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Benign Multicystic Peritoneal Mesothelioma (BMPM) as a rare cause of abdominal pain in a young male: case report and review of the literature

Benign Multicystic Peritoneal Mesothelioma (BMPM) as a rare cause of abdominal pain in a young... Benign Multicystic Peritoneal Mesothelioma (BMPM) is a rare benign cystic neoplasm that arise from the peritoneum, typic- ally found in young females (83%), with a high incidence of local recurrence after resection (almost 50% of cases). Fewer than 200 cases have been reported worldwide in 2017. Due to its rarity and the lack of a classical clinical presentation, the diagnosis pre-operatively is challenging; moreover a great part of the literature is composed of case reports and small group studies. Nowadays, guidelines are still not available, and treatment is en bloc resection of the lesion. Some authors suggest the use of Hyperthermic Intraperitoneal Chemotherapy (HIPEC) associated with the surgical approach, even if comparative data on recurrence and complications are lacking. We report a case of BMPM diagnosed in a 40-year-old male who under- went an exploratory laparoscopy and complete excision of the neoformation. Pathology confirmed the presence of a BMPM. Symptoms are non-specific, such as abdominal or pelvic pain INTRODUCTION and mass, but this entity may be found incidentally at laparot- Benign Multicystic Peritoneal Mesothelioma (BMPM) is an omy or within a hernia sac, grossly composed of multiple cysts exceedingly rare benign cystic intra-abdominal neoplasy that measuring up to 15 cm or more in diameter, microscopically arise from the peritoneum [1], typically found in females of lined by mesothelial cells. Due to its rarity, preoperative diagno- child-bearing age [2], with a high incidence of local recurrence sis is still a challenging clinical problem [6]. No evidence-based [3] after surgical resection, even after a long silent follow-up [4]. guidelines for treatment are actually available, although a surgi- First described in 1979 by Mennemeyer and Smith, fewer than cal resection is recommended: some authors suggest a combin- 200 cases have been reported worldwide in 2017 [5]. Although ation of cytoreductive surgery and Hyperthermic Intraperitoneal classically described as a benign lesion, with low risk of show Chemotherapy (HIPEC) [7], and new strategies based upon immu- malignant transformation and only after repeated post- nohistochemistry knowledge of the neoplasm may lead to others operative recurrences, a case report described malignancy since pharmaceutical strategies [8]. Benign Multicystic Peritoneal its first manifestation. Pathogenesis of this neoplasm is still Mesothelioma (BMPM) is a rare benign cystic neoplasm that uncertain; association with previous abdominal surgery, endo- arise from the peritoneum, typically found in young females metriosis or pelvic inflammatory disease are described [6]. (83%), with a high incidence of local recurrence after resection Received: January 29, 2019. Accepted: February 12, 2019 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2019. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com 1 Downloaded from https://academic.oup.com/jscr/article-abstract/2019/3/rjz057/5378632 by Ed 'DeepDyve' Gillespie user on 16 October 2019 2 S. Gussago et al. (almost 50% of cases). Fewer than 200 cases have been reported margin on omental fat. A small lesion of the colonic serosa was worldwide in 2017. Due to its rarity and the lack of a classical treated with a prophylactic continuous suture with Vycril 2-0. clinical presentation the diagnosis pre-operatively is challen- The postoperative course was uneventful ant the patient was ging, moreover a great part of the literature is composed of case discharged after 2 days without complications. reports and small group studies. Nowadays, guidelines are still On gross examination a soft, yellowish tissue specimen was not available, and treatment is en bloc resection of the lesion. seen, measuring about 13 × 9 × 4 cm, composed of multiple Some authors suggest the use of HIPEC associated with the sur- cysts, up to 1 cm in diameter, containing a clear fluid. It was gical approach, even if comparative data on recurrence and fixed in 10% formalin, embedded in paraffin, and the sections complications are lacking. We report a case of BMPM diagnosed were evaluated by hematoxylin and eosin stain. Histologically, it in a 40-year-old male who underwent an exploratory laparos- copy and complete excision of the neoformation. Pathology confirmed the presence of a BMPM. CASE REPORT A 40-year-old Caucasian male, a past medical history of smok- ing, complaining from 5 years of occasional abdominal