1. Pseudomyxoma Peritonei: case presentation
Kapesh kunwar
2nd year resident Depatment obstetrics and gynecology
TUTH /03/13(27th june 2014

2. Introduction
Pseudomyxoma peritonei (PMP) is a clinical syndrome characterized by copious amounts of mucinous ascites and mucinous peritoneal implants.
Intra-abdominal transformation of the peritoneal mesothelium to a mucin-secreting epithelium secrete mucus, with gradual accumulation in the peritoneal cavity of huge amounts of gelatinous material, constituting the so-called pseudomyxoma peritonei
Clinical gynecologic oncology ,pg ,P.Disaia

3. Introduction contd…..
The incidence- one per million per year and encountered in 2/ laparotomies .
75% of patients female with average age of 53 years.
The most common sites of origin - appendix, colon and ovary .
Rarely from the lung, fallopian tube, uterus, common bile duct, pancreas and stomach .
44% of women with PMP have ovarian mucinous tumor.

4. Case IP no. 479888 Date of admission:2071/01/02
Mrs. Khadka, 45yrs, P₅+₁, illiterate, Hindu housewife from Bajhang, admitted from emergency
Chief complains:
Abdominal distension since five months
lower abdomen discomfort since five months

5. Case Contd…. History of present illness
developed abdominal distension since five months,
insidious in onset ,gradually and progressively increasing,
associated with discomfort on lying supine and eating food and
associated with decreased appetite and weight loss.
No/H/O , nausea, vomiting, PV discharge and bleeding.
Normal bladder and bowel habit.

6. Case Contd…. Menstrual History Obstetrics History 1st : Male 25yrs
K-16 yrs, irregular cycle3-7/30-90 days
LMP in mangsir after 10months of ammenorrhoea .
No history of intermenstrual bleeding, postcoital bleeding or dyspareunia
Obstetrics History
P₅+₁ L₄1st :
1st : Male 25yrs
2nd : Male 23 yrs
3rd: Female 21 yrs
4th : Male 18 yrs
5th baby died of pneumonia at the age of 2 years
All vaginal deliveries home deliveries.

7. Case Contd…. History of past illness: Non significant
Family history: not significant
Personal history: not significant
Drug history: not significant

8. Case Contd…. On examination: GC –Fair
Pallor - , icterus – , dehydration +,
no lympnododes
BP-100/60mmhg, P-78bpm,afebrile
Chest and CVS within normal limits

9. Case Contd…. P/A: soft, grossly distended shifing dullness +
fluid thrill +
No organomegaly
no mass palpable.

10. Case Contd…. P/S- Dirty dicharge +, os- parous and looks healthy.
P/V: cervix firm ,
uterus and adnexa not appreciable
all fonices were full,
non tender

11. Case Contd…. Investigations.
HB:11.9gm%
TLC 6400/cmm
Platelets:2,65,000/cumm
PT:15sec con.12
INR:1.05
Na:138 meq/l
K:4 meq/l
RBS:5.2 mmol/l
Urea:2.6 mmol/l
Creatinine: 72 µmol/l
Bilirubin
T:10µmol/l, D:4µmoml/l,
Protein: 71 g/l
Albumin:41g/l
SGOT:26U/l
SGPT:16 U/l
ALP:68U/l
CA-125=20.8U/ml
CEA=44.5ng/ml(<3ng/ml)
Serum beta HCG=<2.39mIU/ml
Serum LDH=434U/l

12. Case Contd…. Investigations.
USG-No obivious mass noted,Mucinous type ascitis aspiration not successful.
CT scan:3.7*3.5*3.2 cm hypoechoic mass noted with in the pelvis right side. No solid enhancing component noted. Ascitis present.
Chest x-ray-Normal

13. Case Contd….
With provisional diagnosis of malignant right ovarian tumor with ascitis ,she underwent staging laparotomy with TAH BSO with omental biopsy.( )
Per operative finding :
Peritoneal cavity filled with about 2500 ml of mucinous gelatinous material.
Right ovary enlarged to15*15 cm size irregular .
No deposits in intestine and peritoneum, liver surface.

14. Case contd…. Cut section-
15*15 cm irregular multicystic area containing mucinous gelatinous substance with one cyst measuring 3*3 cm containing cheesy material with tuft of hair noted.
Her post operative period was uneventful and discharged on 5th postoperative period.

15. Why are we discussing this case ?
Pseudomyxoma peritonei is a rare and poorly understood condition in which mucus accumulates within the peritoneal cavity .
Diagnosis prior to surgery often delayed and inaccurate.
Diagnosis was made after laparotomy in our case. .
Management primarily surgical along with appedicectomy .

