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CORRESPONDENCE
Year : 2017  |  Volume : 13  |  Issue : 6  |  Page : 1047-1049

Multiple pelvic cysts in a patient with familial Mediterranean fever: Benign cystic mesothelioma


1 Department of Medical Oncology, Akdeniz University School of Medicine, Antalya, Turkey
2 Department of Internal Medicine, Akdeniz University School of Medicine, Antalya, Turkey
3 Department of General Surgery, Akdeniz University School of Medicine, Antalya, Turkey
4 Department of Pathology, Akdeniz University School of Medicine, Antalya, Turkey

Date of Web Publication13-Dec-2017

Correspondence Address:
Dr. Gokhan Tazegul
Department of Internal Medicine, Akdeniz University School of Medicine, Antalya
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcrt.JCRT_125_16

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 > Abstract 

Benign cystic mesothelioma (BCM) is a rare tumor arising from endothelial cells of the pelvic visceral or parietal peritoneum. It is a clinically and histopathologically benign disease. Etiology and pathogenesis of BCM remain unclear. Familial Mediterranean fever (FMF) is an inherited disorder characterized by episodes of fever, and abdominal, chest and/or joint inflammation. Association between malignant mesothelioma and FMF has been reported previously; however, co-existence of FMF and BCM is rare. Here, we report a case of BCM in a 43-year-old male patient with FMF.

Keywords: Abdominal mass, benign cystic mesothelioma, familial Mediterranean fever


How to cite this article:
Eryilmaz MK, Mutlu H, Tazegul G, Eryılmaz R, Müsri FY, Salim DK, Ünal B, Coşkun H&. Multiple pelvic cysts in a patient with familial Mediterranean fever: Benign cystic mesothelioma. J Can Res Ther 2017;13:1047-9

How to cite this URL:
Eryilmaz MK, Mutlu H, Tazegul G, Eryılmaz R, Müsri FY, Salim DK, Ünal B, Coşkun H&. Multiple pelvic cysts in a patient with familial Mediterranean fever: Benign cystic mesothelioma. J Can Res Ther [serial online] 2017 [cited 2020 Jul 10];13:1047-9. Available from: http://www.cancerjournal.net/text.asp?2017/13/6/1047/208750


 > Introduction Top


Benign cystic mesothelioma (BCM) is a rare intra-abdominal tumor of peritoneum and occurs more frequently in reproductive-aged women. However, inflammatory processes may play a role in etiopathogenesis; some predisposing factors involve pelvic inflammatory disease, previous pelvic surgery and endometriosis.[1],[2],[3]

Familial  Mediterranean fever More Details (FMF) is an inherited disorder with autosomal recessive inheritance characterized by episodes of fever, and abdominal, chest and/or joint inflammation. Ninety-five percent of patients with FMF have abdominal serositis, and it may be the first sign in half of the patients.[4]

We report a case of BCM in a 43-year-old male patient with FMF.


 > Case Report Top


A 43-year-old male patient was admitted to general surgery outpatient clinic with progressive abdominal distention and pain. He had distention and pain for 3 months and dysfunctional voiding for the past 15 days. He was diagnosed with FMF in 1995 with recurrent fever, abdominal distention, and pain episodes. He was using 2 mg/day colchicine for 20 years and was complete clinical remission. He had no other known diseases, and he was not using alcohol or smoking cigarettes. He had no history of asbestos exposure. Furthermore, he was not using any drugs apart from colchicine. On physical examination, he had a distended abdomen and bilateral pretibial edema. Laboratory tests including blood count, sedimentation rate, C-reactive protein, coagulation tests, liver enzymes, and urine analysis were within normal limits. Creatinine was found elevated to 1.4 mg/dL. Abdominal ultrasonography showed moderate ascites with a cystic mass organized around the bladder. Computerized tomography showed mild to moderate ascites and multiple cysts filling the right pelvis, extending to the upper border of the prostate, and displacing urinary bladder anteriorly [Figure 1] and [Figure 2]. The patient underwent surgery for sampling and excision. Multiple thin-walled cystic masses on omentum, meso and intestines filling right lower quadrant and extending to bladder and pelvis were seen intraoperatively [Figure 3]. Cysts contained visible serous fluid. Biggest excised cyst was 7 cm in diameter. All visible cysts were excised along with omentectomy and appendectomy. Histopathological diagnosis was consistent with BCM [Figure 4], [Figure 5], [Figure 6].
Figure 1: In this computerized tomography section, multiple cysts filling the right pelvis and mild ascites are seen

