Mucinous Neoplasms of the Appendix and Peritoneum

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

Context.—Appendiceal mucinous neoplasms are consid-
enigmatic tumors of unpredictable biologic potential.
Objectives.—To summarize the literature regarding the
biologic potential of appendiceal mucinous neoplasms and
Their importance lies in their potential to spread to the pseudomyxoma peritonei and to discuss the similarities and
peritoneum and viscera in the form of gelatinous mucin differences between proposed systems for their classification.
deposits. Extra-appendiceal spread of these tumors is the Data Sources.—Literature review and case-derived
most common etiology of pseudomyxoma peritonei, which material.
is a descriptive term encompassing a number of neoplastic Conclusions.—Many studies have contributed to an
and nonneoplastic peritoneal disorders. Many studies increased understanding of the natural progression of
aimed at evaluating the biologic importance of appendi- mucinous neoplasms of the appendix and peritoneum, and
ceal mucinous neoplasms and pseudomyxoma peritonei the adoption of a uniform reporting system, as advocated
have employed inconsistent histologic criteria for their by the American Joint Committee on Cancer and the
diagnosis and descriptive terminology for their classifica- World Health Organization, will facilitate clear commu-
tion. As a result, appendiceal mucinous neoplasms and nication among pathologists and clinical colleagues.
associated peritoneal disease represents one of the most (Arch Pathol Lab Med. 2011;135:1261–1268; doi: 10.
confusing and controversial areas in gastrointestinal 5858/arpa.2011-0034-RA)

P rimary appendiceal tumors are found in less than 2% of
surgically removed appendices, but still pose a variety
of diagnostic and therapeutic challenges. In particular, the
groups are usually familiar with some, but not all, of the ter-
minology used to describe mucinous tumors of the appendix
and peritoneum. As a result, management strategies across
classification and management of appendiceal mucinous different institutions, or even among clinicians at the same
neoplasms has proven problematic for both clinicians and institution, are not standardized. The purpose of this review
pathologists. These tumors characteristically cause cystic is to provide a comprehensive discussion of the biologic
dilatation of the appendix owing to accumulation of copious potential of, and proposed classification schemes for, appen-
gelatinous material in the lumen (Figure 1, A and B). They diceal mucinous neoplasms and pseudomyxoma peritonei.
may disseminate throughout the peritoneal cavity in the
form of gelatinous deposits, termed pseudomyxoma peritonei WHAT IS PSEUDOMYXOMA PERITONEI?
(Figure 1, C and D). Many controversies surrounding Pseudomyxoma peritonei is a term used to describe
appendiceal neoplasms and pseudomyxoma peritonei stem mucinous ascites or mucin deposits within the peritoneal
from the use of inconsistent histologic criteria for their cavity. Pseudomyxoma peritonei consists of organizing
diagnosis and philosophic differences of opinion regarding pools of mucin within peritoneal fat or on the serosal
their pathogenesis. Several investigators have proposed surfaces of the viscera, which contain variable numbers of
classification schemes that employ similar terminology to neoplastic epithelial cells. Most cases reflect dissemination
describe lesions of variable biologic potential or that use of an appendiceal mucinous neoplasm, in which case,
dissimilar terms to describe the same entity. Moreover, an mucin pools that contain scant strips and clusters of low-
origin in the appendix is not suspected before surgery grade neoplastic epithelial cells are typical (Figure 2, D),
in many cases because patients present with ovarian or similar to those present in the appendiceal mucosa
peritoneal tumors that are managed by gynecologists, rather (Figure 2, A and B). However, pseudomyxoma peritonei
than general or colorectal surgeons. These different clinical 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
Accepted for publication March 11, 2011.
cavity, and mucin-producing carcinomas of the colon,
From the Department of Pathology and Laboratory Medicine, Weill
Cornell Medical College, New York, New York. pancreas, and other organs may disseminate throughout
The authors have no relevant financial interest in the products or the peritoneum in the form of gelatinous ascites. Unfortu-
companies described in this article. nately, much of the earlier literature is of limited value in
Presented in part at the Tutorial on Pathology of the GI Tract, assessing the biologic potential of appendiceal mucinous
Pancreas, and Liver; Westin Diplomat Resort & Spa; November 15–19, neoplasms and pseudomyxoma peritonei. Most reports
2010; Hollywood, Florida.
