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Pseudomyxoma Peritonei with ProgressiveAbdominal DistentionNattha Pipopchaiyasit, M.D.*

Saipin Tangkaratt, M.D.†

Anantnuch Sakapiboonnan, M.D.‡*Division of Medical Oncology, National Cancer Institute of Thailand†Division of Radiation Oncology, National Cancer Institute of Thailand‡Division of Pathology, National Cancer Institute of Thailand

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Abstract Pseudomyxoma peritonei is a rare condition, and poorly understood which characterized bymucinous implants diffusely involving the peritoneal surfaces. Most cases originate from ruptured appendicealmucoceles or ovarian cysts. This report describes a case of massive gelatinous ascites which was diagnosed withpseudomyxoma peritonei. The diagnosis processes need multidisciplinary approaches to use. In addition,literature on the clinical presentation, diagnostic procedures, and treatment options have been brieflyreviewed.

Key words: pseudomyxoma peritonei, gelatinous ascites

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Introduction

Pseudomyxoma peritonei, a progressive dis-ease within the peritoneum, is characterized by theproduction of large amounts of mucinous fluid thatgradually fills the peritoneal cavity, resulting in thecharacteristic çjelly bellyé1, 2. The incidence isapproximately one per million per year or 2 per10,000 laparotomies, and two to three times morecommon in female than males3.

There is a considerable debate regarding theappropriate classification and type of tumor to beincluded in this syndrome. The main controversyis related to the inclusion or exclusion of the moreçmalignanté forms of the disease and to the site oforigin of the tumor4. Commonly, it arises frommucinous tumor of the appendix and occasionallyfrom the ovary, colon, rectum, stomach, gallbladder, bile duct, small intestine, urinary bladder,lung, breast, pancreas, and fallopian tube5.Sometimes it arises from retroperitoneal tissueswhich are known as pseudomyxoma extraperitonei6.

We demonstrated one patient who has clinicaldiagnosis with disseminated peritoneal adeno-mucinosis (DPAM) and progression of the dis-ease. The diagnosis is difficult to diagnose.

Case Report

A-78-year-woman presented with slowlyprogressive abdominal distention without othercomplaints for more than 1 year. She visited ourhospital in May 2009. She had diabetes mellitusand hypertension with medical control. She doesnot smoke cigarettes or drinking alcohol. She washealthy and had no serious medical illness before.She was pallor but jaundice and cyanosis wereabsent. Abdominal examination revealed large

abdominal distention (Fig. 1) with massive ascitesbut no stigmata of chronic liver disease, tendernessor intra-abdominal mass. Pelvic examination re-vealed only cysto-rectocele with no significantabnormality. Examinations of respiratory, cardio-vascular and nervous systems were also normal.

Laboratory testing showed elevated serumconcentrations of CEA and CA19-9 (Table 1).Abdominal ultrasonography (USG) showed largeamount of ascites with irregular echogenic density,6.0cm. subcapsular fluid pocket at superolateralaspect of liver. Others visceral organs appearednormal (Fig. 2). A computed tomography (CT)scan after oral and intravenous contrast showedmassive septate ascites with compression of intra-abdominal organ, pattern of scallop liver, invasionof parenchyma of liver, and calcification. Therewas no any intra-abdominal mass that can be seen.(Fig. 3)

Figure 1 Abdominal distention pattern (jelly belly)

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Table 1 Laboratory Findings on Admission

White Blood Cell 4300/ulHemoglobin 9.4 g/dlHematocrit 27.5%Platelet 450 x 103/ulTotal protein 8.5 g/dLAlbumin 3.4 g/dLAspartate aminotransferase 30 U/LAlanin aminotransferase 11 U/LAlkaline phosphatase 95 U/LTotal bilirubin 0.61 mg/dlDirect bilirubin 0.27 mg/dlBlood urea nitrogen 12 mg/dlCreatinine 0.9 mg/dlBlood sugar 102 mg/dlCEA 272.2 ng/mlCA 19-9 241.7 U/mlCA12-5 15.5 U/mlAFP 1.87 ng/mlHBsAg negativeAnti-HBs negativeAnti-HCV negative

An abdominal paracentesis was performed.The aspiration contained a large amount of redjelly-like mucus (Fig. 4). Polymerase chain reac-tion (PCR) of ascitis fluid was negative forMycobacterium tuberculosis. Cytological studyshowed reactive mesothelial cells, lymphocyte andhistiocyte. To confirm the diagnosis, peritoneoscopewas performed with biopsy. Intra-abdominal cavityshowed a but not any mass found. The peritonealbiopsy revealed mucinous lake with fibrous stromaand few lymphocytes in the wall, and the picturewas consistent with PMP (Fig. 5).