16. Disscussion
Pseudomyxoma peritonei (PMP) is a clinical syndrome characterized by copious amounts of mucinous ascites and mucinous peritoneal implants.
It was first described by ROKITANSKY in
WERTH, in 1884, observed an association with an ovarian mucinous tumour and gave the name PMP .
In 1901 FRANCKEL reported the association with an appendiceal cystic tumor .

17. Discussion Grossly - Pseudomyxoma peritonei is characterized
by copious amounts of mucinous ascitis and mucinous peritoneal implants .
In our case -Peritoneal cavity filled with about 2500 ml of mucinous gelatinous material.
Right ovary enlarged to15*15 cm size irregular
No deposits in intestine and peritoneum, liver surface.

18. classification
Disseminated peritoneal adenomucinosis(DPAM)- Classic PMP with paucicellular mucinous ascites remaining localized to the abdomen without metastatic behaviour
Peritoneal mucinous carcinomatosis (PMCA) :PMCA has a higher percentage of malignant cells and a poorer prognosis .It behaves like a mucinous (colloid) carcinoma with metastatic and invasive potential.
Ronnett and colleagues

19. Clinical features Increasing abdominal girth (40%),
Bilateral or unilateral ovarian tumors (20%),
Mucinous ovarian tumors bilateral (80% ).When unilateral, there is a right-sided predominance.
Hernia sac tumors (20%),
Appendicitis-like syndrome (10%),
Infertility (10%)
Intestinal obstruction (rare)
Localizes initially in the right lower quadrant and then becomes more generalized with mucinous peritoneal, serosal, and omental implants.

20. Histopathology Histopathology Case
DPAM- pools of mucin with mucinous epithelial cells without significant cytologic atypia or mitotic activity and without invasion of tissues.
PMCA-pools of mucin contain mucinous epithelial cells showing moderate to marked cytologic atypia ,significant mitotic activity and invasive growth pattern .
Sections from the right ovary shows multiple cysts lined by mucinous epithelium with absence of atypia and infiltration into underlying stroma. Section from omentum shows fatty tissue with mucin.

21. Immunohistochemistry
The ovarian mucinous tumors associated with PMP display diffusely positive staining for cytokeratin 20 and negative staining for cytokeratin 7
In our case we could not test cytokeratin 20 and cytokeratin 7

22. Imaging
Case
USG -Immobile ascites. Gelatinous masses can mark the hepatic and splenic margins (“scalloping”)by extrinsic pressure .
No obivious mass noted,Mucinous type ascitis aspiration not successful

23. CT-scan
A CT-scan is the best exam to make the diagnosis and stage PMP. The mucinous material has a similar density to fat and has a heterogeneous aspect . Sometimes, progressive punctuate calcification can be found in the abdomen.
CT scan:3.7*3.5*3.2 cm hypoechoic mass noted with in the pelvis right side. No solid enhancing component noted. Ascitis present.

24. Tumour markers
The utility of tumour markers in PMP has not been largely studied. CEA and CA 19.9 can be frequently elevated in PMP . Preoperative CEA levels are useful in predicting the extent of disease and surgical success and survival in patients with PMP.
CEA=44.5ng/ml(<3ng/ml)
Almost 15 fold increase in CEA level.

25. Ascitic: cytology
The tapping of the atypical ascites is often unsuccessful.
Most of the time, only low quantities of mucus can be obtained with a few cells .
These cells often have benign features.
The lack of malignant cells in the ascitis does not exclude carcinomatous process
In our case ascitic aspiration was unsuccessful.

26. Management
Treatment remains primarily surgical, and because of the recurrent nature of the lesion, it may be repetitive.
Debulking surgery-treatment of choice for PMP . Most of the mucinous material has to be removed but no excessive surgical risks are taken.
An appendectomy is systematically performed, with histological examination.

27. Management contd…
Prolong remission or cure can be achieved by complete cyto -reduction with perioperative intra peritoneal chemotheray.
Recurrences are frequent and more than one operation is often necessary. They are more and more difficult because of adhesions.
In our patient staging laparotomy with TAH with BSO with omental biopsy done.

28. Management Chemotherapy- The role of chemotherapy yet to be defined.
Commonly used agents –
5- FU ,
cyclophosphamide ,
mytomycin C and
cisplatin.

29. Hyperthermic intraperitoneal chemotherapy (HIPC) is better than systemic chemotherapy due to :
Most of the time, the tumors are of low grade, with a low rate of proliferation.
The cells bathing in an abundant extracellular mucinous matrix have poor vascularization.