Reprints: Rhonda K. Yantiss, MD, Weill Cornell Medical College, before 2000 did not distinguish between neoplastic
1300 York Ave, New York, NY 10065 (e-mail: rhy2001@med.cornell. and nonneoplastic etiologies for pseudomyxoma peri-
edu). tonei, appendiceal and nonappendiceal neoplasms, or
Arch Pathol Lab Med—Vol 135, October 2011 Mucinous Neoplasms of the Appendix and Peritoneum—Panarelli & Yantiss 1261

3 however.and high-grade tumors in the peritoneal were histologically similar. October 2011 Mucinous Neoplasms of the Appendix and Peritoneum—Panarelli & Yantiss . Prayson etal2 which showed identical KRAS mutations in codon 12 in evaluated a series of 10 men and 9 women with tumors from both sites. among tumors in 75% of cases. Mucinous neoplasms often cause appendiceal distention (A) because of massive accumulation of extracellular mucin within the lumen (B). Chuaqui et al6 analyzed loss of pseudomyxoma peritonei and found that 94% of patients heterozygosity on chromosomes 5 and 17 in synchronous had appendiceal mucinous neoplasms. they concluded that these communication with clinicians. leading them to conclude that pseudomyxoma tumors. 5 (83%) of derivedfrom either the ovary or peritoneum. whereas an ovarian ous ovarian tumors and noted that tumors in both sites origin is considered a rare occurrence. or right. The neoplasms may spread to the serosal surfaces of the viscera (C) or form gelatinous tumor deposits in the omentum (D). Young et al4 studied 22 most pseudomyxoma peritonei cases are derived from appendiceal mucinous neoplasms associated with mucin.Figure 1. ORIGIN OF PSEUDOMYXOMA PERITONEI Molecular data further support the notion that pseudo- The combined results of several studies published in myxoma peritonei is usually derived from the appendix. They found either loss of heterozygosity frequency of bilateral ovarian mucinous tumors. Seidman et al3 found a high from 12 patients. whereas unilateral myxoma peritonei as a diagnostic term and limit its inclusion ovarian tumors showed a right-sided predominance. theearly 1990s implicated the appendix as a likely site of rather than the ovary or peritoneum. Subsequent studies have shown women with concomitant mucinous tumors of the progressive loss of heterozygosity in ovarian mucinous appendix. in pathology reports to a comment aimed at facilitating Unlike Seidman et al. Cautrecasas et al5 origin in most of nearly 100 patients with pseudomyxoma analyzed KRAS mutational status in 6 synchronous peritonei but also suggested that some cases may be mucinous tumors of the appendix and ovary. leading most investigators to conclude that primary peritoneal neoplasm. including all of the appendiceal and ovarian mucinous neoplasms obtained women with ovarian tumors.1 In our practice. we generally avoid using pseudo.7. appendiceal mucinous neoplasms. cytologically low.8 1262 Arch Pathol Lab Med—Vol 135. when compared with synchronous appendiceal peritonei may be derived from either site or represent a neoplasms. at the same locus or no loss of heterozygosity in paired sided ovarian mucinous tumors in unilateral cases. features suggested a primary appendiceal origin with secondary ovarian involvement. They also found ovarian cavity. disease to be bilateral in most cases.

