Aug 15, 1983 - ondary to primary mucinous tumors of the appendix and ovary. ... mucinous cystadenoma. and mucinous cystadenocarcinoma of the appendix: ...
Pseudomyxoma Peritonei: Preoperative Diagnosis by Ultrasound and Computed Tomography A Case Report LUCY HANN, MD,'
SUSAN LOVE, MD,t AND RONALD P. GOLDBERG, MD'
Pseudomyxoma peritonei is an unusual condition caused by rupture of a rnucinous neoplasm. Correct preoperative diagnosis can now be made by use of ultrasound and computed tomography. Cancer 52:642-644, 1983.
P
is a rare condition caused by rupture of a primary mucinous malignant neoplasm, spilling mucin producing implants throughout the peritoneal cavity. In the past, preoperative diagnosis has been limited by nonspecific clinical and radiographic findings. We describe a case in which the newer imaging modalities of CT and ultrasound allowed us to make a correct preoperative diagnosis of pseudomyxoma peritonei.
He returned one year later feeling well. but complaining of increasing size of an umbilical hernia and of fullness in the lower abdomen. O n physical examination, a midline 4 X 2 cm mass was noted superficially between the umbilicus and pubis. Repeat barium enema and upper GI series were unchanged. Oltrasound examination showed a large lower abdominal mass (Figs. I A and IB). hut extension into the rectovesical space was less marked than on previous study. Computed tornography confirmed the ultrasound findings showing a large lowdensity mass surrounding the rectum and extending into the pelvis and midabdomen (Fig. 2). Fluid or mass was demonstrated adjacent t o the liver, scalloping its lateral and posterior margins (Fig. 3). Rased on the C T and ultrasound findings, a diagnosis was made of pseudomyxoma peritonei secondary to ruptured appendiceal neoplasm. Gelatinous ascites, omental and peritoneal mucinous implants and a perforated appendiceal adenocarcinoma wcrc found at subsequent laparotomy (Fig. 4).
SEUDOMYXOMA PERITONEI
Case Report A 36-year-old white man experienced severe lower abdominal pain. vomiting, diarrhea, and fever. Fever and abdominal pain persisted for three weeks, with the development o f a lower abdominal mass, and he was admitted to the hospital. Physical examination revealed a mass extending from the pubis to the umbilicus. The mass was palpable and tender on rectal examination. An ultrasound study revealed a multicystic septated mass arising posterior to the bladder and extending into the midahdomen. Imxlated fluid was noted surrounding the right lobe of the liver and spleen. Barium enema and upper gastrointestinal study (GI) showed a large extrinsic mass displacing the bowel from the pelvis. N o intrinsic bowel masses were seen. The terminal ileum was normal. There was n o visualization of the appendix. With the diagnosis of an appendiccal phlegmon. the patient was placed on a course of antibiotics during which he defervcsced and the mass clinically disappeared. The patient refused interval appendectomy and was lost to follow-up.
Discussion Pseudomyxoma peritonei results from gelatinous mucinous peritoneal implants and ascites. usually secondary to primary mucinous tumors of the appendix and ovary. Although originally benign and malignant mucin producing neoplasms were considered as causes of pseudomyxoma peritonei, true implantation of epithelial cells with peritoneal m u c h production is found only in the malignant adenocarcinoma.'.' Despite extensive spread to the omentum, peritoneum and abdominal contents, visceral invasion is extremely rare. Clinical symptoms and signs are nonspecific. Patients may present with abdominal mass, distention or hernia. Occasionally, acute abdomen or intestinal obstruction Prior to the may be the initial clinical advent of computed tomography and ultrasound, radiographic study could demonstrate only a mass extrinsic to bowel and did not enable specific preoperative diagnosis.
~
From the Departments of Radiology* and Surgery.t Beth Israel Hospital. Harvard Medical School, 330 Brookline Avenue. Boston. Massachusetts. Address for reprints: Lucy Hann. MD. Department of Radiology. Beth Israel Hospital, Harvard Medical School. 330 Brookline Ave.. Boston. MA 022 15. Accepted for publication May 14, 1982.
