Mucinous Neoplasms of the Appendix and Peritoneum

Nicole C. Panarelli, MD; Rhonda K. Yantiss, MD

Context.Appendiceal mucinous neoplasms are considNered

enigmatic tumors of unpredictable biologic potential.
Their importance lies in their potential to spread to the
peritoneum and viscera in the form of gelatinous mucin
deposits. Extra-appendiceal spread of these tumors is the
most common etiology of pseudomyxoma peritonei, which
is a descriptive term encompassing a number of neoplastic
and nonneoplastic peritoneal disorders. Many studies
aimed at evaluating the biologic importance of appendiceal mucinous neoplasms and pseudomyxoma peritonei
have employed inconsistent histologic criteria for their
diagnosis and descriptive terminology for their classification. As a result, appendiceal mucinous neoplasms and
associated peritoneal disease represents one of the most
confusing and controversial areas in gastrointestinal
pathology.
rimary appendiceal tumors are found in less than 2% of
P
surgically removed appendices, but still pose a variety
of diagnostic and therapeutic challenges. In particular, the
classification and management of appendiceal mucinous
neoplasms has proven problematic for both clinicians and
pathologists. These tumors characteristically cause cystic
dilatation of the appendix owing to accumulation of copious
gelatinous material in the lumen (Figure 1, A and B). They
may disseminate throughout the peritoneal cavity in the
form of gelatinous deposits, termed pseudomyxoma peritonei
(Figure 1, C and D). Many controversies surrounding
appendiceal neoplasms and pseudomyxoma peritonei stem
from the use of inconsistent histologic criteria for their
diagnosis and philosophic differences of opinion regarding
their pathogenesis. Several investigators have proposed
classification schemes that employ similar terminology to
describe lesions of variable biologic potential or that use
dissimilar terms to describe the same entity. Moreover, an
origin in the appendix is not suspected before surgery
in many cases because patients present with ovarian or
peritoneal tumors that are managed by gynecologists, rather
than general or colorectal surgeons. These different clinical
Accepted for publication March 11, 2011.
From the Department of Pathology and Laboratory Medicine, Weill
Cornell Medical College, New York, New York.
The authors have no relevant financial interest in the products or
companies described in this article.
Presented in part at the Tutorial on Pathology of the GI Tract,
Pancreas, and Liver; Westin Diplomat Resort & Spa; November 1519,
2010; Hollywood, Florida.
Reprints: Rhonda K. Yantiss, MD, Weill Cornell Medical College,
1300 York Ave, New York, NY 10065 (e-mail: rhy2001@med.cornell.
edu).
Arch Pathol Lab MedVol 135, October 2011

Objectives.To summarize the literature regarding the

biologic potential of appendiceal mucinous neoplasms and
pseudomyxoma peritonei and to discuss the similarities and
differences between proposed systems for their classification.
Data Sources.Literature review and case-derived
material.
Conclusions.Many studies have contributed to an
increased understanding of the natural progression of
mucinous neoplasms of the appendix and peritoneum, and
the adoption of a uniform reporting system, as advocated
by the American Joint Committee on Cancer and the
World Health Organization, will facilitate clear communication among pathologists and clinical colleagues.
(Arch Pathol Lab Med. 2011;135:12611268; doi: 10.
5858/arpa.2011-0034-RA)

groups are usually familiar with some, but not all, of the terminology used to describe mucinous tumors of the appendix
and peritoneum. As a result, management strategies across
different institutions, or even among clinicians at the same
institution, are not standardized. The purpose of this review
is to provide a comprehensive discussion of the biologic
potential of, and proposed classification schemes for, appendiceal mucinous neoplasms and pseudomyxoma peritonei.
WHAT IS PSEUDOMYXOMA PERITONEI?
Pseudomyxoma peritonei is a term used to describe
mucinous ascites or mucin deposits within the peritoneal
cavity. Pseudomyxoma peritonei consists of organizing
pools of mucin within peritoneal fat or on the serosal
surfaces of the viscera, which contain variable numbers of
neoplastic epithelial cells. Most cases reflect dissemination
of an appendiceal mucinous neoplasm, in which case,
mucin pools that contain scant strips and clusters of lowgrade neoplastic epithelial cells are typical (Figure 2, D),
similar to those present in the appendiceal mucosa
(Figure 2, A and B). However, pseudomyxoma peritonei
is not synonymous with a neoplasm nor are all neoplastic
cases derived from the appendix. Ruptured diverticula of
the appendix and colon may spill mucin into the peritoneal
cavity, and mucin-producing carcinomas of the colon,
pancreas, and other organs may disseminate throughout
the peritoneum in the form of gelatinous ascites. Unfortunately, much of the earlier literature is of limited value in
assessing the biologic potential of appendiceal mucinous
neoplasms and pseudomyxoma peritonei. Most reports
before 2000 did not distinguish between neoplastic
and nonneoplastic etiologies for pseudomyxoma peritonei, appendiceal and nonappendiceal neoplasms, or