original magnifications 340 [A and C] and 3400 [B and D]). 2 patients with acellular periappen- PSEUDOMYXOMA PERITONEI) diceal mucin developed disseminated peritoneal disease. appendiceal mucinous tumors frequently lack infiltrating. Fifty patients (77%) flect differences of opinion regarding the fundamental had acellular extra-appendiceal mucin. They argue that appendix developed widespread peritoneal tumor depos. October 2011 Mucinous Neoplasms of the Appendix and Peritoneum—Panarelli & Yantiss 1263 . Peritoneal mucin deposits contain scant strips and clusters of mucin-containing epithelial cells (C) that are cytologically low grade. hyperchromatic nuclei that are basally located.Figure 2. A and B). Not uncommonly. These results emphasize the the appendiceal serosa. investigators think that peritoneal mucin deposits are At follow-up. similar to the neoplastic cells present in the appendix (D) (hematoxylin-eosin. Yantiss et al9 NEOPLASMS AND PSEUDOMYXOMA PERITONEI evaluated the prognostic implications of peritoneal mucin The proposed classification schemes for appendiceal localized to the right lower quadrant in 65 patients with mucinous neoplasms and pseudomyxoma peritonei re- appendiceal mucinous neoplasms. C and D). strips or clusters of neoplastic mucinous epithelial cells in periappendiceal mucin. and 15 (23%) had nature of mucin deposits in the peritoneum and compar- periappendiceal mucin deposits that contained scant isons between them are summarized in the Table. its. malignant glands and desmoplastic stroma and note that Arch Pathol Lab Med—Vol 135. 48 (96%) patients without extra-appendiceal the consequence of appendiceal rupture and spillage of epithelium were disease free (mean. whereas mucin and ‘‘adenomatous’’ epithelium into the peri- 33% of patients with any neoplastic epithelium outside the toneal cavity. rather than carcinoma. The tumor cells contain abundant cytoplasmic mucin and enlarged. with minimal cytologic atypia (B). surgical pathologists encounter but neither of those appendices was submitted entirely for appendiceal resection specimens with mucin deposits on histologic examination. 52 months). PERIAPPENDICEAL MUCIN DEPOSITS (LOCALIZED 38 months). similar to cases with widespread CLASSIFICATION OF APPENDICEAL MUCINOUS peritoneal disease (Figure 3. Some mucinous epithelium with low-grade cytologic features. or importance of adequate sampling in determining the limited to the right lower quadrant (Figure 3. including one patient who died of disease (mean. prognosis of patients with appendiceal mucinous neo- Extra-appendiceal mucin deposits limited to the periap. Notably. within the mesoappendix. although one may infrequently observe prognosis. plasms and indicate that tumors without extra-appendi- pendiceal area are usually devoid of neoplastic epithelial ceal neoplastic epithelium are associated with an excellent cells (acellular). Mucinous neoplasms typically display a circumferential growth pattern in the appendiceal mucosa with a variable papillary architecture (A).

Patients with overtly malignant 1264 Arch Pathol Lab Med—Vol 135. there are compelling reasons why the nant potential. 107 appendiceal mucinous tumors. but not infiltrative. whereas associated with progressive mucinous ascites. Adenomas were defined American Joint Committee on Cancer. up (median. 3400 [C]. some cancers. but occasional tumors are associated with extra- appendiceal neoplastic epithelium. and most other investigators consider mucosa with intact muscularis mucosae. 340 [B]. All In a study of 184 epithelial noncarcinoid appendiceal patients with low-grade neoplasms confined to the tumors. which they classified particularly mucinous or colloidal carcinomas may invade as either low. 6 years). so low-grade cytology nomas. mural invasion or acellular mucin pools on the serosa parenchymal invasion of solid organs is common in were considered to be of uncertain malignant potential.10. October 2011 Mucinous Neoplasms of the Appendix and Peritoneum—Panarelli & Yantiss . similar to that seen in patients with pseudomyxoma peritonei (D) (hematoxylin-eosin. tumors of uncertain malig- On the other hand. epithelium outside the appendix were considered carci- mal atypical cytologic features.12–14 were labeled as invasive mucinous adenocarcinomas.11 First. and carcinoma. peritoneal involvement experienced disease recurrence or ciated with decreased survival. The mucin deposits contain inflammatory cells and granulation tissue in most cases (C).or high-grade based on cytoarchitectural tissues with a broad. and 3600 [D]). These authors proposed death from disease.Figure 3. original magnifications 320 [A]. pushing growth pattern. whereas tumors pseudomyxoma peritonei to be carcinoma. Appendiceal mucinous neoplasms may show areas of mucin extrusion through the wall (A) or organizing mucin on the serosal surface (B). that appendiceal mucinous neoplasms be classified as logic features insufficient for a diagnosis of malignancy. some that showed mural invasion or proliferation of neoplastic carcinomas are extremely well differentiated with mini. Carr et al13 found that mucinous neoplasms appendiceal mucosa were free of disease at last follow- limited to the appendix pursued an indolent course. adenomas (or cystadenomas). and death in at least half of patients. whereas 67% of patients with whereas those with extra-appendiceal spread were asso. High-grade diagnosis of malignancy. disease peritoneal deposits containing overtly malignant cells recurrence. does not exclude a diagnosis of malignancy. Neoplasms with pushing. Tumors with low-grade cytology were termed not see infiltrating glands or desmoplasia to make a low-grade appendiceal mucinous neoplasms. extra-appendiceal epithelium usually shows bland cyto. Third. World Health by the presence of mucinous neoplasia confined to the Organization. patients with pseudomyxoma peritonei and is generally Misdraji et al15 later analyzed the histologic features of considered a feature of malignancy. lesions limited to the appendiceal mucosa were termed mucinous neoplasms that spread beyond the appendix are noninvasive mucinous cystadenocarcinomas. Finally and most important. Second. so one need features.

