Appendicular Mucinous Neoplasms are rare pathologies of the appendix that can lead to rupture and the development of Pseudomyxoma Peritonei. In this lecture we present the difference between 4 cases of appendicular Mucinous Neoplasm in which the proper management and early surgical intervention prevented the development of Pseudomyxoma Peritonei

1. Dr. Majd AlHaddadin, MBChB,MS,MRCS
Consultant General & Laparoscopic Surgeon
Al Hammadi Hospital – Riyadh – Nuzha
- Jordanian Board and Arab Board in General Surgery.
- Member of The Spanish Association of Surgery (AEC).
- Member of The Royal College of Surgeons – Ireland
(MRCSI).
- Associate Fellow of the American College of Surgeon
(AFACS).

2. Pseudomyxoma Peritonei
An extremely rare pathology
Is it curative ?
Is it preventable ?
Dr. Majd AlHaddadin, MBChB,MS,MRCS,AFACS.
Consultant General & Laparoscopic Surgery
Al Hammadi Hospital - Nuzha

3. • Although neoplasms of the appendix are rare, they carry a significant disease burden.
Upwards of 58% of malignant tumors arising from the appendix are mucinous in origin.
• When these mucinous tumors are able to penetrate through the appendicular wall and
spread into the peritoneal cavity in the form of gelatinous deposits, the condition is termed
pseudomyxoma peritonei (PMP).
• The incidence of PMP is believed to be approximately 1-3 out of a million.
• Nonetheless, due to its indolent nature, it is usually discovered at an advanced stage and
severely impacts quality of life.

4. • Currently, it has been more widely used to describe peritoneal dissemination of mucus-
producing neoplasm most frequently from the appendix.
• But also, small and large bowel, stomach, pancreas, gallbladder, fallopian tubes, and ovaries
can produce PMP.

5.  The pathological process of PMP starts similar to most primary tumors of the alimentary
tract.
 Neoplastic transformation of the goblet cells results in the formation of a primary mucinous
tumor.
 As mucin levels increase within the mucocele, there is eventual rupture.
 The rupturing of the mucocele is the initiating event that leads to the development of PMP.
 The rupture allows tumor cells to access the peritoneal cavity, and given their lack of cell
adhesion molecules, they passively circulate with ease. It is termed as the so-called
“redistribution phenomenon”.
 This seeding of tumor cells eventually leads to bulky mucinous deposits all along the
abdominal cavity.

6.  Mobile organs such as the small bowel and its mesentery are usually less likely to be involved
compared to more fixed parts such as antrum, duodenojejunal flexure, ligament of Treitz,
ileocecal and rectosigmoid region, early in the disease process.
 But in the end-stage disease, the entire peritoneal cavity could be engulfed, The implanted
tumor cells continue to proliferate and produce a large amount of mucus and eventually
form mucinous ascites over months or even years.
 The excessive tumor burden and subsequent high intra-abdominal pressure can further limit
bowel movement and even result in small bowel obstruction that requires surgical resection.
 The extra-peritoneal invasion has been reported mainly in the pleural cavity.
 Lymphatic or hematogenic metastasis are rare occurrences.

7.  Localized disease:
- Typically presents with appendicitis-like
symptoms or a pelvic mass due to
mucinous deposits on adjacent organs.
 More advanced disease:
- Presents with abdominal distension,
bowel obstruction and ascites.
 The classic sign termed “jelly belly”
- Is an increase in abdominal girth caused
by an accumulation of gelatinous ascites.

8. Appendiceal Mucinous Neoplasms (AMNs)
• Are rare tumors accounting for less than 1% of all cancers.
• Are present in 0.2%–0.3% of appendectomy specimens.

10.  Disseminated peritoneal adenomucinosis ( DPAM) WHO Class : Low grade mucinous carcinoma
 Peritoneal mucinous carcinomatosis, (PMCA) WHO Class: High grade mucinous carcinoma
 Cases with intermediate features WHO Class: Mucinous carcinoma with signet ring cells
Pseudomyxoma peritonei (PMP) is the diffuse collections of gelatinous material in the abdomen and pelvis,
and mucinous implants on the peritoneal surfaces. Therefore, it has been recommended that the term PMP
be limited to describing the clinical entity of mucinous ascites and not be used as a histologic diagnosis.

11. Appendiceal mucinous neoplasms (AMNs)
Epidemiology and Characteristics…
• The incidence of AMN in the U.S. has increased over time, from 0.6 cases per million persons in 1973 to 2.8 cases per
million persons in 2011, with an annual percentage increase of 3.1%.
In addition, age at diagnosis has decreased over the same time period…USA
A similar pattern of increase in incidence and decrease in age at diagnosis was observed in a large registry study from
the Netherlands spanning 1980–2010
• Peritoneal involvement is the presenting stage for the majority (53.2%) of patients with AMN (localized [26.3%]
and regional [20.5%] stages).
There seems to be a trend toward a decrease in regional disease stage over time, while distant stage disease is increasing
in incidence

12. Treatment Options and Controversies
Localized AMNs:
o Because the incidence of nodal spread of well‐differentiated localized appendiceal tumors is less than 2%, most of the published
surgical literature suggests that simple appendectomy is sufficient for tumors exhibiting only local disease.
o Right Hemicolectomy:
- margin involvement after appendectomy.
- tumor size of 2 cm or larger.
- high grade histology.
- Tumor that invades through the muscularis propria.
- involvement of the base of the appendix.
- lymph node involment.
- Perforation.

13. Treatment options and Controversies
 AMN with Peritoneal Metastasis
Evolution:
- Historically, treatment consisted of repeated drainage of the mucinous ascites, and evolved to serial debulking surgeries.
- In the 1980s, Spratt et al. demonstrated that the addition of intraperitoneal chemotherapy to traditional debulking surgery improved disease control duration.
- In the 1990s, Sugarbaker introduced the concept of a one‐stage aggressive cytoreductive debulking procedure via several macroscopic peritonectomies, followed by
intraoperative HIPEC infusion.
Complete Cytoreduction CR-0
and HIPEC
5‐year survival
rates of 86% for
DPAM
5‐year survival
rates of 50% for
aggressive PMCA

14. PCI = 20
<

17. A
A

18. MRI Pelvis

19. CT-Scan Abdomino-pelvic

20. TNM STAGE: pT2, pN0, pMx

22. LAMN – Negative
Margins and No rupture

23.  Appendiceal mucinous neoplasms are a heterogeneous group of tumors
with a worrying-rising incidence.
 Early diagnosis and appropriate surgical management is the corner stone of
the patient evolution.
 Major resection including hemicolectomy should be kept in mind and to be
performed whenever it is indicated without hesitation.
 Cyto-reduction surgery and HIPEC is the main treatment in patients with
advanced stage disease.

24. Thank You…