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Figure 1. Mucinous neoplasms often cause appendiceal distention (A) because of massive accumulation of extracellular mucin within the lumen
(B). The neoplasms may spread to the serosal surfaces of the viscera (C) or form gelatinous tumor deposits in the omentum (D).

cytologically low- and high-grade tumors in the peritoneal

cavity.1 In our practice, we generally avoid using pseudomyxoma peritonei as a diagnostic term and limit its inclusion
in pathology reports to a comment aimed at facilitating
communication with clinicians.
ORIGIN OF PSEUDOMYXOMA PERITONEI
The combined results of several studies published in
theearly 1990s implicated the appendix as a likely site of
origin in most of nearly 100 patients with pseudomyxoma
peritonei but also suggested that some cases may be
derivedfrom either the ovary or peritoneum. Prayson etal2
evaluated a series of 10 men and 9 women with
pseudomyxoma peritonei and found that 94% of patients
had appendiceal mucinous neoplasms, including all of the
women with ovarian tumors. Seidman et al3 found a high
frequency of bilateral ovarian mucinous tumors, or rightsided ovarian mucinous tumors in unilateral cases, among
women with concomitant mucinous tumors of the
appendix, leading them to conclude that pseudomyxoma
peritonei may be derived from either site or represent a
primary peritoneal neoplasm. Young et al4 studied 22
appendiceal mucinous neoplasms associated with mucinous ovarian tumors and noted that tumors in both sites
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were histologically similar. They also found ovarian

disease to be bilateral in most cases, whereas unilateral
ovarian tumors showed a right-sided predominance.
Unlike Seidman et al,3 however, they concluded that these
features suggested a primary appendiceal origin with
secondary ovarian involvement.
Molecular data further support the notion that pseudomyxoma peritonei is usually derived from the appendix,
rather than the ovary or peritoneum. Cautrecasas et al5
analyzed KRAS mutational status in 6 synchronous
mucinous tumors of the appendix and ovary, 5 (83%) of
which showed identical KRAS mutations in codon 12 in
tumors from both sites. Chuaqui et al6 analyzed loss of
heterozygosity on chromosomes 5 and 17 in synchronous
appendiceal and ovarian mucinous neoplasms obtained
from 12 patients. They found either loss of heterozygosity
at the same locus or no loss of heterozygosity in paired
tumors in 75% of cases. Subsequent studies have shown
progressive loss of heterozygosity in ovarian mucinous
tumors, when compared with synchronous appendiceal
neoplasms, leading most investigators to conclude that
most pseudomyxoma peritonei cases are derived from
appendiceal mucinous neoplasms, whereas an ovarian
origin is considered a rare occurrence.7,8

Mucinous Neoplasms of the Appendix and PeritoneumPanarelli & Yantiss

Figure 2. Mucinous neoplasms typically display a circumferential growth pattern in the appendiceal mucosa with a variable papillary architecture
(A). The tumor cells contain abundant cytoplasmic mucin and enlarged, hyperchromatic nuclei that are basally located, with minimal cytologic
atypia (B). Peritoneal mucin deposits contain scant strips and clusters of mucin-containing epithelial cells (C) that are cytologically low grade, similar
to the neoplastic cells present in the appendix (D) (hematoxylin-eosin, original magnifications 340 [A and C] and 3400 [B and D]).