The possibilities of gastric and intestinecancers have been rule out. Gastroduoscopy showedmild gastritis but colonoscopy was not performeddue to patient's condition.

After many investigations were performed;the conclusion of diagnosis was compatible with

Figure 2 Ultrasoundnography showed large

amount of irregularity echoic density

of ascites and 6.0 cm subcapsular

fluid pocket at superolateral aspect

of liver (black arrow)

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Figure 3 Computed tomographic scan of abdomen A. CT showed typical scalloping of liver (black arrow) and low-

attenuation implants in liver, indicating parenchymal invasion (white arrow). B. Massive amounts of septated

ascites and small calcification (arrow). C. Soft tissue infiltration (arrow) in left upper quadrant surrounding the

stomach and spleen, indicating interstitial reticular infiltration of the peritoneum. D. Large amount of ascites with

displacement of small bowel.

Figure 4 Red jelly-like mucinous ascites

disseminated peritoneal adenomucinosis by clini-cal progression, cytological, and imaging patterns.Patient and her relatives were advised to undertakelaparotomy, intra and postoperative intraperitonealchemothery, but they denied. Five months later,she died from complications of gut obstruction andperitonitis.

Discussion

The term pseudomyxoma peritonei (PMP)

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has been used in reference to any condition, benignor malignant, in which the peritoneal cavity isfilled with gelatinous substance7. A clinical caseconsistent with this diagnosis was first describedby Rokitansky in 1842 8, but R. Werth, gyneco-logist, first described PMP in 1884 as a peculiarreaction of peritoneum produced by ovarian neo-plasm9. In 1901 Frankel described it in associationwith appendiceal cyst10. The mean patient age isabout 58 years 3. PMP consists of neoplasticmucin producing cells in the peritoneal cavity.Mucin usually is secreted from ruptured mucinousneoplasm. Recent morphologic, immunohis-tochemical, and molecular genetic studies havesuggested that the appendix is most likely theoriginate site of PMP11-15, which sometimesinvolves other visceral oranges. The ovary, colon,uterus, common bile duct, pancreas, and stomachhave been documented as rare sites of PMPorigin16. Many case report series showed thatprimary tumor causing PMP are associated withappendiceal tumors such as appendiceal mucinousadenoma, appendiceal adenocystadenoma, oradenocarcinoma4,12,15,17.

After the wall of the appendix ruptures,

symptoms and signs of PMP can progress formonths or even years within the abdomen andpelvis without causing any symptoms. As thedisease progresses, the peritoneal cavity is filled ina characteristic pattern with mucinous neoplasmand mucinous ascites. The greater omentum isthickened (omental cake) and infiltrated exten-sively by the tumor. The most common symptomwith PMP is a gradually increasing abdominalgirth18. The Second most common symptom isovarian mass, usually on right side, in women andnew-onset of hernia in men18. The Yhird mostcommon symptom is presenting with appendicitis,a clinical manifestation of ruptured appendicealmucocele with local inflammation19.

PMP has been classified into three pathologicalsubtypes with different pathological characteristics(including malignant features) and associated witha different prognosis: disseminated peritonealadenomucinosis (DPAM), peritoneal mucinouscarcinomatosis with intermediate or discordantfeatures (PMCA-I/D), and peritoneal mucinouscarcinomatosis (PMCA).4 Histopathologically,DPAM is characterized by an abundance ofextracellular mucus with focally adenomucinous

Figure 5 Histological examination of the omental biopsy specimen revealed areas of fibrotic fatty tissue with mucous lake

without identifiable neoplastic cell, suggesting that it would have been difficult to acquire the cells in a biopsy

sample.

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epithelium with hardly any atypia or mitoticactivity which has a good prognosis. PMCA, incontrast, is characterized by peritoneal tumorwhich is composed of more abundant mucinoustumor cells with the architectural and cytologicalfeatures of carcinoma. Finally, the intermediatesubtype PMCA-I is characterized by an abundanceof DPAM lesions, not focal areas with PMCAlesions. The behavior and prognosis of PMCA-Isubtypes is between DPAM and PMCA20-21.Many studies implied that cytomorphologic fea-tures are an important prognostic indicator, such asin the study by Ronett et al.20, which reported 5-year survival rates as 75% for DPAM, 50% forPMCA-I, and 14% for PMCA. In the study byJackson et al22, they reviewed cytology fromperitoneal washing and classified histologicalsubtype. Analysis of follow-up data showed thatDPAM had better prognosis than PMCA. Moreo-ver, study by Gupta S. et al, reported mediansurvival for patients with DPAM, PACA-I, andPMCA were 7.7, 1.2, 0.7 years, respectively23.Beside, there are some serologic markers that mayalso be a survival predictor, CEA and CA 19-9,but there is only a small number of patients.CA19-9 is may be a prognostic factor for predict-ing recurrent disease24 and CEA elevation ispossible in poor prognosis recurrence of dis-eases25.

