The Cystic Mesothelioma case rePort

Case report: A 45 years old and female, presented abdominal pain in the right upper quadrant lasting 6 months. During investigation was evidenced in computerized tomography a massive hypodense formation, net multiseptada. Its surgical hysterectomy for uterine perforation history. Forwards for surgery with macroscopic findings of retroperitoneal cystic lesion, complex and voluminous suggestive of Mesothelioma Cystic peritoneal.


Introduction
The Peritoneal Cystic Mesothelioma was first described by Mennemeyer and Smith as a rare intra-abdominal tumor with preference for peritoneum of pelvic organs [1].
The Peritoneal Cystic Mesothelioma has a high rate of local [2] recurrence, being often necessary the use of new surgical approaches for a new resection.Incidence rates are higher in women (70%) than in men (30%) [3], as well as the recurrence (40-50%) and (33%) respectively.
It has a nonspecific clinical presentation, usually developing with abdominal or pelvic pain, chronic or intermittent distension, abdominal mass or palpable mass within the pelvis, increased waist circumference and constipation [4,5].The differential diagnosis is broad, including cystic lymphangioma (CL), mucinous cystadenoma, cystic teratoma and retroperitoneal pseudomyxoma [6].
The diagnosis is usually occasional, achieved very late and most often occurs in the course of investigation or surgical treatment for acute abdomen [7].Thus, this report aims to describe a case of Peritoneal Cystic Mesothelioma, because of the rarity of this tumor, the similarity of its symptomatic presentation with other diseases, the lack of specific symptoms and characteristics of imaging tests, besides its difficult diagnosis, confirmed only in postoperative histological findings.

Case report
A 45 years old patient, female, reported abdominal pain in the right hypochondrium for about 6 months and during diagnostic investigation after a tomography of the upper abdomen a massive hypodense formation was found, multiseptate cyst containing liquid, measuring approximately 12.00 x 10.00 cm, occupying the hepatorenal recess (Morison´s pouch), which may correspond to mucinous pseudomyxoma (figure 1 and figure 2).She has past history of hysterectomy realized because of uterine perforation.Consequently she was referred to have a surgery performed for tumor resection and macroscopic findings of complex and bulky retroperitoneal cystic lesions, in contact with right and transverse colon, liver, right kidney and stomach.A bilateral oophorectomy was performed, appendectomy and resection of retroperitoneal injury.The histopathological analysis evidenced a multilocular cystic mesothelioma without angiolymphatic invasion and necrosis, and appendix showing lymphoid hyperplasia with nonspecific reaction (figure 3 and figure 4).The patient was monitored for a year in the oncology clinic and developed a clinical improvement, uneventfully and without recurrence of the cyst.

Discussion
The peritoneal cystic mesothelioma was described for the first time by Henke in 1889 as a " tumor, a multiple cystic lymphangioma" [4], therefore in 1979 Mennemeyer and Smith determined its mesothelium origin [1] and started to call it benign cystic mesothelioma in 1980 by Moore, et al [5].Safioleas´s review of literature performed in 2006 found 130 described cases [6].After searching in PubMed with this article as a starting point, it was found another 42 new reported cases, making a total of 173 cases when included the case reported here.(Table 1).Its clinical presentation is nonspecific, such as abdominal or pelvic pain, abdominal tenderness, chronic or intermittent distension, abdominal or pelvic mass, increased waist circumference and constipation, as in the case reported [7,8].Hicham Elbouhaddouti, et al. described a similar case in 2013, in which the patient presented abdominal pain, increased waist circumference and constipation [9].The differential diagnosis is broad, including cystic lymphangioma, mucinous cystadenoma, cystic teratoma and retroperitoneal pseudomyxoma.[1] The pathogenesis is still unclear, however, as most of the cases already reported occurred in women of reproductive age, it is believed that female hormones play a role in its pathogenesis [10,11].Some authors relate it with neoplastic lesion, while others connect it to a reactive process [12][13][14][15][16], relating it to inflammation, previous history of surgery, endometriosis or uterine leiomyoma, suggesting that it is probably a peritoneal reaction to stimuli caused by chronic irritation, with interspersed mesothelium cells, reactive proliferation and cystic formation [17].As described by Safioleas, a woman of 62 years old had history of a hysterectomy procedure performed five years before the appearance of the peritoneal cystic mesothelioma [6].In the reported case, the patient also had previous hysterectomy.Benign cystic mesothelioma (BCM) is a rare tumor that arises from the abdominal peritoneum with a predilection to the pelvic peritoneum.BCM occurs five times more often in women than in men [5].The mean age of patients is 37-38 years at the time of diagnosis [5].Most patients present acute or chronic abdomen/pelvic discomfort, and palpable masses in the abdomen.However, BCM has also been found incidentally during imaging or laparotomy.Patients with BCM had a previous history of abdominal operations (42%), pelvic inflammatory disease (14%), or endometriosis (7%) [8].
It is considered a benign tumor [1,18], with good prognosis [19], with only one death reported in the literature, in which the patient did a partial resection of the abdominal mass, and died 12 years later [20], after refusing to do another surgery due to recurrence [21].
The best treatment according to some authors is to have surgical procedure, with complete enucleation of the cyst to avoid possible recurrence and malignant transformation [22] that can be carried out by laparotomy or laparoscopy [23].
The majority of the patients show a clinical scenario of nonspecific abdominal pain or pelvic discomfort and palpable masses in the abdomen.It often can be found by chance during laparoscopy or laparotomy.Patients with benign cystic mesothelioma (BCM) usually present previous history of abdominal surgery (42%), pelvic inflammatory disease (14%), or endometriosis (7%).Clinical condition is compatible with the described in this case.

Conclusion
Benign cystic mesothelioma (BCM) is a rare tumor of the abdominal peritoneum with preference for the pelvic region.Benign cystic mesothelioma presents five times higher incidence in women of reproductive age.
The recurrence rate of multilocular cystic mesothelioma after complete resection is of about 50%, and the goal should not be the cure, but symptomatic relief associated with radiological review, always individualizing the treatment and analyzing other complications.The patient of the described case developed with no complaints to date and she continues under outpatient monitoring and received orientation to the possibility of relapse despite the benign nature of the condition.
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Figure 1 :
Figure 1: Tomography of the upper abdomen a massive hypodense formation was found, multiseptate cyst containing liquid, measuring approximately 12.00 x 10.00 cm.

Figure 2 :
Figure 2: Tomography of the upper abdomen a massive hypodense formation was found, multiseptate cyst containing liquid, measuring approximately 12.00 x 10.00 cm.