PERIAPPENDICEAL MUCIN DEPOSITS (LOCALIZED

PSEUDOMYXOMA PERITONEI)
Not uncommonly, surgical pathologists encounter
appendiceal resection specimens with mucin deposits on
the appendiceal serosa, within the mesoappendix, or
limited to the right lower quadrant (Figure 3, A and B).
Extra-appendiceal mucin deposits limited to the periappendiceal area are usually devoid of neoplastic epithelial
cells (acellular), although one may infrequently observe
strips or clusters of neoplastic mucinous epithelial cells in
periappendiceal mucin, similar to cases with widespread
peritoneal disease (Figure 3, C and D). Yantiss et al9
evaluated the prognostic implications of peritoneal mucin
localized to the right lower quadrant in 65 patients with
appendiceal mucinous neoplasms. Fifty patients (77%)
had acellular extra-appendiceal mucin, and 15 (23%) had
periappendiceal mucin deposits that contained scant
mucinous epithelium with low-grade cytologic features.
At follow-up, 48 (96%) patients without extra-appendiceal
epithelium were disease free (mean, 52 months), whereas
33% of patients with any neoplastic epithelium outside the
appendix developed widespread peritoneal tumor deposits, including one patient who died of disease (mean,
Arch Pathol Lab MedVol 135, October 2011

38 months). Notably, 2 patients with acellular periappendiceal mucin developed disseminated peritoneal disease,
but neither of those appendices was submitted entirely for
histologic examination. These results emphasize the
importance of adequate sampling in determining the
prognosis of patients with appendiceal mucinous neoplasms and indicate that tumors without extra-appendiceal neoplastic epithelium are associated with an excellent
prognosis.
CLASSIFICATION OF APPENDICEAL MUCINOUS
NEOPLASMS AND PSEUDOMYXOMA PERITONEI
The proposed classification schemes for appendiceal
mucinous neoplasms and pseudomyxoma peritonei reflect differences of opinion regarding the fundamental
nature of mucin deposits in the peritoneum and comparisons between them are summarized in the Table. Some
investigators think that peritoneal mucin deposits are
the consequence of appendiceal rupture and spillage of
mucin and adenomatous epithelium into the peritoneal cavity, rather than carcinoma. They argue that
appendiceal mucinous tumors frequently lack infiltrating,
malignant glands and desmoplastic stroma and note that

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Figure 3. Appendiceal mucinous neoplasms may show areas of mucin extrusion through the wall (A) or organizing mucin on the serosal surface
(B). The mucin deposits contain inflammatory cells and granulation tissue in most cases (C), but occasional tumors are associated with extraappendiceal neoplastic epithelium, similar to that seen in patients with pseudomyxoma peritonei (D) (hematoxylin-eosin, original magnifications
320 [A], 340 [B], 3400 [C], and 3600 [D]).

extra-appendiceal epithelium usually shows bland cytologic features insufficient for a diagnosis of malignancy.
On the other hand, there are compelling reasons why the
American Joint Committee on Cancer, World Health
Organization, and most other investigators consider
pseudomyxoma peritonei to be carcinoma.10,11 First, some
carcinomas are extremely well differentiated with minimal atypical cytologic features, so low-grade cytology
does not exclude a diagnosis of malignancy. Second,
parenchymal invasion of solid organs is common in
patients with pseudomyxoma peritonei and is generally
considered a feature of malignancy. Third, some cancers,
particularly mucinous or colloidal carcinomas may invade
tissues with a broad, pushing growth pattern, so one need
not see infiltrating glands or desmoplasia to make a
diagnosis of malignancy. Finally and most important,
mucinous neoplasms that spread beyond the appendix are
associated with progressive mucinous ascites, disease
recurrence, and death in at least half of patients.1214
In a study of 184 epithelial noncarcinoid appendiceal
tumors, Carr et al13 found that mucinous neoplasms
limited to the appendix pursued an indolent course,
whereas those with extra-appendiceal spread were associated with decreased survival. These authors proposed
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that appendiceal mucinous neoplasms be classified as