0008-543X/83/08 1510642 $0.95 0 American Cancer Society
642
No. 4
-
PSEUDOMYXOMA PERITONEI Hunn et al.
643
FIG.3. CT scan of the upper abdomen shows the liver margin scalloped laterally and posteriorly (arrows) by the low density mass indicating local pressure effect from the pseudomyxoma implants against the hepatic parenchyma.
In this condition, ultrasound demonstrates multiloculated cystic masses in the abdomen displacing bowel.6 While lymphoma may enter the differential diagnosis. such solid masses are not as compressible during ultraFIGS. 1 A A N D I B. (A, top) Longitudinal sonogram demonstrates a multiloculated solid and cystic mass (arrows) arising posterior and cephalad to the bladder. (B. bottom) Transversely. 2 cm helow the umbilicus the mass (arrows) entirely fills the peritoneal cavity and displaces the bowel out of the pelvis.
FIG. 2. CT scan of the pelvis demonstrates a mass of low attenuation abutting the rectum and extending far anteriorly (arrows). The ureters are laterally positioned.
FIG. 4. Gross specimen of omentum shows multiple clusters of metastatic mucus filled globules of varying size studding the omental surface.
CANCERArcgirsi 15 1983
644
sound examination. CT findings suggesting the correct preoperative diagnosis include large low-density intraperitoneal masses and scalloping of the liver by gelatinous Although ascites could be confused with pseudomyxoma peritonei. there are important differential characteristics. Bowel floats freely to the anterior abdominal wall and shifts with position when surrounded by benign ascites. In pseudomyxoma peritonei, the gelatinous mass is fixed in position and anterior between’ bowel and peritoneum. Malignant or loculated ascites may mat the bowel posteriorly but does not scallop the liver contour. There are two distinguishing features of pseudomyxoma peritonei: ( I ) multiple rounded fluid-filled masses surrounding and displacing bowel as demonstrated by ultrasound: and (2) indentation of the liver edge, better seen on CT. This case illustrates the usefulness of CT and ultrasound in evaluation prior to surgery so that appropriate surgical intervention can be planned preoperatively.’.’-’
’
Vol. 52
REFERENCES I . Higa E. Rosai J , Piitimhono CA, Wise I.. M u c m l hyperplasia. mucinous cystadenoma. and mucinous cystadenocarcinoma of the appendix: A reevaluation of appendiceal “mucwele”. Ciinwr 1973; 32: 1525- 1541. 2. Fernandci RN. Daly JM. Pseudomyxoma peritonei. Arch Sttrg 1980; I l5:409-413. 3. Limher CiK, King RE. Silverhcrg SG. Pseudomyxoma peritonaei: A report of ten cases. Ann Surg 1972; 178(5):587-593. 4. Aho AJ. Heinonen R, Lauren P. Benign and malignant mucocele of the appendix. Acm Chir Sciind 1973: 139:392-400. 5. Parsons J, Gray G, Thorbjarnarson R. Pseudomyxoma peritonei. :lrch Sirrg 1970: 101(5):545-549. 6. Menitt CB. Williams SM. Ultrasound findings in a patient with pseudomyxoma peritonei. J Clin Lhzsound 1978; 6:4 17-4 18. 7. Seshul MB, Coulam CM. Pseudomyxoma peritonei: computed tomography and sonography. Am .I Rounlgenol 198I: 136:803-806. 8. Mayes GR, Chuang VP, Fisher RG. CT of pseudomyxoma peritonei. A m .I Roenrgcwol 1981: 136:807-808. 9. Wolff M, Ahmed N. Epithelial neoplasms of the vermiform a p pendix (exclusive of carcinoid). I: Adenocarcinoma of the appendix. Cnncw 1976; 37:2493-75 10. 10. Long RTL. Spratt jS. Dowling E. Pseudomyxoma perilonei: New concepts in management with a report ofseventeen patients. Am J Sirrg 1969: 1 17: 162- 168. 11. Ghosh RC. Huvos AG, Whiteley HW. Pseudomyxoma peritonei. Dis Colon R t w i m 1972: 15(6):420-425.
