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  Volume 95
Numero 6
novembre-dicembre 2009 		I documenti sono in formato PDF, consultabili utilizzando Acrobat Reader
 
Benign multicystic peritoneal mesothelioma associated with hydronephrosis and colovesical fistula formation: report of a case

James C McCaffrey, Fung J Foo, Neha Dalal, Kamran H Siddiqui
1Department of General Surgery, 2Department of Histopathology, Tameside General Hospital, Manchester, UK

Key words: benign multicystic peritoneal mesothelioma, hydronephrosis, fistula.

abstract

Mesotheliomas usually arise from the pleura and are malignant. We report an unusual case of benign peritoneal mesothelioma presenting in a 59-year-old woman. The disease resulted in bilateral hydronephrosis, colovesical fistula formation, recurrent small bowel obstruction and chronic abdominal pain. To date only a handful of cases have been reported and to the best of our knowledge, none has been so aggressive. 

Introduction
Benign multicystic peritoneal mesothelioma (BMPM) is a rare type of tumour arising from the peritoneal mesothelium and was first described in 1979 by Mennemeyer and Smith1. It usually occurs in young to middle-aged women with a history of pelvic surgery2, endometriosis3,4 or pelvic inflammatory disease5,6. The aetiology of the condition is controversial, with ongoing discussion as to whether the disease process is primarily reactive or neoplastic.
Although BMPM is associated with a favourable short-term prognosis, and was considered to be a completely benign condition, longer-term follow-up has shown high recurrence rates after surgery6,7. There is also mounting evidence that malignant transformation can occur8. No consensus on treatment strategy has yet been reached.
The disease typically follows a slow, progressive course and usually results in chronic abdominal pain. To the best of our knowledge, this is the first reported case of BMPM with such an aggressive nature resulting in bilateral hydronephrosis, colovesical fistula formation, and recurrent small bowel obstruction.

Case report
A 59-year-old woman was admitted with worsening left iliac fossa pain. She had a 6-month history of generalized abdominal discomfort associated with episodes of diarrhoea and 30 kilograms of weight loss. There was no history of prior abdominal or gynaecological surgery, pelvic inflammatory disease, endometriosis or asbestos exposure. Clinical examination revealed a left iliac fossa mass.
CT scan showed multiloculated fluid collection with gas between the bowel loops extending from the lower abdomen into the pelvic cavity posteriorly (Figure 1). High-density contrast in the margins of bowel loops suggestive of diverticular disease was identified. Bilateral hydronephrosis was also noted.



The abdominal mass was initially thought to be a diverticular abscess and the patient was managed conservatively with intravenous antibiotics. When her condition failed to improve, an exploratory laparotomy was performed. This revealed a large cystic mass in the pelvis which had encased the sigmoid colon and rectum. The mass was fixed to the pelvic walls and was unresectable. The uterus and ovaries could not be visualised and ovarian malignancy was suspected. A defunctioning loop colostomy was performed and multiple pelvic biopsy and cytology samples were obtained.
Histology on the endometrial and cervical tissue showed no evidence of neoplasia. Biopsy from the pelvic tumour itself confirmed benign cystic mesothelioma of the peritoneum (also referred to as peritoneal inclusion cysts) (Figure 2). A similar mesothelial inclusion cyst was identified within the omentum. The bilateral hydronephrosis identified was managed by means of ureteric stenting.



Six years after the diagnosis of BMPM, the woman re-presented with small bowel obstruction. Initial conservative management failed and a laparotomy with adhesiolysis was performed. The intraoperative findings showed aggressive BMPM encasing the pelvic organs.
The patient also began experiencing signs and symptoms of a colovesical fistula, which was confirmed by cystoscopy. A decision against definitive surgical intervention was made, given the findings from the previous laparotomy.
She continued to suffer recurring episodes of small bowel obstruction, which have so far responded to conservative treatment. Urinary tract infections associated with her colovesical fistula have been controlled with low-dose prophylactic oral antibiotics.

