410 CASE REPORT Ultrasound Obstet Gynecol 2002; 19: 410 – 412 Blackwell Science Ltd Incidental ultrasound diagnosis of pseudomyxoma peritonei in an asymptomatic woman S. KHAN*, A. G. PATEL† and D. JURKOVIC* *Early Pregnancy and Gynaecology Assessment Unit and †Department of Surgery, King’s College Hospital, London, UK KEYWORDS : Laparoscopic surgery, Pseudomyxoma peritonei, Ultrasound ABSTRACT An incidental finding of pseudomyxoma peritonei is reported in a woman with a 6-month history of postmenopausal bleeding. A transvaginal ultrasound scan revealed a poorly defined echogenic mass in the right iliac fossa above the right ovary and free fluid of mixed echogenicity in the pouch of Douglas. Laparoscopic appendicectomy and aspiration of mucinous fluid was performed without adjuvant chemo- therapy. Regular postoperative follow-up scans, which are needed as the disease may have an indolent course, showed no signs of recurrence. The differential diagnosis and manage- ment of pseudomyxoma peritonei are discussed. INTRODUCTION Pseudomyxoma peritonei is characterized by an extensive accumulation of thick mucinous material within the perito- neal cavity. It is a rare condition that is slightly more common in women 1,2 . Pseudomyxoma peritonei usually presents as acute appendicitis or as increasing abdominal distension. Alternatively, an ovarian mass in women or inguinal hernia in men may be the initial presenting feature 2 . In this report, we describe an incidental finding of pseu- domyxoma peritonei in a woman who attended for ultrasound scan with a history of postmenopausal bleeding. Ultrasound diagnosis and controversies in the treatment of this condition are discussed. CASE REPORT A 49-year-old woman on sequential estrogen hormone replacement therapy was referred for transvaginal ultrasound examination with a 6-month history of irregular vaginal bleeding. She became menopausal 3 years previously and had been using hormone replacement therapy for 2 years. She had regular cervical smears, which were all normal. She had three children. Her first two pregnancies were uncomplicated, whilst her last child was delivered by an emergency Cesarean section 8 years previously. Twenty years prior to presenta- tion, she had an open cholecystectomy. Her medical history was otherwise unremarkable. Her maternal grandmother had died of ovarian carcinoma at the age of 97 years. There were no other cases of cancer in her family history. General clinical and abdominal examination revealed no abnormalities. On speculum examination, the vagina and the cervix appeared healthy. On transvaginal ultrasound exam- ination, the uterus appeared normal. The endometrial thickness was 8.2 mm. The endometrium appeared irregular with ultra- sound features suggestive of an endometrial polyp (Figure 1). The ovaries appeared normal. A poorly defined echogenic mass, measuring 25 mm in diameter, was noted in the right iliac fossa above the ovary (Figure 2). A considerable amount of gelatinous fluid of mixed echogenicity was seen in the pouch of Douglas (Figure 3). A presumptive diagnosis of pseudomyxoma peritonei due to ruptured appendix mucocele was made and the patient was booked for an urgent laparoscopy and hysteroscopy. Preoperative hematological, Correspondence: Dr D. Jurkovic, Early Pregnancy and Gynaecology Assessment Unit, Department of Obstetrics and Gynaecology, King’s College Hospital, Denmark Hill, London SE5 8RX, UK (e-mail: [email protected]) Accepted 19-10-01 Figure 1 A longitudinal section of the uterus showing an endometrial polyp (P) measuring 8.2 mm in anteroposterior diameter.
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410 CASE REPORT
Ultrasound Obstet Gynecol 2002; 19: 410–412
Blackwell Science LtdIncidental ultrasound diagnosis of pseudomyxoma peritonei in an asymptomatic woman
S. KHAN*, A. G. PATEL† and D. JURKOVIC**Early Pregnancy and Gynaecology Assessment Unit and †Department of Surgery, King’s College Hospital, London, UK
An incidental finding of pseudomyxoma peritonei is reportedin a woman with a 6-month history of postmenopausalbleeding. A transvaginal ultrasound scan revealed a poorlydefined echogenic mass in the right iliac fossa above the rightovary and free fluid of mixed echogenicity in the pouch ofDouglas. Laparoscopic appendicectomy and aspiration ofmucinous fluid was performed without adjuvant chemo-therapy. Regular postoperative follow-up scans, which areneeded as the disease may have an indolent course, showedno signs of recurrence. The differential diagnosis and manage-ment of pseudomyxoma peritonei are discussed.
