail to show any survival benet for prophylactic oophorectomy, and most studies
demonstrate that when ovarian metastasis
is present, it is a very poor prognostic sign.
• Based on available information, it is reasonable to offer prophylactic oophorectomy to all
postmenopausal patients, in particular to those
women who have undergone pelvic radiation
as part of their treatment for rectal cancer.
• For premenopausal patients, only those with
established peritoneal metastases, those that
486
G. T. Ault and K. G. Cologne
Ovarian assessment
Normal
Pre-menopausal Post-menopausal
Consider
No indication for
oophorectomy
Fig. 36.6 Treatment algorithm for ovarian metastases
prophylactic
oophorectomy
if discussed
pre-procedurally
are proven to have an increased risk of developing ovarian carcinoma (strong family history, known carriers of breast cancer
(BRCA), or those with a HNPCC mutation),
or those who have already completed their
families should be considered for prophylactic oophorectomy.
• See Fig. 36.6 for an algorithm outlining the
treatment of ovarian metastases.
Other Sites ofMetastasis
Bone
Abnormal
Pre-menopausal
Unilateral or en
bloc oophorectomy
of obvious tumor
(consider
preservation of
normal-appearing
ovary until
discussion on
fertility)
Post-menopausal
En bloc
oophorectomy.
Consider
prophylactic
contralateral
oophorectomy
• There are no curative modalities, but palliation of pain, fractures, and spinal cord
involvement are important issues for these
patients.
– Symptomatic relief from bony metastases
can usually be accomplished with radiation, chemotherapy, as well as bisphosphonate therapy with zoledronic acid.
– Pathologic fractures are best treated by
operative internal xation.
– The systemic issues related to bone metas-
tases are serious and include debilitation,
immobility, hypercalcemia, and thromboembolic disease.
• Before the introduction of modern chemotherapy and targeted treatment options, bone
metastases were reported in as high as 24% of
cases. With modern therapies, bone metastases
from colorectal cancer reportedly occur now in
7–9% of cases and most often present in the
context of widespread metastatic disease.
• Routine diagnostic bone imaging is not indicated in colorectal cancer patients, unless
there are specic bone-related symptoms.
• When bone metastasis occurs, it most
commonly occurs in the spine (65%), followed by the hip/pelvis (34%) and long
bones (17%).
Brain
• Cerebral metastases from colorectal cancer
are uncommon, occurring in 1–4% of colorectal cancer cases.
• Colorectal tumors account for approximately
3% of all metastatic brain tumors.
• These are generally found in the context of
widespread metastases to multiple organ sites
but on rare occasion can present as an isolated
brain metastasis.
• There is no role for routine brain imaging at
primary presentation or at presentation with
36 Colorectal Cancer: Management ofStage IV Disease
487
metastases elsewhere, unless there are specic
neurologic symptoms.
• Once brain metastases occur, symptoms are
common; palliative therapies include steroids
to decrease swelling and anticonvulsants to
control seizures.
• Denitive therapy of colorectal brain metastases usually involves surgery, radiation, or a
combination of the two.
• For isolated, single brain metastases, resection
can result in survival beyond 1–2years; however because brain metastases are infrequently
the sole site of metastatic disease, and because
survival is dismal regardless of therapy chosen, craniotomy is rarely indicated.
Pancreas
• The pancreas is an uncommon location for
solitary metastases from other primary
cancers.
• While the prevalence of pancreatic metastases
has been described as high as 6% to 11%,
reports of solitary resectable pancreatic metastases from colorectal cancer are extremely
rare.
• Although long-term survival is rare, surgical
resection can be performed safely in patients
with isolated pancreatic metastases from
colorectal cancer and in selected patients with
extrapancreatic disease.
• As with other sites of multiorgan metastases, a
multimodality approach is strongly recommended, and consideration for surgical resection should be taken in context with an overall
treatment plan and chances for improving survival while maintaining quality of life.
Adrenal
• Adrenal metastases are uncommon with 14%
found in one autopsy series.
• Isolated adrenal metastases are even rarer.
• Aggressive surgical resection for isolated
adrenal metastases is described in only a few
case reports or small series.
• In contrast to the situation with isolated adrenal metastases, the development of adrenal
metastases after liver resection for colorectal
cancer is associated with a poor prognosis,
and adrenalectomy is not warranted.
Retroperitoneal Lymph Nodes
• Isolated retroperitoneal nodal recurrence
occurs in less than 2% of patients following a
colorectal cancer resection with curative
intent.
• Salvage surgery has been previously avoided
due to the poor prognosis; however this concept is being challenged, and surgical resection can be entertained in select cases.
Metastatic Disease intheElderly
• Colorectal cancer remains one of the most
commonly diagnosed cancers in the world
with 60% of patients being over 70 years old
and 43% over 75.
• Therefore multidisciplinary teams will
increasingly see older patients with colorectal
cancer, and management of this distinct group
deserves special mention.
