Pseudomyxoma Peritonei Manifesting as

Intestinal Obstruction
Arif Nawaz, MD, Ali Karakurum, MD, David Weltman, MD, Ahmed Shehata, MD, Imtiaz
Mohammed, MD, Carylann Hadjiyane, MD, Crescens Pellecchia, MD, Nassau County Medical
Center, State University of New York at Stony Brook, East Meadow.
South Med J. 2000;93(9)

Abstract and Introduction

Abstract
Pseudomyxoma peritonei is an appendiceal tumor with distinct clinical and pathologic features. It
frequently presents a problem in diagnosis and management. We report a case of
pseudomyxoma peritonei, which initially appeared with intestinal obstruction. This is a rare initial
manifestation in patients who have not had multiple surgical procedures. We review the literature
and discuss the unique clinical features and misconceptions surrounding pseudomyxoma
peritonei.

Introduction
Pseudomyxoma peritonei is a rare but frequently misdiagnosed condition. It has diverse and
slightly confusing presentations, including abdominal distention, ovarian tumors, or appendicitislike syndromes. In this article, we emphasize the unique pathophysiologic, clinical, and
management aspects of this disease entity.Continue Reading

Case Report
A 45-year-old black man was admitted with a 1-week history of cramp-like lower abdominal pain
and incessant vomiting. He had no history of surgery or medical illnesses. He had used cocaine
and alcohol in the past but was currently taking no drugs. Physical examination showed a
distended abdomen with mild lower abdominal tenderness. Findings on the rectal examination
were normal. Laboratory data showed a white blood cell count of 20,600/dL, with normal
hemoglobin and hematocrit values. The patient was dehydrated with hyponatremia and increased
serum urea nitrogen concentration. Radiologic studies (abdominal film and computed tomography
[CT]) were suggestive of intestinal obstruction (Figure).

(Enlarge Image)

Figure 1.

Computed tomography without contrast medium showed (A) multiple tubular, fluid-filled masses in pelvis; (B)
calcification along wall of mass (arrow); and (C) air bubbles within mass (arrow). (D) At 1-year follow-up, cystic
pelvic mass was seen (arrow).

The patient had exploratory laparotomy, and intraoperative findings consisted of a large mass in
the left lower quadrant adherent to the omentum and sigmoid colon. After careful dissection, it
was thought to be necrotic small bowel. Since the mass appeared to be of small bowel origin,
small bowel resection and an ileostomy and mucus fistula were done. Pathology was consistent
with well-differentiated adenocarcinoma of appendiceal origin. The patient remained
asymptomatic for approximately 6 months, then had recurrent abdominal pain. Workup revealed
recurrence of the tumor (Figure). Chemotherapy was begun, and the patient was doing well with
some decrease in the bulk of the tumor 2 years after the initial diagnosis.Continue Reading

Discussion
For years, the clinical syndrome of pseudomyxoma peritonei has been
enigmatic. The term pseudomyxoma peritonei was first used by Werth[1] in
1884 to describe massive intraperitoneal accumulation of gelatinous
pseudomucin due to the perforation of ovarian pseudomucinous cystomas.
Based on recent studies reevaluating this condition, tumors previously
designated pseudomyxoma peritonei, can now be viewed as two
pathologically and prognostically distinct disease processes. [1-9]
Pseudomyxoma peritonei is a disease process characterized by copious
mucinous ascites and histologically bland peritoneal mucinous tumor. It is
attributable to a ruptured mucinous cystadenocarcinoma (appendiceal origin
in most cases). It has an indolent course but may recur over months to years.
Peritoneal mucinous carcinomatosis is a disease process characterized by
abundant peritoneal mucinous tumor, and its clinical appearance is similar to
that of pseudomyxoma peritonei. However, the peritoneal cells have
architectural and structural features of carcinoma. These cells are derived
from the gastrointestinal mucinous adenocarcinomas and are associated with
a significantly worse prognosis.[2]
In the past, pseudomyxoma peritonei was said to occur from a variety of
primary tumors.[3,4] This may be true, but in the vast majority of cases, the
patients have an appendiceal tumor giving rise to this clinical entity.
[4]
Mucinous peritoneal carcinomatosis may arise from other sites, such as the

