CASE REPORT

A Recurrence of Benign Multicystic Peritoneal Mesothelioma Treated Through Laparoscopic Excision: A Case Report and Review of the Literature Tadeusz D. Witek, BS,* Joseph W. Marchese, BS, MPH,* and Timothy J. Farrell, MD*w

Abstract: Benign multicystic peritoneal mesothelioma is a rare cystic tumor with approximately 140 cases reported to date. It is found mainly in women of reproductive age and is commonly attached to the serosal surface of pelvic viscera. The pathogenesis is uncertain and debate regarding its neoplastic or reactive nature exists. The presenting symptoms are often nonspecific and most commonly include vague lower abdominal pain and fullness. Imaging studies are used to identify the lesion, but histologic studies with the aid of immunohistochemical techniques are needed for definitive diagnosis as the differential diagnosis is quite extensive. Surgical resection is the primary form of treatment, and follow-up is important due to the high rate of recurrence and the rare possibility of malignant transformation. Here, we present a case of a 50-year-old female with a recurrence of benign multicystic peritoneal mesothelioma treated through laparoscopic excision and also a review of the literature. Key Words: ascites, cystic mesothelioma, peritoneal mesothelioma, laparoscopic removal, peritonectomy

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enign multicystic peritoneal mesothelioma (BMPM) is a rare cystic neoplasm most commonly found attached to the serosal surface of pelvic viscera. The disease is typically found in women of reproductive age1–2 and most of the time it is found incidentally.1 When on occasion it does produce symptoms, they are usually vague but can also present with an extensive range of symptoms, most commonly nausea, bloating, and vomiting. Imaging studies are usually able to identify the lesion but histologic studies are needed for definitive diagnosis. Approximately 140 cases have only been reported.3 Because of its rarity, there is inadequate knowledge of the entire spectrum of BMPM. Although its origin is known, the etiology is still debated.4–8 There are a number of other lesions that can present similar to BMPM, and it is important to differentiate between them. Surgical removal is the main form of treatment but other forms of therapy are being attempted. Follow-up is important because of the high rate of recurrence4,9 as well as possible, although rare, malignant transformation.10,11 We present a case of a 50-year-old female with a recurrence of BMPM and also a review of the literature. Received for publication September 16, 2012; accepted October 16, 2012. From the *The Commonwealth Medical College; and wGeisingerCommunity Medical Center, Scranton, PA. No funding was received from the National Institutes of Health (NIH); Wellcome Trust; Howard Hughes Medical Institute (HHMI), etc. The authors declare no conflicts of interest. Reprints: Tadeusz D. Witek, BS, The Commonwealth Medical College, 525 Pine Street, Scranton, PA 18509 (e-mail: [email protected]). Copyright r 2014 by Lippincott Williams & Wilkins

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CASE HISTORY A 50-year-old woman presented with intermittent right-sided abdominal pain of 6 months duration. She had undergone diagnostic laparoscopy as well as appendectomy and laparotomy 3 years prior for what proved to be a benign multicystic mesenteric mesothelioma of the peritoneum located posterior to the right colon. This new onset of pain resembled the former suggesting a recurrence of intra-abdominal mesothelioma. No other symptoms were reported. On examination, the abdomen showed sharp, right-sided tenderness with an associated fullness. There was no hepatosplenomegaly or ascites. Serum chemistry was normal. Ultrasound demonstrated a 6.3 6.5 3.0 cm cystic lesion inferior to the right kidney that was not noted on CAT scan 3 years prior. Repeat CAT scan of the pelvis showed multiloculated fluid collections along the right side of the ascending colon approaching the hepatic flexure (Fig. 1A). There was also a complex cystic mass intimately associated with the hepatic flexure (Fig. 1B). During diagnostic laparoscopy and excision, small areas of recurrent cystic masses were identified on the ascending colon (Fig. 2) in addition to a new cystic mass located below the liver edge and lateral to the hepatic flexure. All tumors were freely dissected. The patient made an uneventful postoperative recovery and has remained asymptomatic with no recurrence 5 months after surgery. Grossly, the specimen showed a large gelatinous cystic mass containing smaller cystic spaces. Cut surfaces revealed multiple, thin-walled, fluid-filled cystic spaces. Microscopically, the tissue contained numerous cysts of various sizes separated by fibrous stroma with scattered lymphocytes and few hemosiderin macrophages. The cells lining the cysts ranged from flat to low cuboidal resembling a hob-nail appearance (Fig. 3). Immunohistochemically, these cells stained positively for calretinin and cytokeratins. The final diagnosis was recurrent BMPM.

