pseudomyxoma peritoni

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Pseudomyxoma peritonei Review of thedisease

• Shafquat Ali Abbasi

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• Abdomen is called a Pandora's box.
•   "to open Pandora's box"
• means to perform an action that may seem small
  or innocent, but that turns out to have severely
  detrimental and far-reaching consequences.

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What is Pseudomyxoma Peritonei?

• Pseudomyxoma peritonei (PMP) is the
  debilitating
• syndrome of grossly apparent gelatinous ascites
  
• associated with peritoneal deposits of
  mucinous
• tumours.
• Mainly previously attributed to mucinous
  neoplasms arising in the ovary.
• Most of this syndrome is related to
  discontinuous
• spread from the appendix.

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Clinical presentation

• The most important symptom is increasing
  abdominal girth (50)
• Median age at presentation is about 50 years.
• There is no obvious sex predilection/some studies
  showing females predilection.
• The suspected acute appendicitis, increasing
  abdominal girth, or an umbilical or inguinal
  hernia

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Pathophysiology

• Gelatinous ascites deposits of PMP have a
  characteristic distribution, relatively sparing
  the small bowel serosa and concentrating on the
  right hemidiaphragm, retrohepatic space, ligament
  of Treitz, greater omentum.
• Tends to localize away from peristaltic activity
  collect where peritoneal fluid is re-absorbed
  encase the splenic capsule and liver. ovaries
  are often diffusely involved (pseudomyxoma
  ovarii).

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Pathophysiology

• Chronic increases in intra-abdominal volume, as
  in morbidly obese patients, lead to a slower
  increase in IAP as the abdominal wall
  accommodates and becomes more compliant with
  time, the phenomenon of stress-relaxation.
• With this gradual increase, the various organ
  systems are able to compensate for the changes in
  IAP. Consequently, the acute deterioration seen
  with Abdominal CS does not occur in these
  patients
• Usually first discovered at laparotomy or
  laparoscopy in developed countries.

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Objective and subjective

• 63 ,Male
• Progressively increasing abdominal size 2 months
• Hiccup and occasional vomiting
• Lack of sleep
• No previous medical illness
• No history of jaundice/Dyspepsia
• No previous surgery /blood transfusion/IV
  drugs/promiscuity
• No weight loss/change of bowel habit
• No shortness of breath

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Assessment

• Vital signs stable
• No jaundice /Lymphadenopathy
• No shortness of breath
• Gross Ascitis
• Positive fluid thrill
• Abdominal Girth

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Physical Manifestation
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Provisional diagnosis

• Ascitis secondary to chronic liver disease

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Hospital course

• Labs
• Chest Xray
• CT scan abomen
• Upper GI Endoscopy
• Peritoneal tap

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to
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Ronnett et al pathological classification

• DPAM disseminated peritonealadenomucinosis
• (extra cellular mucin with few benign looking
  epithelial cells)
• PMCAperitoneal mucinous carcinomatosis
• (abundant mucinous epithelium with cytological
  feature of carcinoma)
• PMCA-I/DPMCA with intermediate or discordant
  features(focal areas of carcinomas in peritoneal
  lesions)

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Pathology

• Practical issues remain regarding nomenclature.
• Most had agreed on the appendix as the primary
  culprit for PMP(50-55)may also complicate
  mucocele.
• Regardless of how well differentiated they are,
  all cases of PMP are examples of mucinous
  carcinoma.
• Grading as either low- or high-grade carcinoma is
  indicated for prognostication.

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Establishment of diagnosis

• CT Scan typical appearance main stay of
  diagnosis.
• MRIfat suppressed godalium-enhanced breath-hold.
• PET
• Radio immunoscitography
• Immunochemistry
• Tumour markersCEA,CA 19.9
• mainly for post op follow up but pre-op
  benchmark

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Treatment options

• Non surgical periodic draiage,peritoneal wash
  with 5 dextrose systemic chemotherapy alone
• Surgical options traditional treatment repeated
  debulking with ovariectomy/appendicectomy
• Recurrences imminent and usual course on 3rd
  recuurence with intestinal obstuction leading
  stoma for 4th or 5th recurrence death through
  obstruction and complications of treatment.