• When considering surgery – comorbidity,
functional dependency, and older age are
associated with early postoperative mortality
in patients with gastrointestinal malignancies,
with 30-day postoperative mortality rates
underestimating postoperative mortality in
older patients.
• In regard to chemotherapy, the data available
indicates that older patients derive the same
benet and have the same degree of toxicity as
younger patients. The clinical trial data however may not be reective of the average
elderly patient seen in practice that is often
suffering from more comorbidities and has
greater functional impairment.
– For these reasons, the International Society
of Geriatric Oncology (SIOG) previously
recommended that colorectal cancer
patients >65years of age requiring surgery
488
G. T. Ault and K. G. Cologne
should undergo a preoperative whole
patient evaluation of the most common
physiological side effects of aging, physical and mental ability, and social support.
– Further for those patients assessed as hav-
ing physical or psychological comorbidities, it was recommended that a geriatrician
was involved in the patient management.
• Since those original recommendations, there
have been multiple frailty indices proposed to
detect vulnerability in elderly patients with
cancer so that treatment can be adjusted
accordingly.
– The process of these assessments, however,
can be time-consuming, and prescreening
is often used to identify t patients who are
able to receive standard treatment versus
those in whom a full comprehensive geriatric assessment should be done.
– The four most common indices utilized
appear to be the abbreviated Comprehensive
Geriatric Assessment (aCGA), the
Vulnerable Elders Survey-13 (VES-13),
the Groningen Frailty Indicator (GFI), and
the Geriatric 8 (G8).
– Unfortunately at present, there is no uni-
versal screening tool that adequately identies frailty in at-risk older patients, and
sensitivity and specicity for these indices
ranged from 67% to 87% and 59% to 73%,
respectively, which questions the value
they may offer to the clinician.
– The American College of Surgeons NSQIP
Risk Calculator (http://riskcalculator.facs.
org) also uses degree of independence and
other comorbidity variables that can be
used to assess surgical risk in the elderly. In
particular, it also gives an assessment of the
risk of discharge to a long-term care facility in addition to the risk of common
complications.
Appendiceal Neoplasms
ConstantineP.Spanos andAndreasM.Kaiser
37
Abbreviations
AJCC American Joint Committee on
Cancer
DPAM Disseminated peritoneal
adenomucinosis
ENETS European Neuroendocrine Tumor
Society
HIPEC Hyperthermic (or heated) intra-
peritoneal chemotherapy
LAMNs Low-grade appendiceal mucinous
neoplasms
PCI Peritoneal carcinomatosis index
PMAC Peritoneal mucinous
adenocarcinomatosis
PMCA Peritoneal mucinous
carcinomatosis
PMP Pseudomyxoma peritonei
C. P. Spanos
Department of Colorectal Surgery, Keck School of
Medicine, University of Southern California,
Los Angeles, CA, USA
Department of Surgery, Aristotelian University of
Thessaloniki, Panorama-Thessaloniki, Greece
A. M. Kaiser (*)
Keck Medical Center of the University of Southern
California, Los Angeles, CA, USA
e-mail: akaiser@usc.edu
Key Concepts
• Although appendectomy for appendicitis is
the most commonly performed emergency
operation performed by general surgeons, primary neoplasms of the vermiform appendix
are rare, and each individual general surgeon
will have limited experience in the management of such lesions.
• Most primary neoplasms of the appendix are
not associated with specic signs or symptoms and are incidentally diagnosed after
pathological analysis of the appendectomy
specimen or detected incidentally on computed tomography (CT) for other indications.
• Primary neoplasms of the appendix can generally be divided into epithelial, non-epithelial,
and mixed lesions. Epithelial lesions include
adenoma and adenocarcinoma. Non-epithelial
tumors include neuroendocrine tumors (carcinoids), lymphoma, leiomyoma, leiomyosarcoma, and other even rarer rarities. Goblet cell
carcinoids are mixed lesions with features of
carcinoid as well as mucinous adenocarcinoma.
• Epithelial tumors, and specically mucinous
adenocarcinomas, are the most common primary appendiceal neoplasms.
• Pseudomyxoma peritonei is the result of a perforation and peritoneal dissemination of a
mucin-producing epithelial neoplasm, most
commonly originating from the appendix or
the ovaries. In select patients, cytoreductive
surgery with HIPEC should be considered.
© ASCRS (American Society of Colon and Rectal Surgeons) 2019
S. R. Steele et al. (eds.), The ASCRS Manual of Colon and Rectal Surgery,
https://doi.org/10.1007/978-3-030-01165-9_37
489
490
C. P. Spanos and A. M. Kaiser
• A mucocele is a morphologic cystic manifestation of an epithelial appendiceal neoplasm.
Perforation leads to pseudomyxoma peritonei.
Therefore, intact removal en bloc is of utmost
importance.