DISCUSSION BMPM is a rare medical entity. It was first described in 1979 by Mennemeyer and Smith.12 Currently, approximately 140 cases have been reported. Most of the information that is known has been compiled from isolated case reports. Because of its rarity and the absence of thorough investigations, discrepancies and challenges exist in the origin, pathogenesis, diagnosis, and therapy of BMPM.13 BMPM occurs predominately in females of reproductive age,1–2 although cases have been reported in the elderly3,14 and rare cases reported in males.15,16 Unlike pleural mesothelioma, no relationship has been found between BMPM and asbestosis.17 The most common presenting symptoms are vague lower abdominal pain and fullness. Several reports have shown BMPM to present as an acute abdomen.13,18 Sometimes BMPM may present with an abdominal mass and also with obstructive symptoms such as nausea, bloating, or vomiting. However, this condition is most commonly diagnosed incidentally upon laparotomies for other reasons,1 with 1 case presenting as LLQ pain and

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FIGURE 1. A, Cystic lesions associated with the ascending colon and (B) approaching the hepatic flexure.

pneumoperitoneum on imaging.19 Current available modern imaging techniques, such as ultrasonography, computerized tomography, and magnetic resonance imaging, can demonstrate the lesion. Other diseases that present as cystic neoplastic or inflammatory lesions, especially in similar anatomic areas, can make a differential diagnosis difficult.20–22 Preoperative fine-needle aspiration biopsy of cystic lesions may help to formulate a differential diagnosis15,23,24 but confirmation of the diagnosis is usually made by electron microscopy and immunohistochemistry.25 BMPM arises from epithelial and mesenchymal elements of the mesothelial cells and has not been shown to metastasize.26 The tumor has a strong predilection for the surface of the pelvic viscera and has been shown to attach to the serosal surface of the intestine and omentum. Several cases have also involved the retroperitoneal space and occasionally the small intestine, spleen, liver, kidney, bladder, previous scars, or the appendix.1–3,27,28 The tumor is composed of mesothelial-lined, fluidfilled cystic spaces separated by fibrous stroma. The cysts range in size from several millimeters to 20 cm in diameter and are filled with mucinous or gelatinous fluid.29 The mesothelial cells occasionally have a “hob-nail”

FIGURE 2. Cystic masses on ascending colon. r

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appearance, as seen in our case. The linings of the cells can undergo adenomatoid or squamous metaplasia as seen in up to one third of cases.4,13 Controversy exists regarding the pathogenesis of BMPM with uncertainty of whether the process is neoplastic or reactive.5,9,17–19 Several common features such as history of previous surgery, endometriosis, uterine leiomyoma, and inflammation suggest a reactive process to a chronic irritant. The reactive process involves a peritoneal reaction with mesothelial cell entrapment, reactive proliferation, and cystic formation.22 Microscopically, an inflammatory component is seen in many cases. BMPM has also been associated with familial Mediterranean fever, characterized by periodic fever and peritonitis, further supporting this theory.29 In contrast, the neoplastic theory is based on features such as the slow but progressive growth of untreated lesions, tendency toward recurrence, a low incidence of previous abdominal infection, and a high disease-related mortality.4,5 In addition, the malignant transformation of BMPM indicates a neoplastic phenomenon as well as the necessity of long-term follow-up.10 Also of note, with the great majority of patients being women of

FIGURE 3. Flat to cuboidal cells lining the cysts resembling a hob-nail appearance.

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reproductive age, female sex hormones may play a key role in the pathogenesis.30 The differential diagnosis includes a number of benign and malignant lesions that present as cystic or multicystic abdominal masses. Benign lesion include cystic lymphangioma (cystic hygroma),5,31 cystic adenomatoid tumors,32 cystic forms of endosalpingiosis,33,34 endometriosis,35 Mullerian cysts involving the retroperitoneum,36 and cystic mesonephric duct remnants.4 Of the benign lesions, it is important to differentiate BMPM from cystic lymphangioma and adenomatoid tumors. Cystic lymphangioma is more common in young males. The cystic component is often chylous on gross examination, and on microscopic examination lymphoid aggregates and smooth muscle are present. The cystic spaces are lined by a single layer of flattened endothelial cells which are immunoreactive to vascular markers (CD31, CD34, factor VIII, and VEGFR3).13 Cystic adenomatoid tumors can also be easily confused with BMPM. The main difference is the identification of a recognizable solid element in the cystic component of the adenomatoid tumor. There are cases of tumors with mixed features of both37,38 indicating that the 2 lesions are probably pathologically related. Histochemical staining can be used to further differentiate. Positive staining with calretinin, vimemtin, and Wilms tumor antigen, and negative staining for Ber-EP-4, carcinoembryonic antigen, and CD15 indicate mesothelial lineage.9 Cystic forms of endosalpingiosis differ from BMPM in their histologic makeup. They are made up of tubal-type epithelium that may include peg cells, ciliated cells, and/or secretory cells. They may also include blunt papillae and psammoma bodies.13 Endometrial cysts usually contain a dark-colored material and are composed of endometrial stroma lined by endometrial-type epithelium.13 A Mullerian cyst, as name implies, is composed of mullerian-type serous and mucinous epithelium. Smooth muscle fibers can be seen in their walls.13 The differential diagnosis for malignant lesions is not as lengthy but still needs to be considered. Malignant conditions that mimic BMPM include malignant mesothelioma and serous tumors of the peritoneum.7 Features that are common in malignancies such as, cellular atypia, increased mitotic count, abnormal mitoses, and destructive infiltration of the underlying stroma, can be used to differentiate it from BMPM.13 Multiple plaques and nodules with ascites can also be a finding associated with malignant mesothelioma.9 Regardless of the etiology, whether benign or malignant, electron microscopy and immunohistochemistry are needed to confirm the diagnosis.25 At this point, complete surgical resection of the cystic lesion is the mainstay of treatment for BMPM. To prevent progression or recurrence, aggressive surgical approaches including cytoreductive surgery with peritonectomy are recommended.39 Laparoscopic approaches, as performed in our patient, have been attempted.40,41 Currently adjuvant chemotherapy and radiotherapy are not indicated due to the benign character of the disease. However, several other therapies were attempted in individual cases including sclerosive therapy with tetracycline, continuous hyperthermic peritoneal perfusion with cisplatin, and peritonectomy with intraperitoneal chemotherapy. These have varied in their degrees of success. Case series have been published showing the effectiveness of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for treatment of BMPM. The use of this therapy demonstrated a relatively lower recurrence rate at 16.7% compared with standard surgical therapy (41.7% to 50%). However, the

