mucinous and gelatinous material. It is usually secondary to a rupture of a mucocele of the appendix or cystadenocarcinoma of the ovary. It is clinically difficult to ...
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Journal of the Royal Society of Medicine Volume 82 October 1989
Clinical and radiological features of pseudomyxoma peritonei
J Hossain M?RCP
T Malabarey FRCR
I Al-Mofleh FAIM
ofMedicine and Radiology, King Khalid University Hospital, Riyadh Radiology, Riyadh Central Hospital, Riyadh, Saudi Arabia
N E-D Hawass FRCR A H Ismail MD
Departments Department of
Keywords: pseudomyxoma peritonei; computed tomography; laparoscopy; diagnosis
Summary Pseudomyxoma peritonei is due to diffuse involvement of the peritoneal cavity with mucinous material. Four patients were found in two major general hospitals in Riyadh. All patients were male. Cardinal clinical features were abdominal distension, pain and weight loss. Computed tomography was helpful preoperatively. Laparoscopy or laparotomy were performed to arrive at a correct diagnosis.
Introduction Pseudomyxoma peritonei is a rare clinical condition in which peritoneal cavity is diffusely involved with mucinous and gelatinous material. It is usually secondary to a rupture of a mucocele of the appendix or cystadenocarcinoma of the ovary. It is clinically difficult to diagnose pseudomyxoma peritonei preoperatively. We report our experience with four cases of pseudomyxoma peritonei with reference to clinical features, new imaging techniques and role of laparoscopy.
Figure 1. CT of upper abdomen. Note the scalloped right borders of the liver which is surrounded by low attenuation soft tissue masses extending to the left flank surface and intestine. Peritoneal biopsy showed metastatic mucin-producing adenocarcinoma.
Materials and methods Between 1982 and 1988, four patients with mucinous ascites were diagnosed at King Khalid University Hospital and Riyadh Central Hospital. We reviewed the records of the patients who had pseudomyxoma peritonei. All four patients had ultrasonographic and computed tomographic (CT) studies. The diagnosis was confirmed by laparoscopy in two and laparotomy in the other two patients provided histopathological proof. We report here the clinical course and compare the new imaging techniques.
Case 2 A 75-year-old Saudi male had increasing abdominal distention and fullness of three months' duration. Two years prior to this presentation he had resection of left inguinal mass. On exanation of abdomen he had multiple irregular, firm, non-tender mobile masses in the epigastrum and paraumbilical region. There was ascites. Complete blood count showed haemoglobin of 10.1 g/dl, platelets 82300/cm with erythrocyte sedimentation rate of 85/mm/h. Ultrasound examination of abdomen showed a coarse pattern of omentum with ascites. CT scan of abdomen
Cas 1 A 52-year-old Saudi male noticed progressive swelling of abdomen with abdominal discomfort and occasional vomiting. On examination of abdomen he had ascites with a firm mobile mass measuring 15 x 15 cm non-pulsatile and with an irregular margin. Routine blood test showed haemoglobin of 10.8 gIdI with erythrocyte sedimentation rate 120 mm/h. Liver function tests were normal except for a raised alkaline phosphatase (331 u/I). Ultrasound examination of the abdomen showed a large diffus complex, mainly solid, in the upper abdomen. An abdominal CT examination was performed with both oral and intravenous contrast media. This examntion demontr huge ascites with multiple heterogenous soft tissue masses occupying the entire abdomen and pelvis, and intervening between the liver, spleen and lateral abdominal wall. Scalloping of the lateral aspect of the liver edge was present (Figure 1). The masses were floating diffusely throughout the ascitic fluid. The pelvic component of the lesion was of a multiloculated type. Slight enhancement of the soft tissue masses were noted after intravenous contrast media administration. Laparoscopy revealed ascites with multiple whitish modules, whilst a gelatinous mass covered the omental
Figure 2. CT scan of lower abdomen. The abdomen is filled with low attenuation mass within which are bowel loops. Some bowel loops are seen circumscribing the mass anteriorly. Strands with branches are noted within the mass, and are seen extending centrifugally
0141-0768/89/ 100600-03/$02.00/0 i 1989 The Royal Society of Medicine
Journal of the.Royal Society of Medicine Volume 82 October 1989 demonstrated a very large low attenuation mass of fat density occupying the entire abdomen and extending into, the pelvis. There were multiple incomplete ramifications within this low attenuation area (Figure 2). The edgoof the. liver was smooth with normal liver parenchyma. 'No loculated cystic lesions seen. The bowel-loops were seen within the low attenuation mass. Laparoscopy-showed ascites with whitish flakes of mucinous material and jelly-like nodules covering the surface of omentum, bowel wall, liver.; Biopsy of the peritoneum showed clusters of epithelal mucous secreting cells, of low grade adenocarcinoma. Case 3 A 60-year-old Saudi male was admitted with pain in right lower abdomen, diarrhoea, anorexia and fulness of abdo On examination he had a firm, non-tender mass (5x8 cm) in right iliac fossa. An ultrasound examination of abdomen showed a large anechoic mass in right iliac fossa and extending up to the paraumbilical region with indistinct margins. CT examination of abdomen showed a large partially septate low attenuation lesion occupying the abdomen and involving the pelvis the lesion displacing the bowel into the anterior and lateral abdominal walla. The attenuation of this lesion was very similar to abdominal wall fat density (Figure 3). There were also few intra-abdominal calcifications. The liver edge appeared normal. Laparotomy showed the peritoneal cavity to be filled with multiple jelly-like material with an appendicular mass. Some white jelly-like projections were noted over the mesentery. Appendectomy was performed. Histopathology of the appendix confirmed rupture of mucinous cystadenoma. Case 4 A 55-year-old Yemeni male presented with abdominal pain and weight loss with progressive abdominal swelling of two months' duration. Examination of the abdomen revealed ascites with a firm mass in the left lumber region. Routine blood examination showed a haemoglobin of 10.5 g/dl, and platelets 945000/cm. Ultrasound examination showed ascites. CT examination showed ascites only. CT examination demonstrated massive cyst completely occupying the abdomen and pelvis with multiple soft tissue masses scattered through it. Some ofthese were loculated and intervened between liver and lateral abdominal wall. The liver edge were grossly scalloped (Figure 4). There was also a large well-defined soft tissue mass with some cystic components in the right lower lumbar region. The patient underwent exploratory laparotomy. Peritoneal cavity was filled with mucinous cysts and similar cysts were covering liver surface, small and large bowel. There was massive ascites. A retrocaecal mass was found in which the appendix was incorporated. Resection of the appendicular mass was technically difficult.