INTRODUCTION
Pseudomyxoma peritonei is characterized by an extensiveaccumulation of thick mucinous material within the perito-neal cavity. It is a rare condition that is slightly more commonin women1,2. Pseudomyxoma peritonei usually presents asacute appendicitis or as increasing abdominal distension.Alternatively, an ovarian mass in women or inguinal herniain men may be the initial presenting feature2.
In this report, we describe an incidental finding of pseu-domyxoma peritonei in a woman who attended for ultrasoundscan with a history of postmenopausal bleeding. Ultrasounddiagnosis and controversies in the treatment of this conditionare discussed.
CASE REPORT
A 49-year-old woman on sequential estrogen hormonereplacement therapy was referred for transvaginal ultrasoundexamination with a 6-month history of irregular vaginalbleeding. She became menopausal 3 years previously andhad been using hormone replacement therapy for 2 years. Shehad regular cervical smears, which were all normal. She hadthree children. Her first two pregnancies were uncomplicated,
whilst her last child was delivered by an emergency Cesareansection 8 years previously. Twenty years prior to presenta-tion, she had an open cholecystectomy. Her medical historywas otherwise unremarkable. Her maternal grandmotherhad died of ovarian carcinoma at the age of 97 years. Therewere no other cases of cancer in her family history.
General clinical and abdominal examination revealed noabnormalities. On speculum examination, the vagina and thecervix appeared healthy. On transvaginal ultrasound exam-ination, the uterus appeared normal. The endometrial thicknesswas 8.2 mm. The endometrium appeared irregular with ultra-sound features suggestive of an endometrial polyp (Figure 1).The ovaries appeared normal. A poorly defined echogenicmass, measuring 25 mm in diameter, was noted in the rightiliac fossa above the ovary (Figure 2). A considerable amountof gelatinous fluid of mixed echogenicity was seen in thepouch of Douglas (Figure 3). A presumptive diagnosisof pseudomyxoma peritonei due to ruptured appendixmucocele was made and the patient was booked for an urgentlaparoscopy and hysteroscopy. Preoperative hematological,
Correspondence: Dr D. Jurkovic, Early Pregnancy and Gynaecology Assessment Unit, Department of Obstetrics and Gynaecology, King’s College Hospital, Denmark Hill, London SE5 8RX, UK (e-mail: [email protected])
Accepted 19-10-01
Figure 1 A longitudinal section of the uterus showing an endometrial polyp (P) measuring 8.2 mm in anteroposterior diameter.
Ultrasound in Obstetrics and Gynecology 411
Pseudomyxoma peritonei Khan et al.
biochemical and serum tumor markers measurements wereall within normal ranges. A computerized tomography scanconfirmed the presence of fluid within the pelvis, which wasconsistent with possible pseudomyxoma peritonei.
At hysteroscopy, a small endometrial polyp was found inthe lower part of the uterine cavity for which an uneventfulpolypectomy was performed. Laparoscopy showed a normaluterus, Fallopian tubes and ovaries. Approximately 80 mL ofmucin was present in the pouch of Douglas. Mucus was seenoozing from a ruptured appendix mucocele measuring 3 cmin size. Additional deposits of mucoid material were identi-fied on the cecum, whilst the remaining bowel was normal.Laparoscopic appendicectomy was performed and mucinwas aspirated from the abdominal cavity. The patient’s post-operative recovery was uncomplicated and she was dis-charged home 2 days later.
Histopathological reports confirmed the diagnosis of abenign endometrial polyp. The examination of the appendix
showed a mucin-producing tumor arising from the appendi-ceal mucosa. The tumor was villous in architecture and therewas no evidence of invasion. The peritoneal fluid wasdescribed as stringy and mucoid and contained a number ofinflammatory cells. Malignant cells were not present.
Follow-up ultrasound examinations were carried out at 3,4 and 8 months. At the 8-month scan, there was no residualmucinous fluid left and the patient was advised to attend forregular yearly scans in the future.
DISCUSSION
Pseudomyxoma peritonei literally means a ‘false mucinoustumor of the peritoneum’. It is a rare condition in whichthere is an accumulation of gelatinous material within theperitoneum. Traditionally, the condition has been linkedto seeding of mucin-secreting cells from an ovarian tumor3.However, more recent evidence suggests that pseudomyxomaperitonei results from a ruptured appendiceal lesion, namelya simple mucocele, an appendiceal hyperplasia or an adenocar-cinoma. Any ovarian tumors present usually are the resultof a secondary spread from an appendiceal adenocarcinoma1.