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results are not significant due to the small number of patients in the individual cases series (cumulative total = 12).3,39,42–44 BMPM does carry a tremendous prognosis with only 1 death ever reported—a patient who refused to undergo resection 12 years after diagnosis.4 Even with removal of the entire tumor, recurrence is still high. Reports show that about half of the patients experience recurrence, as was the case with our patient.4,9 Even with recurrence, the rate of transforming into a malignancy is extremely low, with 2 cases of malignant transformation ever reported.10–11 Extended and perhaps even lifelong, systematic follow-up of these patients is required as, just as in our patient, the lesions tend to reappear, requiring further resection or other therapy. With approximately 140 cases of BMPM previously described in the literature, and without a consensus of agreement of its etiology and therapy, further reports are not only needed, but will continue to benefit.

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17. Ruth SV, Bronkhosrt MW, Coevorden FA, et al. Peritoneal benign cystic mesothelioma: a case report and review of the literature. Eur J Surg Oncol. 2002;28:192–195. 18. Pitta X, Andreadis E, Ekonomou A, et al. Benign multicystic peritoneal mesothelioma: a case report. J Med Case Rep. 2010; 4:385. 19. Snyder J, Carman R Jr, Aggon A, et al. Benign multicystic peritoneal mesothelioma: a rare case presenting as pneumoperitoneum and pneumotosis intestinalis. J Gastrointest Oncol. 2011;2:55–58. 20. Romero JA, Kim EE, Kudelka AP, et al. MRI of recurrent cystic mesothelioma: differential diagnosis of cystic pelvic masses. Gynecol Oncol. 1994;54:377–380. 21. Ozgen A, Akata D, Akhan O, et al. Giant benign cystic peritoneal mesothelioma: US, CT, and MRI findings. Abdom Imaging. 1998;23:502–504. 22. Abdullahi H, Fawzi H. Gynaecological presentation of benign multicystic mesothelioma. J Obstet Gynaecol. 2003;23:576–578. 23. Tao LC. Aspiration biopsy cytology of mesothelioma. Diagn Cytopathol. 1989;5:14–21. 24. van Ruth S, Bronkhorst MW, van Coevorden F, et al. Peritoneal benign cystic mesothelioma: a case report and review of the literature. Eur J Surg Oncol. 2002;28:192–195. 25. Petrou G, Macindoe R, Deane S. Benign cystic mesothelioma in a 60-year-old woman after cholecystectomy. ANZ J Surg. 2001;71:615–618. 26. U¨zu¨m N, O¨zc¸ay N, Ataoglu O¨. Benign multicystic peritoneal mesothelioma. Turk J Gastroenterol. 2009;20:138–141. 27. Cuartas JE, Maheshwari AV, Qadir R, et al. Benign multicystic peritoneal mesothelioma in a cesarean-section scar presenting as a fungating mass. Int J Clin Oncol. 2008;13: 275–278. 28. Bansal A, Zakhour HD. Benign mesothelioma of the appendix: an incidental finding in a case of sigmoid diverticular disease. J Clin Pathol. 2006;59:108–110. 29. Curgunlu A, Karter Y, Tufekci IB, et al. Benign cystic mesothelioma: a rare cause of ascites in a case with familial Mediterranean fever. Clin Exp Rheumatol. 2003;21:S41–S43. 30. Sawh RN, Malpica A, Deavers MT, et al. Benign cystic mesothelioma of the peritoneum: a clinicopathologic study of 17 cases and immunohistochemical analysis of estrogen and progesterone receptor status. Hum Pathol. 2003; 34:369–374.

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A recurrence of benign multicystic peritoneal mesothelioma treated through laparoscopic excision: a case report and review of the literature.

Benign multicystic peritoneal mesothelioma is a rare cystic tumor with approximately 140 cases reported to date. It is found mainly in women of reprod...
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