adenomas (or cystadenomas), tumors of uncertain malignant potential, and carcinoma. Adenomas were defined
by the presence of mucinous neoplasia confined to the
mucosa with intact muscularis mucosae, whereas tumors
that showed mural invasion or proliferation of neoplastic
epithelium outside the appendix were considered carcinomas. Neoplasms with pushing, but not infiltrative,
mural invasion or acellular mucin pools on the serosa
were considered to be of uncertain malignant potential.
Misdraji et al15 later analyzed the histologic features of
107 appendiceal mucinous tumors, which they classified
as either low- or high-grade based on cytoarchitectural
features. Tumors with low-grade cytology were termed
low-grade appendiceal mucinous neoplasms. High-grade
lesions limited to the appendiceal mucosa were termed
noninvasive mucinous cystadenocarcinomas, whereas
peritoneal deposits containing overtly malignant cells
were labeled as invasive mucinous adenocarcinomas. All
patients with low-grade neoplasms confined to the
appendiceal mucosa were free of disease at last followup (median, 6 years), whereas 67% of patients with
peritoneal involvement experienced disease recurrence or
death from disease. Patients with overtly malignant

Mucinous Neoplasms of the Appendix and PeritoneumPanarelli & Yantiss

Comparisons Among Classification Schemes for Appendiceal Mucinous Neoplasms and Pseudomyxoma Peritonei
Source, y
Carr and Sobin,11
2010

Misdraji et al,15 2003

Pai and
Longacre,17 2009

Ronnett et al,1
1995

Bradley
et al,12
2006

AJCC and
WHO10,11 2010

Tumor confined
to appendix
Limited to
mucosa
Low-grade
cytology
High-grade
cytology
Positive
surgical
margin
Neoplastic
epithelium
in appendix
wall
Tumor beyond
appendix
Low-grade
epithelium
in peritoneal
mucin
High-grade
epithelium
in peritoneal
mucin

Adenoma
Adenoma
Adenoma
Uncertain
malignant
potential

Low-grade
appendiceal
mucinous neoplasm
Noninvasive mucinous
cystadenocarcinoma
Low-grade
appendiceal
mucinous neoplasm
Low-grade
appendiceal
mucinous neoplasm

Adenoma

NA

NA

Adenoma

Adenoma

NA

NA

Adenoma

Uncertain
malignant
potential
Uncertain
malignant
potential

NA

NA

Adenoma

NA

NA

Invasive Mucinous
Adenocarcinoma

Disseminated
peritoneal
adenomucinosis

Low-grade
Low-grade
mucinous
mucinous
carcinoma
adenocarcinoma
peritonei
High-grade High-grade
mucinous
mucinous
carcinoma
adenocarcinoma
peritonei

Invasive mucinous Low-grade

adenocarcinoma
appendiceal
mucinous neoplasm

High-risk for
recurrence

Invasive mucinous Invasive mucinous

adenocarcinoma
adenocarcinoma

Invasive mucinous Peritoneal

adenocarcinoma
mucinous
carcinomatosis

Abbreviations: AJCC, American Joint Committee on Cancer; NA, not applicable; WHO, World Health Organization.

peritoneal disease had 3- and 5-year survival rates of 90%

and 44%, respectively, compared with 100% and 86%,
respectively, among patients with low-grade peritoneal
disease. The authors failed to identify any pathologic
features in the appendiceal component that reliably
predicted the presence or absence of pseudomyxoma
peritonei and suggested that all low-grade tumors be
labeled low-grade appendiceal mucinous neoplasm, regardless
of the extent of disease, which would be documented in
the pathology report.
Two years later, Pai and Longacre16 proposed an
alternative classification scheme that built on that suggested by Carr et al.13 They recognized lesions confined to
the appendiceal mucosa as adenomas but expanded the
definition of mucinous tumors of uncertain malignant
potential to include those tumors with mucosal disease at
the proximal margin, those with mucin and epithelium in
the appendiceal wall without obvious destructive invasion, and those in which the diagnosis of extra-appendiceal epithelium was in doubt. Mucin pools containing
scant, low-grade epithelium outside the appendix were
classified as mucinous neoplasms of low malignant potential,
whereas those with destructive invasion were considered
to represent adenocarcinomas.
This same group later analyzed 116 mucinous appendiceal neoplasms and modified their original classification
scheme to a 4-tiered system.17 Low-grade tumors confined
to the appendiceal mucosa were classified as adenomas,
whereas similarly low-grade tumors with acellular extraappendiceal mucin were considered to be of low risk for
recurrence. High-grade tumors in the peritoneal cavity
were classified as adenocarcinomas, similar to their prior
Arch Pathol Lab MedVol 135, October 2011

