Imaging appearance of benign multicystic peritoneal mesothelioma: a case report and review of the literature

Imaging appearance of benign multicystic peritoneal mesothelioma: a case report and review of the literature

    Imaging appearance of benign multicystic peritoneal mesothelioma: a case report and review of the literature Varun Mehta, Varun Chowd...

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    Imaging appearance of benign multicystic peritoneal mesothelioma: a case report and review of the literature Varun Mehta, Varun Chowdhary, Richa Sharma, Jennifer S Golia Pernicka PII: DOI: Reference:

S0899-7071(16)30148-6 doi: 10.1016/j.clinimag.2016.10.008 JCT 8128

To appear in:

Journal of Clinical Imaging

Received date: Revised date: Accepted date:

7 July 2016 30 September 2016 14 October 2016

Please cite this article as: Mehta Varun, Chowdhary Varun, Sharma Richa, Golia Pernicka Jennifer S, Imaging appearance of benign multicystic peritoneal mesothelioma: a case report and review of the literature, Journal of Clinical Imaging (2016), doi: 10.1016/j.clinimag.2016.10.008

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Imaging Appearance of Benign Multicystic Peritoneal Mesothelioma: A Case Report and Review of the Literature

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Varun Mehta, MD; Varun Chowdhary, MD; Richa Sharma, MD; Jennifer S Golia Pernicka, MD

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Affiliation: Department of Radiology, Staten Island University Hospital, Northwell Health New York City, NY 10305

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Corresponding Author: Varun Mehta, MD Radiology Resident Department of Radiology, Staten Island University Hospital Northwell Health 475 Seaview Ave Staten Island, NY 10305, USA Phone: +1-718-226-8297 Fax: +1-718-226-8335 Email: [email protected]

Other Authors:

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Varun Chowdhary, MD Radiology Resident Department of Radiology, Staten Island University Hospital Northwell Health 475 Seaview Ave Staten Island, NY 10305, USA Phone: +1-718-226-8297 Fax: +1-718-226-8335

Jennifer S. Golia Pernicka, MD Radiology Attending Department of Radiology, Staten Island University Hospital Northwell Health 475 Seaview Ave Staten Island, NY 10305, USA Phone: +1-718-226-8297 Fax: +1-718-226-8335

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Richa Sharma, MD Radiology Attending Department of Radiology, Staten Island University Hospital Northwell Health 475 Seaview Ave Staten Island, NY 10305, USA Phone: +1-718-226-8297 Fax: +1-718-226-8335

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Conflict of Interest: Varun Mehta declares that he has no conflict of interest. Varun Chowdhary declares that he has no conflict of interest. Richa Sharma declares that she has no conflict of interest. Jennifer S Golia Pernicka declares that she has no conflict of interest.

ACCEPTED MANUSCRIPT 1.0 Introduction

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Primary tumors of the peritoneum are rare lesions that arise from the mesothelial or

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submesothelial peritoneal layers. Such entities include primary malignant mesothelioma, benign multicystic mesothelioma, primary peritoneal serous carcinoma, leiomyomatosis peritonealis

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disseminate, and desmoplastic small round cell tumor [1]. Benign multicystic peritoneal mesothelioma (BMPM) is a rare benign entity most frequently reported in women of

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reproductive age [1, 3]. BMPM arises from the mesothelial layer of the serosal surface with a

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predilection for the pelvis. It usually has the imaging appearance as a multiseptated cystic mass [1, 5, 6]. These lesions are often discovered secondary to symptoms related to the mass effect of the tumor [1, 2, 6]. En bloc surgical removal is the best treatment strategy for BMPM [2].

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Despite being benign, there is a high rate of recurrence after complete resection [1]. The precise

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pathophysiology of BMPM is not well understood and it is debated these tumors are result of a

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2.0 Case Report

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reactive process following a primary injury versus a neoplastic process [12, 16].

A 22 year-old G1P1001 female without any significant past medical history presented to our Emergency Department after 14 days of progressively worsening postpartum right lower quadrant pain and nausea. She denied any associated fevers, chills, vomiting, vaginal bleeding, discharge, or odor. At the time of arrival she was afebrile and initial lab work, including a complete blood count and urinalysis, were within normal limits.

Imaging included a pelvic ultrasound, a CT of the abdomen and pelvis, and an MRI. The transabdominal ultrasound demonstrated a lobulated multicystic mass surrounding the right

ACCEPTED MANUSCRIPT adnexa (Figures 1). CT showed cystic masses along both paracolic gutters, abutting the colon

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as well as the lobule in the anterior omentum (Figure 2).