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Figure 2: Cysts extend to the upper border of prostate and significantly displace the urinary bladder anteriorly

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Figure 3: Multiple thin-walled cystic masses on omentum, meso and intestines filling right lower quadrant were seen intraoperatively

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Figure 4: Multiloculated cyst lined by cuboidal mesothelial cells. (H and E, ×100)

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Figure 5: There is no significant atypia. (H and E, ×200)

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Figure 6: (a-c) Mesothelial cells express (a) CK7 and (b) desmin diffusely and (c) WT-1 focally. (a and b) ×100, (c) ×200

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 > Discussion Top


BCM is a rare intraabdominal tumor arising from endothelial cells of the visceral or parietal peritoneum. The most common sites of the disease are serosal surfaces of pelvic peritoneum. BCM is mostly a silent tumor and incidentally diagnosed. However, it may present with large cystic abdominal masses with thin-walled septa which may cause obstructive symptoms, abdominal pain, ascites, constipation, urinary urgency, and/or uterine bleeding.[1],[2],[5],[6] Etiology of BCM is unclear, however since it is mostly seen in reproductive-aged females led to believe that sex hormones may play a role in pathogenesis. Some authors believe it to be an inflammatory process, some predisposing factors involve pelvic inflammatory disease, previous surgery and endometriosis which could result in mesothelial cell entrapment, reactive proliferation and cystic formations.[7] Kurisu et al.[8] reported small foci of endometriosis in and adjacent to cystic walls. Other authors believe BCM to be of a neoplastic origin of slow growth since untreated lesions grow progressively, but slow and lesions show a tendency to recur after resection.[9],[10]En bloc resection is considered the treatment of choice and is based on the removal of the cysts from the abdominal cavity. Long term follow-up of these patients is necessary to identify recurrences and the potential of malignant transformation. In one demonstrated case, BCM was transformed into malignant mesothelioma.[11],[12]

FMF is an inherited disorder with autosomal recessive inheritance. The gene for FMF (Mediterranean fever) was mapped to the short arm of chromosome 16. This gene that causes FMF encodes 781-amino acid pyrin protein. Eighty-five percent of the genetic mutations are encoded in exon 10 and exon 2. Exon 10 contains 4 principal mutations (M694V, V726A, M680I, and M694I) and exon 2 contains 1 common mutation (E148Q). FMF episodes are characterized by recurrent and short-lasting fever, peritonitis, synovitis, pleuritis, and rarely, inflammation and serositis that also include pericarditis. The episodes are self-limiting generally within 12–72 h. Ninety-five percent of patients with FMF have abdominal serositis, and it may be the first sign in half of the patients. Mild to moderate ascites may be seen in episodes. Recurrent peritoneal inflammation during episodes may cause peritoneal fibrosis and adhesions.[4] FMF has been previously associated with increased frequency of both benign and malignant lesions such as hematological neoplasms, pericardial cysts, and malignant mesothelioma.[13],[14],[15],[16] However, in the literature, only one case has been reported co-existence of FMF and BCM. In this case, even though the authors discussed that chronic peritoneal irritation may have contributed on the pathogenesis of BCM, they argue that remission after colchicine treatment makes this explanation unlikely.[17]

As a result, our case is the second case report of BCM in a patient with FMF. Co-existence with FMF and pathogenesis of BCM are not well documented, but possible cause is considered as a pelvic inflammatory process. FMF may play a role in the pathogenesis of BCM due to aberrant inflammation during episodes and subclinical inflammation in between episodes. In patients with FMF who have abdominal mass and ascites, BCM should be considered in differential diagnosis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
 > References Top

