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Case of PMP

Case of PMP

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44
Hospital Physician
September 2008 
 www.turner-white.com
CASE PRESENTATIONInitial Presentation and History
 A 40-year-old man presented with a 4-month history of intermittent right lower quadrant abdominal painassociated with increasing abdominal girth, early satiety,loose stools, unintentional weight loss of 20 lb, and gen-eralized fatigue. He denied episodes of nausea, vomit-ing, melena, hematochezia, or fever. The patient’s past medical history was significant only for an inguinal her-nia repair 10 years earlier. He worked as a groundskeep-er and reported being in excellent physical condition.He acknowledged that he had previously been a heavy drinker of alcohol, but he quit consuming alcohol6 months prior to presentation. He denied illicit drugor tobacco use, medication use, recent travel, or a fam-ily history of significant medical conditions.
Physical Examination
On physical examination, the patient had normal vital signs. He appeared to be in no physical distressbut did exhibit bitemporal wasting. Sclerae were an-icteric, lungs were clear to auscultation, and heart rhythm and sounds were normal. Bowel sounds werepresent and normal. The patient’s abdomen was mod-erately distended and dull to percussion, with mildtenderness evoked upon palpation of the right lowerquadrant. Rebound and guarding were absent, but a fluid wave and shifting dullness were both present.Two enlarged lymph nodes were palpated in the right inguinal region. Brown, hemoccult-negative stool waspresent in the rectal vault.
Diagnostic Studies
Laboratory studies and complete blood count wereordered. Hemoglobin was 10.8 g/dL (normal, 13.5–17 g/dL), iron was 15
µ
g/dL (normal, 60–150
µ
g/dL),and total iron binding capacity was 206
µ
g/dL (nor-mal, 250–400
µ
g/dL), which was indicative of mildiron-deficiency anemia. The white blood cell count,platelet count, transaminases, bilirubin, amylase, li-pase, coagulation studies, and urinalysis were all withinnormal limits. Albumin was slightly low at 3.3 g/dL(normal, 3.9–5.0 g/dL). Levels of carcinoembryonicantigen (CEA) and cancer antigen (CA) 19-9 wereelevated at 91.5 ng/mL (normal, 0–5 ng/mL) and470.0 ng/mL (normal, 0–15.8 ng/mL), respectively. A plain radiograph of the abdomen was unremarkable.Computed tomography (CT) showed extensive sep-tated ascites throughout the abdomen, with scallopingof the liver margin and omental thickening. Also noted was a thickened tubular structure in the area of the ap-pendix (
Figure 1
).Bedside paracentesis was attempted but no abdomi-nal fluid was recovered, most likely due to the viscos-ity of the fluid. Ultrasound-guided paracentesis using a20-gauge needle was then attempted, which again failedto obtain fluid despite the position of the needle beingconfirmed in the ascites. Finally, an omental biopsy wassuccessfully performed by an interventional radiologist.The fluid from this procedure revealed numerous neo-plastic cells floating in pools of mucin (
Figure 2
).
WHAT IS YOUR DIAGNOSIS?
(A) Acute appendicitis(B) Carcinoid tumor of the appendix(C) Cirrhosis with ascites(D) Disseminated echinococcal disease(E) Pseudomyxoma peritonei
Clinical Practice Exam 
A 40-Year-Old Man with IntermittentRight Lower Quadrant Pain andIncreasing Abdominal Girth
Daniel L. Cohen, MD Paul A. Feldman, MD Stephen E. Vernon, MD 
 Dr. Cohen is a fellow, Division of Gastroenterology, New York University Medical Center, New York, NY. Dr. Feldman is an assistant professor, Division of Gastroenterology, University of Miami Miller School of Medicine, and attend- ing physician, Miami Veterans Affairs Medical Center, Miami, FL. Dr. Ver- non is an associate professor, University of Miami Miller School of Medicine; and senior attending pathologist, Department of Pathology, Jackson Memorial Medical Center, Miami, FL.
 