The presentation of pseudomyxoma peritonei is variable. Inmany cases, the diagnosis of the condition has been made unex-pectedly at laparotomy, with the preoperative diagnosis beingeither an appendicitis or an ovarian tumor; hence preoperativediagnosis of this condition has rarely been made. With theadvent of non-invasive imaging, such as ultrasound, there is nowthe opportunity to establish the diagnosis preoperatively.
First reports on the ultrasound diagnosis of pseudomyxomaperitonei describe findings of multiple septations in the peri-toneal cavity surrounded by ascites4. Hann et al.5 subsequentlydescribed similar findings of multiloculated cystic masses whichfix the bowel posteriorly. Hinson and Ambrose1 describednon-mobile echogenic gelatinous ascites with ‘scalloping’ ofthe hepatic and splenic margins due to extrinsic pressure ofadjacent peritoneal implants.
Although ascites could be confused with pseudomyxomaperitonei, there are important differences: the bowel floatsfreely to the anterior abdominal wall and shifts with positionwhen surrounded by benign ascites. In pseudomyxomaperitonei, the gelatinous mass is fixed in position andseparates the bowel from the anterior abdominal wall4.Ascitic septations seen on ultrasound are not pathognomonicof pseudomyxoma peritonei, the differential diagnosis includeswidespread lymphoma, carinomatosis and pyogenic peri-tonitis. Gupta et al.6 described an unusual case of a primaryperitoneal mesothelioma with ultrasound findings mimick-ing pseudomyxoma peritonei. Ultrasound scanning can aidin the differentiation of lymphoma or carcinoma by demon-strating the absence of hepatic parenchymal involvement incases of pseudomyxoma peritonei4.
Most studies agree on the highly typical sonographicfindings of pseudomyxoma peritonei, such as scalloping ofthe liver margin and ascitic septations. However, one ultra-sound report on pseudomyxoma peritonei described thepresence of cystic balls with echogenic walls and an anechoicinterior within the abdominal cavity7. Another unusual casepresented with amorphous calcifications on the scan, which
Figure 2 A transverse section demonstrating a well-defined round lesion (M) above the right ovary. The lesion contained fluid of mixed echogenicity, while its wall was hyperechogenic. A presumptive diagnosis of an appendix mucocele was confirmed at surgery.
Figure 3 A transverse section through the pouch of Douglas showing a considerable amount of thick gelatinous fluid (F), which was the main ultrasound finding suggestive of pseudomyxoma peritonei.
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were widely distributed within the pelvis and upperabdomen8. It has also been suggested that benign andmalignant pseudomyxoma peritonei appear differently onultrasound: scalloping of the liver margins is often seen inadenomucinosis but not in adenocarcinomatosis7.
In our case, which should be classified as adenomucinosis,the only ultrasound finding suggestive of pseudomyxomawas the presence of gelatinous fluid within the pouch ofDouglas. The absence of other signs, such as scalloping,may be explained by the fact that the diagnosis was made inan asymptomatic patient, early in the course of disease, whenthe amount of mucin in the abdomen was very small.
The management of pseudomyxoma peritonei varies betweendifferent centers. Most groups would advocate thoroughsurgical debulking as well as consideration of the use of intra-peritoneal adjuvant therapy. Ultraradical surgery combinedwith intraoperative and further postoperative chemotherapyis recommended by some9 but not generally accepted. Recur-rence rates of pseudomyxoma peritonei are high (76% of allpatients) and the 5-year survival rate ranges between 86% inbenign lesions and 7% in adenocarcinomatosis9. Althoughphase III trials are not available to compare patients treatedby aggressive surgery with intraperitoneal chemotherapy tosimple debulking, phase II studies suggest a slight improve-ment in outcome with the more aggressive approach. How-ever, this benefit is offset by the high complication rate and5% direct mortality associated with the radical appproach9.
In conclusion, this case highlights an unusual presentationof pseudomyxoma peritonei and illustrates the possibility ofaccurate preoperative diagnosis by ultrasound. Early diagnosisof benign appendix mucocele enabled the use of minimallyinvasive surgery with a good short-term outcome. However,long-term follow-up is required in view of the significant riskof recurrence.