The diagnosis of PMP is often difficultbecause it usually has an insidious presentation,while radiological feature is an important tool fordiagnosis PMP. Ultrasonography is the first imagingtechnique to use for further establishment of thediagnosis. Typical findings are nonmobile echogenicascites with multiple semisolid masses and scal-loping of the hepatic and splenic margins due to

extrinsic pressure of adjacent peritoneal im-plants26,27.

Computer tomography (CT) of the abdomenand pelvis are the most widely applied technologywhich has been used with great success in thediagnosis of the PMP syndrome. CT findings areoften highly suggestive of PMP, and sometimesthese are pathognomonic.2 The most commonfinding is a large volume of mucinous ascites,which has the density properties (Hounsfield Units[H.U.]) higher than normal (5-20 H.U. vs. +/- 0H.U.)28 and displaces the small bowel. Othercharacteristic findings are omental thickenings,multiseptated lesions, scalloping of organs, andcurvilinear calcifications26,29. Bechtold et al. haverevealed the pattern of CT for classified histologicalcharacteristics of the disease. DPAM wascharacterized by larger amount of ascites, noomental cake, typical hepatic scalloping, nolymphadenopathy, some calcified mass, and noprimary lesion is present. However, PMCA werecommonly found in the presence of omental cake,coexistent disease in the chest, lymphadenopathyand visualization of a primary mass30.

There is currently no accepted standardtreatment for PMP. The choice of treatmentstrategy has varied much in the past. Only onereport suggested observation31, but survival datawas not supported from large studies. UntreatedPMP patients would eventually suffer death throughintestinal obstruction by massive mucinous ascitesand large tumor deposits32. Traditional surgicaltreatment is repeated interval debulking proce-dures for relief of symptoms, but with limitedexpectation of long-term survival and no prospectof cure2.

Currently a cytoreductive surgery (CRS) and

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perioperative loco-regional chemotherapy (PLC)regimen have been shown in multiple studies toimprove survival, as compared with historicalcontrols21,33-39. Aggressive cytoreductive surgeryincluding parietal peritonectomy and resection ofinvolved viscera are used to reduce macroscopictumor masses. Technique for PLC is usuallyhyperthermic intra-peritoneeal chemotherapy(HIPEC) with mitomicin-C or 5-fluorouracil foreradication residual microscopic tumors. In studyby Smeek RM, et al. report survival analysis inpatients who received cytoreductive surgery withhyperthermic intra-peritoneal chemotherapy showsurvival benefit in DPAM subgroup than PMCA-I and PMCA with statistical significantly, hazardratio 1.9 (1.0-3.5) and 4.1 (1.5-11.1), respec-tively40. On the other hand, combined treatmenthas relatively high morbidity and mortality ratesdue to complications from extensive surgery.Treatment related morbidity and mortality seem tobe related to age, tumor load, extent of cytoreductivesurgery, and associated operative factors34,35,39-42.

In this patient, clinical presentation was anabdominal distention like jelly belly pattern, andwith large amounts of gelatinous ascites. She didnot have any previous surgical treatment such asappendectomy or Cesarean section. Radiographicshowed typical patterns of scallop liver, andparenchymal invasion. No lymph node involve-ment or no primary lesion was presented. Perito-neal biopsy was done under peritoneoscope. His-topathology is compatible with DPAM, due toits abundance of extracellular mucus withoutcellular atypia. However, tissue sampling may notbe sufficient, because it was found only fibroticfatty tissue with mucous lake, and without ad-equate cellularity. Histopathology classification

may be under diagnosis that reflects to theprognosis. The primary tumor may be raised fromboth appendix and ovary because it is the mostcommon organ causing of this disease, but it issuggestively originated from appendix. She hadnormal gynecologic examination and tumor markerof the ovary is in normal range. There were manylimitations to diagnose and definite treatments inthis case especially she did not receivedcytoreductive surgery that was a major indicator tothe survival.

She seemed to have good prognosis or slowprogression. She had some bad indications suchas old age, inadequate tissue sampling, cytoreductiveincomplete, serum CEA, and CA19-9 elevation.All of this factors effect to prognosis and survivalof her disease.

Conclusion

Even though clinical diagnostic modalitieslike USG, CT and MRI can provide supportiveevidence in favor of PMP, but the definitivediagnosis can only be made by explorativelaparotomy. It is useful for diagnosis especiallyhistological pattern and it may identify primarysite of the origin. Prognosis of this diseasemajority depends on histological pattern. There isevidence that treatment should be more aggressivefor improving survival. In the present, combina-tion of CRS-PLC is possibly becoming the newstandard of care.

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