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The MRI demonstrated multiple well-defined, lobulated cystic masses with enhancing septations surrounding the adnexa bilaterally and extending superiorly along the paracolic gutters, anterior

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to the ascending and descending colon, respectively. A few isolated similar-appearing lesions anteriorly along the omentum below the umbilicus and posteriorly in the rectouterine pouch were

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also noted. A few of these lobulated lesions demonstrated T1 hyperintensity suggestive of

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hemorrhage. The ovaries were normal bilaterally (Figures 3 and 4).

The patient subsequently underwent surgical resection of the BMPM at an outside facility and

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reportedly had a favorable outcome. The pathological evaluation of the tissue sample

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demonstrated the cells lining the cystic spaces positive for calretinin, D2-40 and DK5/6

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confirming the diagnosis of multicystic peritoneal mesothelioma.

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3.0 Discussion and Review of the Literature

3.1 Clinical features

BMPM is a rare intraperitoneal tumor originating from peritoneal mesothelial cells and characterized by large multicystic and multi-septated masses with thin septations adherent to the peritoneal wall [1]. BMPM, also referred to as multilocular peritoneal inclusion cysts, was first described in 1979 [3]. Unlike the malignant form of peritoneal mesotheliomas, BMPM has no documented association with asbestos exposure [11]. Futhermore, BMPM is a benign entity which is considered to have no malignant potential; however, it does possess a high recurrence rate after surgical removal [4, 7].

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BMPM is most frequently encountered in young women of reproductive age and most

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commonly arises from the pelvic surfaces of the peritoneum. Cases in men and children are far

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less common but have also been reported [1, 4]. Patients often present with signs and symptoms of mass effect which includes abdominal distention, abdominal and pelvic

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discomfort/pain, constipation, dyspareunia, urinary obstruction, or a fixed palpable abdominal mass in rare cases [1, 2, 11]. The pathogenesis of BMPM remains unclear but the formation of

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inclusion cysts as a secondary reactive process has been hypothesized [3,10]. There is

pelvic inflammatory disease [1, 4, 6].

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suggestion of an association between BMPM and endometriosis, prior abdominal surgeries, and

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The primary mode of treatment is complete surgical removal though adjunctive use of hormone

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therapy for burden reduction and symptomatic control has been reported [7, 8, 12]. The pathologic sample of BMPM demonstrates thin-walled, irregularly spaced cysts containing

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serous fluid lined by flattened or cuboidal mesothelial cells [1, 12]. The recovered cells are positive for calretinen and cytokeratins which are both markers of mesothelial origin and confirm

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the diagnosis [2,10].

3.2 Review of literature and imaging features

BMPM in the literature is usually described as having an appearance of cystic structures in close association with the peritoneal surface. In women, they often adhere closely to the uterus and the ovaries [1, 2, 6]. An ultrasound usually shows anechoic to mildly echogenic, multiseptated cystic structures in the pelvis with a varying number of lobulations and cysts. In rare occasions, intraabdominal fluid or hemorrhage is also reported [1, 2, 6, 10]. Rare wall calcifications have been noted [6]. On CT, BMPM usually appears as low-density, multi-

ACCEPTED MANUSCRIPT loculated, multi-cystic, thin walled lesions that may engulf the surrounding soft tissue; however, invasion has not been reported. On MRI, they appear as multi-loculated cystic masses that are

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hypointense on T1 weighted images and hyper to intermediate intensity on T2 weighted

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sequences. The walls may demonstrate mild enhancement with contrast. Fat within the cysts

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has not been demonstrated [1, 2, 6].

We performed a literature review for benign multicystic peritoneal mesothelioma which yielded a

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total of 191 published articles [using search terminology of “benign multicystic peritoneal

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mesothelioma” and “peritoneal inclusion cyst”]. Although many articles provided nonspecific appearances of BMPM due to their typical cystic appearance, they were largely based on a small subject group. We sequentially reviewed all available articles, out of which 56 had imaging

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performed [either ultrasound, CT, and/or MRI]. This comprehensive literature review is

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summarized in Table 1. Out of all modalities, CT was the most commonly utilized and was followed closely by ultrasound. MRI was almost always accompanied by either a CT or

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ultrasound. The most commonly used terminology was multicystic as it was used in up to 80.6% of descriptions. Additionally, a pelvic location [83.3%] was more common than an abdominal

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location [40.3%] within the cases reviewed. Of note, there was a larger incidence of right-sided BMPM [31.9%] than left-sided [13.9%]. Hemorrhage, which was described in our case, was only found once on ultrasound during the literature review.