sion. the pathology report. colon.17 Low-grade tumors confined of disseminated peritoneal adenomucinosis were derived to the appendiceal mucosa were classified as adenomas. from the appendix and were associated with 5. All 65 cases scheme to a 4-tiered system. WHO. ance of peritoneal epithelium.and 10-year whereas similarly low-grade tumors with acellular extra.and Arch Pathol Lab Med—Vol 135.and high-grade features were designated classified as mucinous neoplasms of low malignant potential. Ronnett et al1 considered gested by Carr et al. High-grade tumors in the peritoneal cavity frequently derived from nonappendiceal primary tumors were classified as adenocarcinomas. peritoneal mucinous carcinomatosis. Mucin pools containing mucinous carcinomatosis (Figure 4. appendiceal epithelium (previously termed mucinous respectively. All patients predicted the presence or absence of pseudomyxoma with invasive carcinoma and who had available follow-up peritonei and suggested that all low-grade tumors be data died of disease with significantly decreased overall labeled low-grade appendiceal mucinous neoplasm.11 Pai and Ronnett et al. thus. compared with 100% and 86%. peritoneal disease had 3. not applicable. respectively.and 5-year survival rates of 90% proposal. similar to their prior and pursued an aggressive clinical course with 5. respectively.17 2009 1995 2006 WHO10. A and B). mucinous tumors of the peritoneum with more-abundant. which would be documented in extra-appendiceal disease.13 They recognized lesions confined to pseudomyxoma peritonei with low-grade cytologic fea- the appendiceal mucosa as adenomas but expanded the tures to represent passive spread of adenomatous definition of mucinous tumors of uncertain malignant epithelium into the peritoneum. The authors applied this classification to represent adenocarcinomas. among patients with low-grade peritoneal neoplasms of low malignant potential) recurred in 21 of 27 disease. survival rates of 75% and 68%.15 2003 Longacre. Both inter- appendiceal mucin were considered to be of low risk for mediate and high-grade mucinous carcinomas were more recurrence. or small intestine and diceal neoplasms and modified their original classification compared survival rates among the 3 groups. derived from the appendix. and those in which the diagnosis of extra-appendi. intermediate grade (Fig- whereas those with destructive invasion were considered ure 4. thus.12 AJCC and 2010 Misdraji et al. termed such cases disseminated perito- the proximal margin. those with mucin and epithelium in neal adenomucinosis (Figure 4. Cytologically low-grade tumors with extra- and 44%. Pai and Longacre16 proposed an pseudomyxoma peritonei based on the cytologic appear- alternative classification scheme that built on that sug. World Health Organization. Comparisons Among Classification Schemes for Appendiceal Mucinous Neoplasms and Pseudomyxoma Peritonei Source. C and D). were reclassified as low-grade features in the appendiceal component that reliably mucinous neoplasms with high-risk of recurrence. y Bradley Carr and Sobin. scheme to 109 multifocal peritoneal mucinous tumors This same group later analyzed 116 mucinous appen. low-grade epithelium outside the appendix were both low. E and F). Other authors have devised classification schemes for Two years later. American Joint Committee on Cancer. secondary to appendiceal potential to include those tumors with mucosal disease at rupture. They consider the appendiceal wall without obvious destructive inva. and. The authors failed to identify any pathologic cases (78%) and. and cases with scant. NA. cytologically malignant epithelium to represent peritoneal ceal epithelium was in doubt.11 2010 Tumor confined to appendix Limited to mucosa Low-grade Adenoma Low-grade Adenoma NA NA Adenoma cytology appendiceal mucinous neoplasm High-grade Adenoma Noninvasive mucinous Adenoma NA NA Adenoma cytology cystadenocarcinoma Positive Adenoma Low-grade Uncertain NA NA Adenoma surgical appendiceal malignant margin mucinous neoplasm potential Neoplastic Uncertain Low-grade Uncertain NA NA Invasive Mucinous epithelium malignant appendiceal malignant Adenocarcinoma in appendix potential mucinous neoplasm potential wall Tumor beyond appendix Low-grade Invasive mucinous Low-grade High-risk for Disseminated Low-grade Low-grade epithelium adenocarcinoma appendiceal recurrence peritoneal mucinous mucinous in peritoneal mucinous neoplasm adenomucinosis carcinoma adenocarcinoma mucin peritonei High-grade Invasive mucinous Invasive mucinous Invasive mucinous Peritoneal High-grade High-grade epithelium adenocarcinoma adenocarcinoma adenocarcinoma mucinous mucinous mucinous in peritoneal carcinomatosis carcinoma adenocarcinoma mucin peritonei Abbreviations: AJCC.1 et al. October 2011 Mucinous Neoplasms of the Appendix and Peritoneum—Panarelli & Yantiss 1265 . regardless survival rates compared with patients with low-grade of the extent of disease.