1.
Weiss SW, Tavassoli FA. Multicystic mesothelioma. An analysis of pathologic findings and biologic behavior in 37 cases. Am J Surg Pathol 1988;12:737-46.  Back to cited text no. 1
[PUBMED]    
2.
Safioleas MC, Constantinos K, Michael S, Konstantinos G, Constantinos S, Alkiviadis K. Benign multicystic peritoneal mesothelioma: A case report and review of the literature. World J Gastroenterol 2006;12:5739-42.  Back to cited text no. 2
[PUBMED]    
3.
Park BJ, Alexander HR, Libutti SK, Wu P, Royalty D, Kranda KC, et al. Treatment of primary peritoneal mesothelioma by continuous hyperthermic peritoneal perfusion (CHPP). Ann Surg Oncol 1999;6:582-90.  Back to cited text no. 3
[PUBMED]    
4.
Kucuk A, Gezer IA, Ucar R, Karahan AY. Familial mediterranean fever. Acta Medica (Hradec Kralove) 2014;57:97-104.  Back to cited text no. 4
[PUBMED]    
5.
Momeni M, Pereira E, Grigoryan G, Zakashansky K. Multicystic benign cystic mesothelioma presenting as a pelvic mass. Case Rep Obstet Gynecol 2014;2014:852583.  Back to cited text no. 5
[PUBMED]    
6.
O'Connor DB, Beddy D, Aremu MA. Benign cystic mesothelioma of the appendix presenting in a woman: A case report. J Med Case Rep 2010;4:394.  Back to cited text no. 6
    
7.
Wang TB, Dai WG, Liu DW, Shi HP, Dong WG. Diagnosis and treatment of benign multicystic peritoneal mesothelioma. World J Gastroenterol 2013;19:6689-92.  Back to cited text no. 7
[PUBMED]    
8.
Kurisu Y, Tsuji M, Shibayama Y, Yamada T, Ohmichi M. Multicystic mesothelioma caused by endometriosis: 2 case reports and review of the literature. Int J Gynecol Pathol 2011;30:163-6.  Back to cited text no. 8
[PUBMED]    
9.
Stroescu C, Negulescu R, Herlea V, David L, Ivanov B, Nitipir C, et al. Recurrent benign cystic peritoneal mesothelioma. Chirurgia (Bucur) 2008;103:715-8.  Back to cited text no. 9
[PUBMED]    
10.
Witek TD, Marchese JW, Farrell TJ. A recurrence of benign multicystic peritoneal mesothelioma treated through laparoscopic excision: A case report and review of the literature. Surg Laparosc Endosc Percutan Tech 2014;24:e70-3.  Back to cited text no. 10
[PUBMED]    
11.
González-Moreno S, Yan H, Alcorn KW, Sugarbaker PH. Malignant transformation of “benign” cystic mesothelioma of the peritoneum. J Surg Oncol 2002;79:243-51.  Back to cited text no. 11
    
12.
Bierhoff E, Pfeifer U. Malignant mesothelioma arising from a benign mediastinal mesothelial cyst. Gen Diagn Pathol 1996;142:59-62.  Back to cited text no. 12
[PUBMED]    
13.
Oktenli C, Celik S. High frequency of inherited variants in the MEFV gene in patients with hematologic neoplasms: A genetic susceptibility? Int J Hematol 2012;95:380-5.  Back to cited text no. 13
[PUBMED]    
14.
Çelik A, Çalapkorur B, Özdogru I. Case images: A pericardial cyst due to familial Mediterranean fever. Turk Kardiyol Dern Ars 2010;38:379.  Back to cited text no. 14
    
15.
Gentiloni N, Febbraro S, Barone C, Lemmo G, Neri G, Zannoni G, et al. Peritoneal mesothelioma in recurrent familial peritonitis. J Clin Gastroenterol 1997;24:276-9.  Back to cited text no. 15
    
16.
Belange G, Gompel H, Chaouat Y, Chaouat D. Malignant peritoneal mesothelioma occurring in periodic disease: Apropos of a case. Rev Med Interne 1998;19:427-30.  Back to cited text no. 16
    
17.
Curgunlu A, Karter Y, Tüfekci IB, Tunckale A, Karahasanoglu T. Benign cystic mesothelioma: A rare cause of ascites in a case with familial Mediterranean fever. Clin Exp Rheumatol 2003;21 4 Suppl 30:S41-3.  Back to cited text no. 17
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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