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Sugarbaker procedure

• Sugarbaker procedure is performed about 1,000
  times a year at more than 100 medical centers
  and is named after this 71-year-old blue-eyed
  surgeon, who has dedicated an entire career to
  pioneering, honing and promoting this treatment,
  which has left him with a permanent stoop from
  having spent so much time hunched over patients.
  He performs about 70 such operations each year on
  cancers that have spread into the abdomen from
  places such as the appendix and colon.

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Treatment Aimprevention of recurrence

• Not only macroscopic but also microscopic
  complete cytoreduction CRS(Cytoreductive surgery)
  is the only hope
• HIPEC(Hyperthermic Intraperitoneal Chemotherapy)
  is essential in these patients to avoid
  recurrences
• microscopic to minimal macroscopic tumor residue
• cytotoxic effect up to a tumor depth of 2-5 mm.
• Monoclonal antibodies Bevacizumab

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Controversies

• Quality improvement major concern-if most patient
  are not expected to benefit from knowledge ground
  it is research only and not recommended
• If secondary procedures required still it is
  research
• CRS with HIPEC published results are
  generalizable
• Selection of patients
• PCI (peritoneal carcinomatosis index)
• judged before cytoreductive surgery also on good
  PET/CT scan)
• numeric score 0-39
• less than 20 median survival 119 months
• more than 20 median survival 39 m0nths

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CRS-HIPEC Rationale Procedure

• Peritoneal cavity is exposed to higher
  concentration
• Penetration of drug to 2-5 mm
• Hypper thermia is cytotoxic also increase drug
  toxicity
• MacroMicroscopic complete cytoreduction
• Resection of involved viscerae hyperthermic
  intraperitoneal chemotherapy(HIPEC) co regional
  approach.mitomycin C5FU heated to 42 c(30 to 120
  min)
• IV therapyEarly post op chemo with 5FU and other
  drugs
• Monoclonal antibodies Bevcizumab in colon cancer

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peritoneum (1) and colon (2).
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Low-grade mucinous carcinoma peritonei (MCP) with
a broad front of neoplastic invasion and abundant
extracellular mucin.
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Well differentiatedmucinous adenocarcinoma.
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• Mayo clinic(26 patients) 53 5 year survival end
  of study 3 disease free
• Dutch trial (500 patients)2 year survival in
  HIPEC group 43 while 16 in control group.
  probability of survival at 10 years 37
• 5-year and 10-year survival for patients with
  DPAM (75, 68) were significantly better than
  for patients with either PMCA (14, 3) or
  PMCA-I/D (50, 21). However, some cases of
  PMCA-I/D may have been more appropriately
  assigned to the PMCA group.
• Evidence of actual lymphovascular invasion and/or
  extra-abdominal spread is not commonly
  documented.

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Conclusions

• Pseudomyxoma peritonei (PMP) is a rare disease,
  with a
• grim prognosis when not treated properly. The
  first step
• to improve the prognosis is to recognize this
  clinical syndrome.
• Preferably in an early stage. Knowledge of
  pathogenesis
• and common diagnostic tools is essential in this
  regard.
• CT imaging should be the choice of radiological
  assistance in the diagnostic process.

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Conclusions

• Surgical debulking is the standard treatment for
  PMP, combined modality treatment, consisting of
  aggressive peritonectomy with (intraoperative)
  HIPEC, seems to win ground as the new standard
  approach.
• Centralization of patients in centers that treat
  these patients on a regular basis is fundamental
  to prevent high morbidity and mortality.

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Peritonectomy and HIPEC for
pseudomyxoma peritonei

• http//www.youtube.com/watch?vg7A08VLBvls

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References

• R.F. Bradley, G. Cortina, K.R. Geisinger,
  Pseudomyxoma peritonei Review of the
  controversy, Current Diagnostic Pathology, Volume
  13, Issue 5, October 2007, Pages 410-416, ISSN
  0968-6053.\
• R.M. Smeenk, V.J. Verwaal, F.A.N. Zoetmulder,
  Pseudomyxoma peritonei, Cancer Treatment Reviews,
  Volume 33, Issue 2, April 2007, Pages 138-145,
  ISSN 0305-7372
• Mohamed, F., Cecil, T., Moran, B., Sugarbaker,
  P. (2011). A new standard of care for the
  management of peritoneal surface
  malignancy. Current Oncology, 18(2), e84-e96.
  doi10.3747/co.v18i2.663

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Questions?