proposal. Cytologically low-grade tumors with extraappendiceal epithelium (previously termed mucinous
neoplasms of low malignant potential) recurred in 21 of 27
cases (78%) and, thus, were reclassified as low-grade
mucinous neoplasms with high-risk of recurrence. All patients
with invasive carcinoma and who had available follow-up
data died of disease with significantly decreased overall
survival rates compared with patients with low-grade
extra-appendiceal disease.
Other authors have devised classification schemes for
pseudomyxoma peritonei based on the cytologic appearance of peritoneal epithelium. Ronnett et al1 considered
pseudomyxoma peritonei with low-grade cytologic features to represent passive spread of adenomatous
epithelium into the peritoneum, secondary to appendiceal
rupture, and, thus, termed such cases disseminated peritoneal adenomucinosis (Figure 4, A and B). They consider
mucinous tumors of the peritoneum with more-abundant,
cytologically malignant epithelium to represent peritoneal
mucinous carcinomatosis (Figure 4, E and F), and cases with
both low- and high-grade features were designated
peritoneal mucinous carcinomatosis, intermediate grade (Figure 4, C and D). The authors applied this classification
scheme to 109 multifocal peritoneal mucinous tumors
derived from the appendix, colon, or small intestine and
compared survival rates among the 3 groups. All 65 cases
of disseminated peritoneal adenomucinosis were derived
from the appendix and were associated with 5- and 10-year
survival rates of 75% and 68%, respectively. Both intermediate and high-grade mucinous carcinomas were more
frequently derived from nonappendiceal primary tumors
and pursued an aggressive clinical course with 5- and

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1265

Figure 4. Mucin deposits in the peritoneal cavity classified as disseminated peritoneal adenomucinosis (A and B), peritoneal mucinous
carcinomatosisintermediate grade (C and D), and peritoneal mucinous carcinomatosis (E and F). Disseminated peritoneal adenomucinous contains
paucicellular mucin pools (A) with scant strips of low-grade neoplastic epithelium (B). Peritoneal mucinous carcinomatosis displays mucin pools
with abundant epithelium (E) that show overtly malignant cytologic features (F). Peritoneal mucinous carcinomatosisintermediate grade is less
cellular than peritoneal mucinous carcinomatosis (C), but the degree of cytologic atypia exceeds that of disseminated peritoneal adenomucinosis (D)
(hematoxylin-eosin, original magnifications 3100 [A], 3600 [B and F], 340 [C], 3200 [D], and 320 [E]).

10-year survival rates of 50% and 21% for intermediategrade peritoneal mucinous carcinomatosis and 14% and
3% for high-grade peritoneal mucinous carcinomatosis,
respectively.1,14 Although these results suggest histologic
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grade is important in predicting behavior among peritoneal mucinous tumors, inclusion of intestinal carcinomas
in the analysis of high-grade tumors makes it difficult to
interpret the data.