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Figure4. CT scan of upper abdonen showing low attenuation mass surrounding within the abdomen and ilso enveloping the spleen as well as the liver which has saloped borders
Discussion The clinical entity of pseudomyxoma peritonei is characterized by diffus involvement of the peritoneal cavity with masses of gelatinous tumour-like material accompanied by mucinous ascites. This condition was first recognized in 1842 by Rokitanasky and in 1884 Werth- introduced the term pseudomyxoma peritonei because the material obtained from the peritoneal cavity was different chemically from mucin. The majority of pseudomyxoma are secondary to mucocele of the appendix or low grade primary ovarian tumourl. Other primary sites in the uterus, bowel, urachus, umbilicus, common bile duct2 and pancreas3 have been reported. The tumour seedling in the peritoneum occurs following the rupture or direct penetration of the primary tumour capsule and subsequently the neoplastic cells spread diffusely in the peritoneum. Controversy exists as to whether pseudomyxoma peritonei is a benign or malignant condition2. All our patients were male, aged over 50 years. This is in contrast to the findings of Fernandez' who reported a female preponderance in 38 patients of 32 female patients (84%). It would be difficult to draw any conclusion from our small number of patients regarding the sex distribution. The major clinical features were abdominal pain, weight loss, ascites and a palpable mass. This is similar to the pattern of presentation as reported by
Fernandez'. The CT finding of massive ascites of fat density with scattered masses of soft tissue density within it, some ofwhich might be loculated, and the scalloping ofthe
Figure 3. CT scan of mid-abdomen showing a large mass of low attenuation, similar in density to subcutaneous fat filling the abdomen and with the loops displayed peripherally around it. The lesion shows branching strands within it
liver margins appear to be characteristic. All these changes have been consistently described6-"1, and most were noted in our cases. Liver scalloping was found in three of our patients. The ascites and the scattered soft tissue masses were observed in all cases. Plain radiography did not show calcification in our cases. However, abdominal curvilinear calcifications when present might suggest the diagnosis on the plain film. Two of our patients underwent laparoscopical evaluation. In both cases multiple jelly-like materials were noted over the peritoneum and other intraabdominal organs. This picture may mimic intraabdominal tuberculosis and hydatid disease, but their characteristic pattern of gelatinous materials is highly suggestive of pseudomyxoma peritonei. Laparoscopy may be helpful in early diagnosis of
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pseudomyxoma peritonei while laparotomy may be required to confirm the diagnosis and for treatment'2. Pseudomyxoma peritonei is a slowly growing tumour and is compatible with prolonged survival. This is supported by the fact that two of our patients reported were still alive 6 years after the diagnosis. It is noted that the site of the primary tumour may be difficult to locate as observed in our two patients. The modalities of therapy available are surgery, chemotherapy and radiation. There is no general agreement concerning the therapy. Two of our patients underwent surgery for diagnostic reasons. In case 3 at operation mucocele of the appendix was removed. In case 4 it was difficult to resect the appendicular mass, and the surgical procedure was abandoned. It is stated that it may be difficult to resect the diseased appendix'. At operation, the peritoneal cavity is usually full of mucinous material floating as a homogenous mass or multiple cystic masses5. As the disease is a slow growing tumour some authors suggest aggressive surgical debulking procedure of the tumour mass and installation of alkylating agents4. Other authors suggest radiation of the tumour may offer improved survival rate'. We conclude that preoperative diagnosis of pseudomyxoma peritonei is possible with recent imaging techniques. Laparoscopy or laparotomy confirmed the diagnosis of this condition. This disease is associated with prolonged survival. Acknowledgments: The authors thank Professor TM Kolawole for critical review of this manuscript and Ms Bennie Campos for secretarial assistance.
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