inclusion of intestinal carcinomas 3% for high-grade peritoneal mucinous carcinomatosis. grade is important in predicting behavior among perito- grade peritoneal mucinous carcinomatosis and 14% and neal mucinous tumors. and peritoneal mucinous carcinomatosis (E and F). 10-year survival rates of 50% and 21% for intermediate. 1266 Arch Pathol Lab Med—Vol 135. original magnifications 3100 [A]. peritoneal mucinous carcinomatosis–intermediate grade (C and D).1. but the degree of cytologic atypia exceeds that of disseminated peritoneal adenomucinosis (D) (hematoxylin-eosin. Mucin deposits in the peritoneal cavity classified as disseminated peritoneal adenomucinosis (A and B). in the analysis of high-grade tumors makes it difficult to respectively.14 Although these results suggest histologic interpret the data. 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 carcinomatosis–intermediate grade is less cellular than peritoneal mucinous carcinomatosis (C). October 2011 Mucinous Neoplasms of the Appendix and Peritoneum—Panarelli & Yantiss .Figure 4. 340 [C]. 3200 [D]. 3600 [B and F]. and 320 [E]).

1993. Chuaqui RF. and mucinous neo. They found that nei. The histologic grade of should also be informed of the remote possibility that a peritoneal disease is extremely important. Genetic analysis of synchronous mucinous tumors of the ovary and appendix. Shmookler mucinous adenocarcinomas and subcategorized as low. Hart WR. Young RH. Disseminated peritoneal adenomucinosis and peritoneal mucinous or high-grade because increasingly severe cytoarchitec. general principles. Petras RE. Pseudomyxoma peritonei: a clinicopath- assign tumor (T) stage for appendiceal mucinous neo. plasm at high-risk for recurrence.10. ment. BM. achieved 10-year survival had tumors of low-histologic grade mucinous carcinoma peritonei. Zahn CM. Matias-Guiu X.15(5):415–429. we predictable clinical course based on tumor stage and consider these tumors to be adenomas as well. Prognostic without various forms of chemotherapy. so adoption of classification and staging guidelines put forth by the a uniform reporting system for appendiceal mucinous American Joint Committee on Cancer. New York.33(2):248–255. Am nated peritoneal adenomucinosis. M1a. October 2011 Mucinous Neoplasms of the Appendix and Peritoneum—Panarelli & Yantiss 1267 . Outcome data from other studies using these that low-grade mucinous carcinoma peritonei was asso. intraoperative intraperitoneal chemother- cytologic grade in predicting behavior of pseudomyxoma apy. TREATMENT OF PSEUDOMYXOMA PERITONEI mucinous tumors including Krukenberg tumor. Am J Surg Pathol. Am J ment and extra-appendiceal disease limited to the right Surg Pathol. whereas any proliferation of neoplastic completely submitted for histologic evaluation to exclude epithelium beyond the mucosa places the patient at risk the possibility of extra-appendiceal epithelium. et al. Misdraji J.11 neoplasms with peritoneal metastases by the World The World Health Organization regards any neoplastic Health Organization and American Joint Committee on epithelial proliferation confined to the appendiceal mu. Trotti A. Edge SB. 2006. Shia J. it is appropriate to classify them as adenomas or cystadenomas. consisting of a combination of chemotherapy and diceal mucin and thus. Low. whereas disease-free survival and that 90% of patients who cases with severe cytologic atypia were classified as high. Gilks CB. regardless grade peritoneal disease.13(5):205–227. Seidman JD. Any proliferation of neoplastic epithelium tumors probably do not benefit from aggressive debulking beyond the muscularis mucosae is at risk for peritoneal but may be better served by systemic chemotherapy. 1995. of the degree of dysplasia. clinical follow-up should probably cytoreductive surgery. Cancer represents a major advancement in the field. SUMMARY AND CONCLUSIONS ceal mucinous neoplasms associated with acellular mucin Appendiceal mucinous neoplasms represent a relative- limited to the appendiceal wall and/or mesoappendix are ly homogeneous group of neoplasms that pursue a also cured by excision. dissemination and should be graded and staged as a Decisions regarding clinical management require clear carcinoma. Hahn HP. should probably be 6. tumors of the ovary and appendix: a clinicopathologic study of 25 cases. 