30. Radiotherapy
External irradiation was previously used for PMP but the results were disappointing and the abdominal morbidity was intolerable .
Mucolytic agents
Several authors advocated the use of mucolytic agents, which could allow easy drainage of the mucinous material during a palliative operation
Intraoperative irrigation or percutaneous lavage with dextrose expidites the removal of mucin and prevents the reaccumulation.

31. Recurrence PMP has high recurrence rate.
PMP can occur years (ranging from 5 to 35 years) after the initial presentation .
10% of patients die of PMP within 5.5 years of their initial presentation.
Overall survival of patients is about 75% and 68% for 5 years and 10 years, respectively.

32. Follow Up
A baseline CT scan after 3 month postoperatively, then 6 monthly for detection of recurrence or surveillance of progressive disease.
Baseline elevated tumor markers CEA, CA 19.9 and CA 125 may indicate an increased risk of recurrent disease in patients.

33. Case reports
A 39 year old woman presented with a three month history of abdominal distension and an episode of abnormal per vaginal bleeding.
Clinical examination revealed a mass arising out of the pelvis and ultrasound examination demonstrated a large multicystic complex mass containing fluid and solid components, arising from the left ovary.
The tumour marker CA125 was normal .
At laparotomy, copious amounts of free mucin and tenacious mucinous deposits were seen on the surface of the omentum and bowel. There was a 10 × 12 cm left ovarian mass and a small right ovarian cyst.

34. Case report contd…
Peritoneal lavage, hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and appendicectomy were performed.
Nine months after surgery, an abdominal computed tomography scan shows no evidence of PMP and tumour markers such as carcinoembryonic antigen, CA125, and CA19-9 are within normal limits.

35. CASE REPORT
A 60-year-old female presented Dec 2007 with post-op complaints of diffuse abdominal pain and distension of the abdomen.
She had a surgical history of right ovarian cyst salpingoopherectomy , left ovarian cyst excision and appendecectomy 2 mths back.
All her investigations were WNL.
Gross and histological examination suggested invasive well differentiated mucinous adenocarcinoma appendix with features of Pseudomyxoma peritonei.

36. CASE REPORT
A 39 y ear old woman presented with a three month history of abdominal distension and an episode of abnormal per vaginal bleeding.
Clinically a mass arising out of the pelvis and ultrasound examination - a large multicystic complex mass containing fluid and solid components, arising f rom the lef t ov ary . The tumour marker CA125 was normal .
At laparotomy , copious amounts of f ree mucin and tenacious mucinous
deposits on the surf ace of the omentum and bowel. There was a 10 × 12 cm left ovarian mass and a small right ovarian cyst.

37. CASE REPORT
Peritoneal lavage,hysterectomy , bilateral salpingo-oophorectomy , omentectomy , and appendicectomy performed.
Nine months after surgery , an abdominal computed tomography scan shows no evidence of PMP and tumour markers such as carcinoembry onic antigen, CA125, and CA19-9 are within normal limits.

38. Take home messages
Pseudomyxoma peritonei (PMP) - copious amounts of mucinous ascites and mucinous peritoneal implants.
Commonly diagnosed during laparotomy, performed for other indications like apedicitis,malignant ovarian tumor.
The most common sites of origin of PMP are tumors of the appendix, colon and ovary .
Increasing abdominal girth is the most common symptom.

39. Take home messages
Treatment is primarily surgical, and because of the recurrent nature of the lesion, it may be repetitive.
Standard procedure is to perform an appendectomy routinely during the staging of ovarian neoplasms .
The pathological, histological, and tumor marker characteristics of our patient make it difficult to classify this tumor into a distinct category ( disseminated peritoneal adenomucinosis or peritoneal mucinous carcinomatosis) and advise the patient on the appropriate treatment

40. References
Clinical Gynecologic Oncology 7th Edition,2007, DiSaia creasman
Berek and Novak’s Gynaecology, 14th edition, gestational trophoblastic disease, page no 1549
Jeffcoate’s Principles of gynaecology, 7th edition gestational trophoblastic disease
Patient’s file
D C Dutta text book of gynecology
PSEUDOMYXOMA PERITONEI AND MUCINOUS OVARIAN TUMORS
Biljana Djordjevic, Simonida Stojanovic, Nadica Ljubenovic and Ivana Djordjevic Institute of Pathology, Faculty of Medicine, University of Nis Faculty of Medicine, University of Nis Military Hospital in Nis

41. Thank You