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Challenging and Unusual Cases

CASE 3. PERITONEAL CYSTIC MESOTHELIOMA

The Washington Cancer Institute, Washington Hospital Center, Washington, DC; and the Department of Surgical Oncology, Institut Gustave Roussy, Villejuif, France

A 50-year-old man presented with diffuse abdominal discomfort and increasing distension. He had undergone umbilical herniorrhaphy 18 months previously and was found to have ascites. Subsequent laparoscopy revealed multiple cystic lesions that were biopsied, and histopathologic diagnosis of peritoneal cystic mesothelioma was confirmed by immunocytochemistry. Computed tomography scan of the abdomen showed massive accumulation of multiloculated ascitic fluid with compartmentalization of the small bowel (Fig 1). At surgical exploration, there was a conglomeration of cystic structures contained within the greater omentum ranging in size from 1 mm to 10 cm in diameter, filling the abdomen and pelvis. The greater omentum was most extensively involved (Fig 2). The parietal peritoneal surfaces contained small nodules that could be debrided away. The surface of the liver, gallbladder, and spleen were free of tumor. There was no evidence of soft tissue invasion.

View larger version (90K): [in this window] [in a new window]   Fig. 1. Computed tomography scan of the abdomen, showing the pathognomic apearance of cystic peritoneal mesothelioma. A conglomerate of multiple thin-walled cysts occupies the abdomino-pelvic space.

View larger version (110K): [in this window] [in a new window]   Fig. 2. Intraoperative photograph, showing characteristic thin-walled cysts in the pelvis.

View larger version (142K): [in this window] [in a new window]   Fig. 3. Histology showing variably sized coalescent cysts with fibro-collagenous walls and flattened cuboidal mesothelial cells.

The patient required a second surgery at 24 hours postoperatively for a bile leak from the liver edge, which was sutured. The patient remains free of disease after 5 years and has no sign of recurrence clinically or on recent CT scan.

Peritoneal cystic mesothelioma is a rare tumor for which pathophysiology remains equivocal and controversial. It occurs predominantly in young to middle-aged women; only 17% of these tumors occur in men. It is frequently associated with a positive history of previous abdominal surgery, pelvic inflammatory disease, and leiomyoma of the uterus. No racial, genetic, or environmental (such as asbestos exposure) influence has been reported. It is difficult to make a firm preoperative diagnosis of cystic mesothelioma. Computed tomography scan typically shows a well-defined, noncalcified, multiloculated mass. The cyst contents have a Hounsfield density similar to that of water. The cyst walls are thin and enhance with contrast.

Weiss and Tavassoli¹ reviewed 37 patient cases from the Armed Forces Institute of Pathology, Washington, DC, accumulated between the years 1972 and 1987. Although the cytopathology was bland and the inflammatory changes marked, they suggested that this entity be regarded as a neoplastic rather than a reactive process. They noted recurrences and occasional transition to conventional malignant mesothelioma.¹ Ross et al² reported 12 of 25 patients who developed a recurrence. Sometimes the lesions showed changes suggestive of malignant mesothelioma. Lack of follow-up prevented a prognostic assessment.² Immunohistochemical studies allow the separation of reactive and neoplastic lesions.³

Cystic mesotheliomas are thought to be benign because they do not metastasize; however, they have a high propensity for local recurrence.^4,5 Recurrence may occur in 27% to 75% of the patients within 3 months to 22 years of the initial resection.^6,7 The concept of peritoneal stripping for these patients was introduced by Datta and Paty.⁸ Combined modality treatment with cytoreductive surgery plus heated intraoperative intraperitoneal chemotherapy may minimize the high risk of recurrence. The use of heated intraoperative intraperitoneal chemotherapy (procedure is in trials) may eradicate free tumor cells that implant and then progress over an extended time. This strategy has been successful in the treatment of pseudomyxoma peritonei⁹ and may lead to prolonged disease-free survival for patients with cystic mesothelioma.

REFERENCES

1. Weiss SW, Tavassoli FA: Multicystic mesothelioma: An analysis of pathological findings and biologic behavior in 37 cases. Am J Surg Pathol 12:737–746, 1988[Medline]

2. Ross MJ, Welch WR, Scully RE: Multilocular peritoneal inclusion cysts (so-called cystic mesotheliomas). Cancer 64:1336–1346, 1989[CrossRef][Medline]

3. Cusatelli P, Altavilla G, Marchetti M: Benign cystic mesothelioma of peritoneum: A case report. Eur J Gynaecol Oncol 18:124–126, 1997[Medline]

4. O’Neill JD, Ros PR, Storm BL, et al: Cystic mesothelioma of the peritoneum. Radiology 170:333–337, 1989[Abstract/Free Full Text]

5. Schneider JA, Zelnick EJ: Benign cystic peritoneal mesothelioma. J Clin Ultrasound 13:190–192, 1985[Medline]

6. Katsube Y, Mukai K, Silverberg S: Cystic mesothelioma of the peritoneum: A report of 5 cases and review of literature. Cancer 50:1615–1622, 1982[CrossRef][Medline]

7. Miles JM, Hart WR, McMahon JT: Cystic mesothelioma of the peritoneum—Report of a case with multiple recurrences and review of the literature. Cleve Clin Q 53:109–114, 1986[Medline]

8. Datta RV, Paty PB: Cystic mesothelioma of the peritoneum. Eur J Surg Oncol 23:461–462, 1997[CrossRef][Medline]

9. Sugarbaker PH, Ronnett BM, Archer A, et al: Pseudomyxoma peritonei syndrome. Adv Surg 30:233–280, 1996[Medline]

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