 www.turner-white.com Hospital Physician
September 2008 
 
45
Cohen et al : Clinical Practice Exam : pp. 44–48 
ANSWER
The correct answer is (E), pseudomyxoma peritonei(PMP).
DISCUSSION
The patient’s presentation, physical examination,laboratory data, and imaging studies were all consistent  with a presumptive diagnosis of PMP. Although dissemi-nated echinococcal disease can cause septated ascites, aprimary cystic lesion (hydatid cyst) is usually seen in theliver.
1
The patient’s laboratory study results and the pres-ence of septated ascites fluid were not consistent withthe diagnosis of cirrhosis, nor did his liver appear to becirrhotic on imaging.
2
Acute appendicitis would demon-strate an inflamed appendix on CT imaging, and is asso-ciated with fever, an elevated white blood cell count, anda more abrupt time course.
3
A carcinoid tumor of theappendix can present with many of the constitutionalsymptoms experienced by the case patient in additionto an enlarged appendix on imaging.
4
However, it is not associated with septated ascites or scalloping of the livermargin. Finally, the presence of mucinous ascites withneoplastic cells confirms the diagnosis of PMP.
5
CLINICAL COURSE OF THE CASE PATIENT
The case patient underwent an exploratory lapa-rotomy, which revealed massive amounts of gelatinousmaterial throughout the abdomen (
Figure 3
). Tumorcells were affixed to the stomach, liver, small bowel, andperitoneal walls, and an appendiceal mass was palpated.The appendiceal mass was resected via an ileal cecec-tomy with primary anastomosis. In addition, omentec-tomy and splenectomy were performed. Due to the ex-tent of PMP, complete cytoreduction was not possible.Pathologic examination revealed a well-differentiated,low-grade mucinous cystadenocarcinoma of the appen-dix (
Figure 4
). Mucin was noted to be exuding througha perforation in the appendiceal wall. The patient un-derwent perioperative intraperitoneal chemotherapy (PIC) with 5-fluorouracil and mitomycin-C.Two years after his initial presentation, the patient continued to be asymptomatic and reported feeling well. However, follow-up CT scans demonstrated recur-rence of abdominal fluid collection. CEA and CA 19-9levels, which had significantly decreased after surgery and PIC, were rising again. The patient was scheduledfor close follow-up, as he eventually will require repeat debulking surgery and possibly additional chemo-therapy.
PSEUDOMYXOMA PERITONEI
PMP (literally, “false mucinous tumor of the peri-toneum”) is a rare condition that was first describedby Werth
6
in 1884. PMP has an estimated incidence of 1 case per million persons annually.
7
The characteristicfeature of PMP is large quantities of mucinous ascites, which is often referred to as “jelly belly.”
7,8
PMP is theclinical term used to describe this condition, but it doesnot specify the underlying lesion responsible for themucinous ascites. In most cases, the lesion is a slow-growing but progressive appendiceal neoplasm. As thetumor enlarges, it obstructs the lumen of the appendix,thus causing perforation.
5
As a result, mucin-producingneoplastic cells are disseminated throughout the peri-toneal cavity where they produce copious amounts of mucin, which accumulates due to gravity in various areasof the abdomen. Most of these tumors are minimally ag-gressive, rarely metastasize, and tend to remain confinedto the peritoneum.
5
If not treated, patients slowly devel-op terminal starvation due to abdominal distention andincreased tumor volume.
5
 Although PMP is most commonly associated withan appendiceal tumor, it may also arise from othersites. Recent studies have shown that ovarian lesions,previously thought to be even more common than ap-pendiceal lesions, are likely due to metastatic spreadfrom an appendiceal primary site.
8,9
Rare cases havealso been reported to arise from tumors in the colon,rectum, pancreas, stomach, small bowel, gallbladderand bile ducts, lung, breast, fallopian tubes, urachus,and urinary bladder.
10
 As PMP is a clinical term, various classification sys-tems have been devised to categorize this entity by its
Figure 1.
Computed tomography demonstrating massive amountsof septated ascites (
white arrows
) and a tubular appendiceal struc-ture (
black arrow 
).
 
Cohen et al : Clinical Practice Exam : pp. 44–48 
46
Hospital Physician
September 2008 
 www.turner-white.com
pathologic findings. In a landmark paper, Ronnett et al
11
 described 3 categories: disseminated peritoneal adeno-mucinosis (DPAM), peritoneal mucinous carcinomato-sis (PMCA), and peritoneal mucinous carcinomatosis-intermediate (PMCA-I). DPAM is characterized by PMPcontaining epithelial cells with little cytologic atypia ormitotic activity, whereas PMCA contains more abundant mucinous epithelial cells with the architectural andcytologic features of carcinoma. PMCA-I, as the namesuggests, is an intermediate group that contains peri-toneal lesions most consistent with DPAM but has focalareas of mucinous carcinoma consistent with PMCA.Ronnett et al
11,12
found that this classification systempredicted mortality, with DPAM having the best survival,followed by PMCA-I and PMCA. Bradley et al
13
appliedthis classification to their patient cohort and foundthat PMCA-I patients had the same survival as DPAMpatients. As a result, they suggested merging DPAM andPMCA-I into a single group and proposed a simplifiedclassification system with 2 categories: high-grade muci-nous carcinoma peritonei (MCP-H) and low-grade mu-cinous carcinoma peritonei (MCP-L). These differingpathologic classifications have understandably createda great deal of confusion and contention in the medicalliterature.
14
Further adding to the confusion is a debateabout whether the term PMP should be used only inreference to mucinous spread from low-grade appendi-ceal lesions (whether it is called DPAM or MCP-L) or inreference to the whole spectrum of lesions regardless of pathologic grade. Ronnett et al
12
have suggested limitingthe term PMP to DPAM cases, given the more benignnature of this entity. However, this opinion has not beenfully accepted, and it remains an area of debate.
Disease Manifestations and Diagnosis
 Asymptomatic cases of PMP may be found inciden-tally during surgery and are estimated to occur in ap-proximately 2 out of every 10,000 laparotomies.
9
Whenpatients develop symptomatic disease, they most com-monly present with suspected appendicitis (27%), in-creased abdominal girth (23%), an ovarian mass (20%of female patients), or a new hernia (14%),
15
which arenot specific for PMP. On laboratory evaluation, patientsoften have elevated tumor markers. CEA is elevated in56% to 75% of cases, and CA 19-9 is elevated in 58% to67% of cases.
16,17
Normal levels at presentation may pre-dict improved survival,
16
and elevated levels after treat-ment may predict recurrence and a worse outcome.
17
In patients who present with increased abdominalgirth and ascites and in whom PMP is suspected, an
Figure 4.
Histopathology showing a well-differentiated mucinouscystadenocarcinoma of the appendix (hematoxylin and eosin, 400
×
).
Figure 3.
Laparotomy revealing large quantities of intraperito-neal mucinous material (“jelly belly”).
Figure 2.
Neoplastic cells isolated from abdominal fluid (ob-tained via paracentesis) floating in pools of mucin (hematoxylinand eosin, 400
×
).

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