• Appendiceal carcinoids are rarely associated
with carcinoid syndrome or multicentricity.
• The newest tumor staging guidelines distinguish
appendiceal tumors from colon cancer and separate between epithelial and non- epithelial lesions.
• The extent of surgical resection depends on
the cell type, the preoperative staging, the
ability to achieve negative resection margins,
and the probability of nodal disease.
• Surgery is the primary treatment for localized
disease, whereas its role in metastatic disease
needs to be individually analyzed and weighed
against systemic chemotherapy.
Introduction
• Appendectomy is the most frequent emergency operation performed by general surgeons with close to 300,000 performed in the
United States annually.
• On comparably rare occasion, the pathology
of the appendectomy specimen incidentally
reveals an appendiceal neoplasm (“incidentaloma”), which sometimes is recognized
even before or at least during surgery, but
more often only after the patient has already
been discharged from the hospital.
• Paradoxically and despite the fact that abdominal surgeons at all levels are very frequently
involved in treating appendiceal pathology,
appendiceal neoplasms are quite infrequent
but may cause rather complex intellectual,
management, and technical challenges in subsequent surgical interventions (Table37.1).
Epidemiology
• Primary neoplasms of the appendix have an
incidence of 0.12 cases per 1,000,000 personyears and are found in 0.9–1.4% of appendectomy specimens.
• They can be asymptomatic, be associated with
appendicitis, or cause noninammatory
symptoms.
• Preoperative diagnosis based on symptoms,
imaging, and laboratory results is extremely
rare.
• Even intraoperatively, less than 50% appendiceal neoplasms are recognized as such.
• A retrospective cohort analysis of the
Surveillance, Epidemiology, and End Results
database suggested that the incidence of
appendiceal neoplasms has increased signicantly in the past few decades from 0.63 to
0.97 per 100,000 population.
– It is unclear though whether this is a true
increase or a simple reection of higher
awareness and reclassication as a separate
entity. The increase appears to have
affected all histological subtypes in an
equal fashion (Table37.2).
• Historically, carcinoid tumors were considered the most frequent neoplasms originating
within the appendix, although more recent
publications demonstrate that epithelial neoplasms are more frequent and represent 58%
of malignant appendiceal tumors.
• At the time of diagnosis, a total of 74% of
appendiceal cancer cases have already spread
and developed regional or distant metastases
in 39% and 35%, respectively.
Anatomical Pathology andStaging
• The literature unfortunately has for a long
time shown little consistency and used a variety of nomenclatures, classication systems,
and descriptive terms when referring to appendiceal neoplasms. Many synonyms for lesions
of such rarity undoubtedly have led to confounding terminology.
• From an anatomical point of view, the appendix
in essence has a smaller diameter but otherwise
a similar layered wall structure as the rest of the
large intestine; however there is a higher representation of immunological tissue components
(GALT, gut-associated lymphoid tissue).
37 Appendiceal Neoplasms
identication of affected individuals
Re-imaging?
Primary or secondary evaluation for
more extensive/oncological surgery/
treatment
Intraoperative determination of
appropriate extent of resection
perforation
Secondary assessment for more
Appropriate treatment for perforation
with primary or secondary evaluation
for more extensive/oncological
surgery/treatment
extensive surgery/treatment
Possible frozen section
tumor locations
Systemic treatment and evaluation for
Preparedness for HIPEC
CRS/HIPEC
PCI
Systemic treatment and evaluation for
CRS/HIPEC
491
(a) Intraoperative
(b) Only on nal pathology
(c) Not recognized at all (missed
P−
Pre-/intraop evidence of
perforation (P) or
dissemination (D) Timing of tumor recognition Impact/action in decision-making
Presumptive preoperative
diagnosis Surgery
Acute
symptoms
D−
(a) Intraoperative
(b) Only on nal pathology
opportunity)
D−
Intraoperative Primary appropriate treatment for
(c) Not recognized at all (missed
opportunity)
D+
Intraoperative: evidence of
P−
Preoperative Oncological resection
localized mucocele or tumor
involving the appendix/cecum
P−
D−
Y
Preoperative PCI
P−
D+
involving appendix/
D+
TBD
of appendix/cecum and
cecum– no signs of PMP
signs of PMP
Preoperative Evaluation for other potential primary
P−
D+
TBD
pathology
Pre-treatment Systemic treatment
P−
D+
N
+ distant metastases
1. Acute Y Appendicitis N – N Rely on indirect signs/risk factors for
Table 37.1 Clinical scenarios depending on the type and timing of diagnosis of appendiceal neoplasms
Scenario
2. Acute Y Appendicitis Y
3. Acute Y Appendicitis Y P+
4. Acute Y Appendicitis Y P+
5. Acute Y Appendicitis Y
6. Elective Y/N Localized mucocele/tumor
7. Elective Y/N Localized mucocele/tumor
8. Elective Y/N PMP, but no obvious cecal
9. Elective Y/N PMP