1996. lower quadrant. 2010:133–141. J Surg Pathol. thus. Kurman RJ. Those confined to the appendiceal mucosa are all neoplasms that are classified as adenomas should be cured by excision. Appendi. have seen a trend toward more aggressive management 10. Hum Pathol. colleagues. Tavassoli FA. ease compared with survival rates of those with high- The combined results of all of these studies can be grade tumors. therefore. ologic study of 19 cases with emphasis on site of origin and nature of associated ovarian tumors. and extent of treatment in 97 patients who in a similar fashion at a single institution. pathologic study of six cases with comparative analysis of c-Ki-ras mutations. part I: historical perspective. complete cytoreduction in 53% of cases. aggressive treatment strategies have also shown improved ciated with a significantly better 5-year survival rate (63%) survival among patients with low-grade peritoneal dis- than was high-grade mucinous carcinoma peritonei (38%). Greene FL. carcinomatosis: a clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features. Yantiss RK. Young R. whereas those with high-grade be considered. These authors evaluated the Miner et al18 retrospectively analyzed the relative contri- natural history of 101 patients with appendiceal mucinous bution of histologic features of peritoneal disease.18:591–603. 7. Mucinous tumors of the appendix associated with mucinous tumors of the ovary and pseudomyxoma peritonei: a lower quadrant (pseudomyxoma peritonei) are consid. in most cases and. consisted of surgical debulking of gross disease. Odze RD. Semin Diagn Pathol. clinicopathologic analysis of 22 cases supporting an origin in the appendix. Bradley et al12 better demonstrated the importance of peritonectomy. plasms similar to those used for colonic adenocarcinoma. which are probably not amenable to summarized into several major points. peritonei in a later study. Fritz AG. therapeutic decisions limited to the appendiceal mucosa have no potential for largely rest on the distinction between low-and high- aggressive disease when completely resected. grade.10 Confusing terminology. Zhuang Z. 1996. complete Cancer Staging Manual. cosa as an adenoma. 1994(6).21(2):134–150. Sugarbaker PH. Prat J. Association of mucinous with the exception that T4a denotes both serosal involve. American Joint Committee on Cancer staging guidelines 2. AJCC strategies that include multiple surgeries. Scully RE. tumors surgical management. Pseudomyxoma peritonei and selected other aspects of the Treatment of pseudomyxoma peritonei has historically spread of appendiceal neoplasms. patient tumors and peritoneal disease. 5. site of origin. Emmert-Buck MR. First. Synchronous mucinous tumors of the appendix and the ovary associated with pseudomyxoma peritonei: a clinico- diceal mucinous neoplasm in the peritoneum. Arch Pathol Lab Med—Vol 135. Prayson RA.17(1):22–34. NY: Springer. Byrd DR. From Krukenberg to today—the ever present problems posed by metastatic tumors of the ovary. Young RH. which achieved used a 2-tiered system to classify pseudomyxoma perito.11 The ‘‘pseudomyxoma peritonei. Cautrecasas M. The authors found grade. 2004.’’ Am J Surg Pathol. as described in the World Health Organization communication among treating physicians. Sobin L. such as low-grade appen. Am J Surg Pathol. all of whom were treated characteristics. These authors underwent extensive debulking surgery. 1991. with or 9.19(12):1390–1408. However. Elsayed AM. Recent years significance of localized extra-appendiceal mucin deposition in appendiceal mucinous neoplasms. 8. Clinicians for peritoneal dissemination. avoided to facilitate clear communication with our clinical 27(2):165–171.20(6):739–746. Adv Anat Pathol. eds. Ronnett BM. and relationship to tural atypia is associated with poorer outcome. Am J ered to represent metastatic disease and are denoted as Surg Pathol. prognosis. and. and additional cycles of postoperative chemotherapy. Appendiceal mucinous tumors with References extra-appendiceal neoplastic epithelium are classified as 1. 2009. 3. Kass ME. 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