3.3 Differential diagnosis

The major radiological differential diagnosis for BMPM are abdominal lymphangiomas which also present as multiloculated cystic masses. Lymphangiomas, however, primarily occur in the pediatric population and do not have a gender predilection [7, 8]. The lesions are usually in the abdomen with presentation reported in the mesenteric, retroperitoneal and omental locations

ACCEPTED MANUSCRIPT with rare pelvic involvement [6]. Additionally, fat may be demonstrated within the neoplasm giving them varying attenuation from fluid to fat demonstrating lymphatic origin which is one of

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the distinguishing feature from BMPM [6, 7, 8]. Lymphangiomas may also demonstrate a

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characteristic elongated shape and may cross from one retroperitoneal compartment to another

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[8].

The list of other differential diagnosis for intraabdominal cystic neoplasms is extensive and

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includes abdominal lymphangioma, endometriosis, pseudomyxoma peritonei, mucinous

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cystadenoma, cystic teratoma, cystic mesothelioma, mullerian cyst, epidermoid cysts, tailgut cyst, bronchogenic cyst, cystic changes in solid neoplasms, and perianal mucinous carcinoma [6, 7, 1, 8]. There is significant overlap between the imaging features of these lesions; however,

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clinical history along with certain features such as location, size, shape, wall thickness,

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calcifications, presence of fat, and involvement of adjacent structures can help narrow the

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differential as was the case for our patient’s MRI.

Due to the overlap in imaging features among many of these peritoneal neoplasms, exploratory

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laparoscopy is often necessary for therapy and definitive diagnosis. This imaging characterization can play an important role in influencing the therapeutic approach depending on the suspected type of neoplasm [6, 8]. For example, the extent of surgical removal between lymphangioma and BMPM is variable since BMPM has a tendency to recur up to 50% of the time whereas lymphangiomas do not [6, 7].

Although tissue sampling and pathological analysis remains the primary mode of diagnosis for BMPM, our literature review and discussion of the imaging features provides a thorough compilation of imaging features encountered in confirmed cases of BMPM which can serve as a

ACCEPTED MANUSCRIPT guideline for differentiating BMPM from other intraperitoneal cystic masses on imaging as well

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as direct patient expectations and treatment strategy.

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ACCEPTED MANUSCRIPT Figure 1. Transabdominal ultrasound demonstrating a lobulated multicystic collection in the

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right adnexa (A). The cysts demonstrate no internal flow (B). Figure 2. (A) Coronal CT abdomen and pelvis demonstrating thin walled, complex cystic masses along the cecum and ascending colon and along the left adnexa (thick arrows). (B) Axial CT of Pelvis demonstrates cystic masses along both paracolic gutters abutting the colon as well as the lobule in the anterior omentum (thin arrows). Figure 3. (A) T2W coronal view through the lower abdomen and pelvis demonstrates fluid intensity containing T2 hyperintense multicystic mass with thin septations (circle). (B) Axial T2W-FS slice through the pelvis demonstrates T2 hyperintense, bilateral fluid containing adnexal cystic masses with thin septations. Coronal (C) and axial (D) T2W images demonstrating sparing of the ovaries bilaterally (arrows) by the encasing T2 hyperintense, cystic mass. Figure 4. Axial T1W pre (A) and post (B) contrast images demonstrate multicystic T1 hypointense cystic masses bilaterally with enhancing septation.

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Figure 1

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Figure 2

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Figure 3

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Figure 4

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Ultrasound (n=29)

MRI (n=13)

Total (n=72)

Multiple

56.7%

48.3%

30.8%

48.6%

Single

3.3%

3.4%

23.1%

6.9%

Cystic

70.0%

86.2%

92.3%

80.6%

Internal Septations

16.7%

31.0%

7.7%

20.8%

Irregular

10.0%

6.9%

9.7%

Non-enhancing

3.3%

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15.4%

-

7.7%

2.8%

Wall enhancing

26.7%

-

15.4%

13.9%

Right-sided

26.7%

37.9%

30.8%

31.9%

Left sided

6.7%

20.7%

15.4%

13.9%

Abdomen

50.0%

41.4%

15.4%

40.3%

63.3%

96.6%

100%

83.3%

13.3%

20.7%

0.0%

13.9%

0%

3.4%

0%

1.4%

-

3.4%

-

1.4%

Hemorrhage

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No vascular flow

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Ascites

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Pelvis

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CT (n=30)

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Table 1

Table 1: Percent usage of terminology for describing BMPM across CT, Ultrasound, and MRI obtained after literature search of 56 published articles containing a total of 72 studies.

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Highlights - Benign multicystic peritoneal mesothelioma (BMPM) is a rare peritoneal multicystic lesion. - BMPM is considered to have no malignancy potential; however, has a high rate of recurrence. - BMPM appears as large multi-cystic and lobulated fluid filled structures in the pelvis on imaging. - BMPM most commonly affects women of child bearing age.