|Year : 2017 | Volume
| Issue : 6 | Page : 1047-1049
Multiple pelvic cysts in a patient with familial Mediterranean fever: Benign cystic mesothelioma
Melek Karakurt Eryilmaz1, Hasan Mutlu1, Gokhan Tazegul2, Ramazan Eryılmaz3, Fatma Yalçın Müsri1, Derya Kıvrak Salim1, Betül Ünal4, Hasan Şenol Coşkun1
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 Publication||13-Dec-2017|
Dr. Gokhan Tazegul
Department of Internal Medicine, Akdeniz University School of Medicine, Antalya
Source of Support: None, Conflict of Interest: None
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 Apr 1];13:1047-9. Available from: http://www.cancerjournal.net/text.asp?2017/13/6/1047/208750
| > Introduction|| |
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.,,
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.
We report a case of BCM in a 43-year-old male patient with FMF.
| > Case Report|| |
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 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|| |
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.,,, 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. Kurisu et al. 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.,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.,
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. FMF has been previously associated with increased frequency of both benign and malignant lesions such as hematological neoplasms, pericardial cysts, and malignant mesothelioma.,,, 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.
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]