colon, gallbladder, pancreas, or stomach, but these tumors usually have

signet ring histology. They may show redistribution but do not spare the small
bowel and will implant and grow in the abdominal cavity in a random fashion
with extensive small bowel involvement, resulting in a much poorer prognosis.
The redistribution phenomenon occurs in patients with pseudomyxoma
peritonei who have not had previous surgery.[2,4-8] Two physiologic principles
are involved. First, the mucinous tumors accumulate at the site of peritoneal
absorption (omentum and undersurface of the diaphragm). Second, gravity
causes the mucinous tumor cells to settle within the dependent portions of the
abdomen (pelvis, right retrohepatic space, left abdominal gutter, and ligament
of Treitz). The visceral peritoneal surfaces are usually spared, since they
relentlessly move during peristaltic activity.[4] For this unique pattern of cancer
dissemination to occur, several biologic requirements and physiologic
phenomena must be operating. Pseudomyxoma cells do not have adherence
molecules on their cell surface. This lack of "stickiness" means that the tumor
cell will not actively attach to an abdominal or pelvic surface. The tumor will
progress by the production of mucus, exfoliation of tumor cells, and a
redistribution of these cells around the abdomen according to the
aforementioned physiologic mechanisms. The "dissecting mucus" that is
recognized by the pathologist as part of the histologic picture of
pseudomyxoma peritonei should not be confused with invasive malignancy,
which is not a part of this syndrome.
Pseudomyxoma peritonei has multiple clinical manifestations that lead to
difficulties in definitive diagnosis and timely treatment. The usual clinical
features of this tumor are increasing abdominal girth (40%), bilateral or
unilateral ovarian tumors (20%), hernia sac tumors (20%), appendicitis-like
syndrome (10%), and infertility (10%).[4] Narrowing of the gastrointestinal tract,
but rarely complete obstruction, frequently occurs at three well-defined
anatomic sites -- the pyloric antrum, the ileocecal valve, and the cul-de-sac of
Douglas.[4] These are three portions of the gastrointestinal tract that are
attached to the retroperitoneum and are relatively motionless. Intestinal
obstruction is rarely an initial manifestation of this disease and usually occurs
when multiple previous surgeries have led to small bowel entrapment. [5]

Our patient had intestinal obstruction and emergency surgery. The urgent
nature of this and other clinical manifestations such as appendicitis-like
syndrome may lead to unnecessary surgical procedures, resulting in poorer
outlook. Unwary clinicians may also mistake pseudomyxoma peritonei for
multiple abscesses or adenocarcinoma, and the patient may have repeated
surgeries, leading to intestinal obstruction and poor outcome. As mentioned
earlier, this disease is distinct from mucinous adenocarcinoma of the colon,
gallbladder, pancreas, or stomach in that, in the absence of repeated surgical
procedures, it spares the small bowel and has a much better prognosis.
Diagnosis of pseudomyxoma requires a high index of suspicion supplemented
by various radiologic studies, including CT, followed by pathologic diagnosis.
[10]
Recently, some sonographic features of pseudomyxoma have also been
reported, thereby adding to the growing list of available diagnostic modalities.
[11]
A case of pseudomyxoma peritonei with abnormal radionuclide uptake has
also been described,[12] though the utility of this diagnostic modality has not
been thoroughly evaluated.
The prognosis of pseudomyxoma peritonei has always been extremely
guarded. Sugarbaker[4] has shown that repeated surgeries result in a median
survival of approximately 2 years, with only a small percentage alive at 5
years. Patients who have repeated surgeries and extensive systemic
chemotherapy show some improvement in survival, but no long-term,
disease-free survival is expected.
The Washington Hospital Center has pursued a "cytoreductive approach" for
these patients.[4,9,13] This treatment approach involves an initial series of welldefined peritonectomy procedures that strip all disease from the parietal
peritoneal surfaces and resect all visceral peritoneal involvement, leaving the
abdomen virtually free of disease. Additionally, perioperative chemotherapy is
used to kill any remaining cancer cells. Sugarbaker [4] recommended that
patients with grade I mucinous adenocarcinoma be treated with extensive
cytoreduction followed by intraoperative and postoperative chemotherapy.
Other investigators[15] have reported on the success of a similar approach in
patients who are in poor general condition. Some centers have also tried
intraperitoneal chemotherapy through implantable port systems with varying

success.[14,15] If managed appropriately, these tumors have an excellent 5-year

survival rate exceeding 80%.Continue Reading

Discussion
Pseudomyxoma peritonei is a distinct clinical entity with unique clinical and
pathologic features. It requires vigilance by the clinician, gastroenterologist, or
surgeon who evaluates the patient initially, since early diagnosis is essential
for the appropriate treatment of this disease. It may rarely manifest as
intestinal obstruction, and the patient may have emergency surgery and small
bowel resection, which lead to an adverse outcome. Success has been
reported with an approach combining cytoreductive surgery and
chemotherapy. If properly treated, patients with these tumors have a 5-year
survival rate exceeding 80%.