Discussion
Mesotheliomas are tumours arising from the epithelial and mesenchymal elements of the mesothelium. The majority of mesotheliomas arise in the pleura, but rare cases of peritoneal2, pericardial9, and tunica vaginalis10 mesotheliomas have also been reported. The ratio of the occurrence of peritoneal to pleural tumour is 1:911. Most mesotheliomas are malignant. Hence, a benign mesothelioma arising from the peritoneum is an extremely rare clinical entity.
BMPM usually arises in young to middle-aged women (mean 37 years)2, although 17% of cases are reported in men12. Typical presentation is with chronic lower abdominal pain and evidence of a pelvic mass. A history of pelvic surgery2, endometriosis3,4 or pelvic inflammatory disease5,6 is common. BMPM has also been reported to cause partial small bowel obstruction13 and acute abdomen7.
Disagreement regarding the aetiology of BMPM persists. One hypothesis, supported by the association with previous surgical intervention or chronic infection, suggests a reactive inflammatory aetiology. The other hypothesis suggests BMPM is a neoplastic process. Most current evidence supports the latter hypothesis, with reports of malignant transformation of BMPM8, and high post-surgical recurrence rates.
Treatment of BMPM is another area of controversy. Currently, palliative surgical debulking with reoperations for recurrence is the mainstay of treatment. However, some have advocated sclerosive therapy with tetracycline14, continuous hyperthermic peritoneal perfusion with cisplatin15, and antioestrogenic drugs16.
Surgical debulking alone is unsatisfactory for several reasons: i) recurrence rates of 50% are reported6,7;
ii) malignant transformation of the lesion can occur8 and; iii) patients continue to suffer chronic abdominal and pelvic pain, which tends to be poorly controlled with analgesics17.
 Sethna et al.17 advocate a more aggressive approach aiming for complete disease eradication using cytoreductive surgery with peritonectomy procedures and heated intraoperative intraperitoneal chemotherapy. They describe 5 patients with BMPM who had undergone multiple laparotomies prior to the definitive cytoreduction. All the patients were asymptomatic and 4 out of 5 were disease free with variable follow-up post-cytoreduction ranging from 6 to 69 months.
A review of cases shows that BMPM typically progresses slowly and is not locally invasive. To the best of our knowledge, this is the first case report of BMPM associated with hydronephrosis and colovesicular fistula formation. It highlights the current difficulties faced in managing patients with this condition and urges caution in the assessment of BMPM as a benign process.

References
 1. Mennemeyer R, Smith M: Multicystic, peritoneal mesothelioma: a report with electron microscopy of a case mimicking intra-abdominal cystic hygroma (lymphangioma). Cancer, 44: 692-698, 1979.
 2. McFadden DE, Clement PB: Peritoneal inclusion cysts with mural mesothelial proliferation. A clinicopathological analysis of six cases. Am J Surg Pathol, 10: 844-854, 1986.
 3. Groisman GM, Kerner H: Multicystic mesothelioma with endometriosis. Acta Obstet Gynecol Scand, 71: 642-644, 1992.
 4. Urbanczyk K, Skotniczny K, Kucinski J, Friediger J: Mesothelial inclusion cysts (so-called benign cystic mesothelioma)-a clinicopathological analysis of six cases. Pol J Pathol, 56: 81-87, 2005.
 5. Tangjitgamol S, Erlichman J, Northrup H, Malpica A, Wang X, Lee E, Kavanagh JJ: Benign multicystic peritoneal mesothelioma: cases reports in the family with diverticulosis and literature review. Int J Gynecol Cancer, 15: 1101-1107, 2005.
 6. Ross MJ, Welch WR, Scully RE: Multilocular peritoneal inclusion cysts (so-called cystic mesotheliomas). Cancer, 64: 1336-1346, 1989.
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 8. González-Moreno S, Yan H, Alcorn KW, Sugarbaker PH: Malignant transformation of “benign” cystic mesothelioma of the peritoneum. J Surg Oncol, 79: 243-251, 2002.
 9. Drut R, Quijano G: Multilocular mesothelial inclusion cysts (so-called benign multicystic mesothelioma) of pericardium. Histopathology, 34: 472-474, 1999.
10. Lane TM, Wilde M, Schofield J, Trotter GA: Benign cystic mesothelioma of the tunica vaginalis. BJU Int, 84: 533-534, 1999.
11. Spirtas R, Beebe GW, Connelly RR, Wright WE, Peters JM, Sherwin RP Henderson BE, Stark A, Kovasznay BM, Davies JN: Recent trends in mesothelioma incidence in the United States. Am J Ind Med, 9: 397-407, 1986.
12. Weiss SW, Tavassoli FA: Multicystic mesothelioma. An analysis of pathologic findings and biologic behavior in 37 cases. Am J Surg Pathol, 12: 737-746, 1988.
13. Enzinger FM, Weiss SW: Mesothelioma. In: Soft tissue tumours, Enzinger FM, Weiss SW (Eds), pp 689-718, CV Mosby, St. Louis, 1988.
14. Benson RC Jr, Williams TH: Peritoneal cystic mesothelioma: successful treatment of a difficult disease. J Urol, 143: 347-348, 1990.
15. Park BJ, Alexander HR, Libutti SK, Wu P, Royalty D, Kranda KC, Bartlett DL: Treatment of primary peritoneal mesothelioma by continuous hyperthermic peritoneal perfusion (CHPP). Ann Surg Oncol, 6: 582-590, 1999.
16. Letterie GS, Yon JL: The antiestrogen tamoxifen in the treatment of recurrent benign cystic mesothelioma. Gynecol Oncol, 70: 131-133, 1998.
17. Sethna K, Mohamed F, Marchettini P, Elias D, Sugarbaker PH: Peritoneal cystic mesothelioma: a case series. Tumori, 89: 31-35, 2003.



 
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