Mucinous Neoplasms of the Appendix and PeritoneumPanarelli & Yantiss

Bradley et al12 better demonstrated the importance of

cytologic grade in predicting behavior of pseudomyxoma
peritonei in a later study. These authors evaluated the
natural history of 101 patients with appendiceal mucinous
tumors and peritoneal disease, all of whom were treated
in a similar fashion at a single institution. These authors
used a 2-tiered system to classify pseudomyxoma peritonei. Low- and intermediate-grade tumors were considered
to be low-grade mucinous carcinoma peritonei, whereas
cases with severe cytologic atypia were classified as highgrade mucinous carcinoma peritonei. The authors found
that low-grade mucinous carcinoma peritonei was associated with a significantly better 5-year survival rate (63%)
than was high-grade mucinous carcinoma peritonei (38%).
The combined results of all of these studies can be
summarized into several major points. First, tumors
limited to the appendiceal mucosa have no potential for
aggressive disease when completely resected, regardless
of the degree of dysplasia, and, therefore, it is appropriate
to classify them as adenomas or cystadenomas. Appendiceal mucinous neoplasms associated with acellular mucin
limited to the appendiceal wall and/or mesoappendix are
also cured by excision, in most cases and, thus, we
consider these tumors to be adenomas as well. However,
all neoplasms that are classified as adenomas should be
completely submitted for histologic evaluation to exclude
the possibility of extra-appendiceal epithelium. Clinicians
should also be informed of the remote possibility that a
small amount of extra-appendiceal epithelium may not be
detected by sampling of tumors with acellular periappendiceal mucin and thus, clinical follow-up should probably
be considered. Any proliferation of neoplastic epithelium
beyond the muscularis mucosae is at risk for peritoneal
dissemination and should be graded and staged as a
carcinoma, as described in the World Health Organization
classification and staging guidelines put forth by the
American Joint Committee on Cancer.10,11
The World Health Organization regards any neoplastic
epithelial proliferation confined to the appendiceal mucosa as an adenoma. Appendiceal mucinous tumors with
extra-appendiceal neoplastic epithelium are classified as
mucinous adenocarcinomas and subcategorized as lowor high-grade because increasingly severe cytoarchitectural atypia is associated with poorer outcome.11 The
American Joint Committee on Cancer staging guidelines
assign tumor (T) stage for appendiceal mucinous neoplasms similar to those used for colonic adenocarcinoma,
with the exception that T4a denotes both serosal involvement and extra-appendiceal disease limited to the right
lower quadrant. Mucinous deposits beyond the right
lower quadrant (pseudomyxoma peritonei) are considered to represent metastatic disease and are denoted as
M1a.10 Confusing terminology, such as low-grade appendiceal mucinous neoplasm in the peritoneum, disseminated peritoneal adenomucinosis, and mucinous neoplasm at high-risk for recurrence, should probably be
avoided to facilitate clear communication with our clinical
colleagues.
TREATMENT OF PSEUDOMYXOMA PERITONEI
Treatment of pseudomyxoma peritonei has historically
consisted of surgical debulking of gross disease, with or
without various forms of chemotherapy. Recent years
have seen a trend toward more aggressive management
strategies that include multiple surgeries, complete
Arch Pathol Lab MedVol 135, October 2011

peritonectomy, intraoperative intraperitoneal chemotherapy, and additional cycles of postoperative chemotherapy.

Miner et al18 retrospectively analyzed the relative contribution of histologic features of peritoneal disease, patient
characteristics, and extent of treatment in 97 patients who
underwent extensive debulking surgery, which achieved
complete cytoreduction in 53% of cases. They found that
low-grade cytology was independently associated with
disease-free survival and that 90% of patients who
achieved 10-year survival had tumors of low-histologic
grade. Outcome data from other studies using these
aggressive treatment strategies have also shown improved
survival among patients with low-grade peritoneal disease compared with survival rates of those with highgrade tumors, which are probably not amenable to
surgical management.1922 Thus, therapeutic decisions
largely rest on the distinction between low-and highgrade peritoneal disease.
SUMMARY AND CONCLUSIONS
Appendiceal mucinous neoplasms represent a relatively homogeneous group of neoplasms that pursue a
predictable clinical course based on tumor stage and
grade. Those confined to the appendiceal mucosa are
cured by excision, whereas any proliferation of neoplastic
epithelium beyond the mucosa places the patient at risk
for peritoneal dissemination. The histologic grade of
peritoneal disease is extremely important. Patients with
low-grade tumors may benefit from aggressive management, consisting of a combination of chemotherapy and
cytoreductive surgery, whereas those with high-grade
tumors probably do not benefit from aggressive debulking
but may be better served by systemic chemotherapy.
Decisions regarding clinical management require clear
communication among treating physicians, so adoption of
a uniform reporting system for appendiceal mucinous
neoplasms with peritoneal metastases by the World
Health Organization and American Joint Committee on
Cancer represents a major advancement in the field.
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