discom- fort started having an acute, stinging pain in the right upper abdomen. A blood sample was taken that showed a smoldering increase of the transaminases and C-reactive protein of 22 mg/ L. He was investigated in the outpatient setting with an abdom- inal ultrasonography that revealed a polycystic lesion of 9 cm of diameter in the right flank. He was than referred to our Hospital, where the physical examination was completely silent. A contrast enhanced CT scan of the abdomen revealed a polylobate hypodense formation, with multiple thin internal septa which are enriched after contrast (dimension 83 × 43 × 61 mm) (Figs 1 and 2). Radiologist posed differential diagnosis among peritoneal lymphangiomatosis, enteric duplication cyst and—even if less likely in a male without history of past abdom- Figure 2: Contrast enhanced axial image that show the presence of a polylobate inal surgery—benign multicystic peritoneal mesothelioma. hypodense formation, with multiple thin internal septa. After multidisciplinary discussion we proceeded to an explora- tory laparoscopy that confirmed the presence of multiple cystic lesions of the peritoneum, some interesting the omentum, with a weak adherence with the ascending colon (Figs 3 and 4). We performed a complete excision of the neoformation, with free Figure 3: Intraoperative image showing the multicystic mass. Figure 1: Computed tomography image results: (1) contrast enhanced sagittal reconstruction showing the right flank neoplasm. Figure 4: Intraoperative image showing the multicystic mass. Downloaded from https://academic.oup.com/jscr/article-abstract/2019/3/rjz057/5378632 by Ed 'DeepDyve' Gillespie user on 16 October 2019 BMPM as a rare cause of abdominal pain in a young male 3 consisted of cysts lined by a single layer of flattened to cuboidal intermediate intensity on T2-weighted sequences, with mild mesothelial cells without atypia. Immunohistochemistry using contrast enhancement of the wall [9]. an automated stainer (Ventana, Tucson, AZ) was performed On radiological imaging abdominal lymphangioma is the and all tumor cells showed immunoreactivity for WT1 and most important differential diagnosis, but this neoplasm is more Calretinin. Based on these findings, the final histological diag- typical of pediatric population. The differential diagnosis must nosis was multicystic benign mesothelioma. include cystic adenomatoid tumor and malignant mesothelioma After discussion with Referral Center was suggested an (Figs 5–8). annual follow-up with MRI, and in case of relapse an approach Microscopically, the cysts of BMPM are lined by cuboidal or with cytoreductive surgery and subsequent HIPEC. flattened mesothelial cells. In the latter case, BMPM could simulate the appearance of a cystic lymphangioma, but the for- mer exhibits mesothelial markers, such as calretinin and WT1. DISCUSSION Ross et al. [10] believe that BMPM is probably the result of a peritoneal reactive proliferation, forming multiple peritoneal Even if other case reports described this condition, is particu- inclusion cysts, and not a true neoplasm. The natural history of larly uncommon to find BMPM in young male patients without this disorder, with its great tendency to local recurrence and its a past medical history of intra-abdominal surgery. tumor-like appearance, suggests its neoplastic pathogenesis, Clinical presentation usually includes chronic abdominal but both of the hypothesis can be argued. discomfort and mass-related symptoms like abdominal disten- Surgical resection en bloc of the BMPM is both diagnostic and sion, tenderness or dyspareunia. Physical examination may therapeutic and usually performed with a laparoscopic notice an abdominal mass. At the time of diagnosis, the mean approach, but it is affected by an high rate of recurrence. The diameter of BMPM is 13 cm [3]. Ultrasound of the abdomen usu- extreme rarity of this pathology is the prominent limitation to ally shows anechoic to mildly echogenic, multiseptated cystic an evidence-based approach. Nowadays, guidelines are still not structures in the pelvis composed of some lobulations and available, even if some authors suggest the combination of cysts [9]. cytoreductive surgery and HIPEC [7]. Chemotherapy and On CT scan, BMPM usually appears as low-density, multi- loculated, multicystic, thin-walled lesion. On MRI, BMPM appears hypointense on T1-weighted images and with hyper to Figure 5: Intraoperative image showing the multicystic mass. Figure 7: Immunohistochemically reaction for WT1. Figure 6: Histopathology results: cysts lined by flattened/cuboidal mesothelial cells. Figure 8: Immunohistochemically reaction for Calretinin. Downloaded from https://academic.oup.com/jscr/article-abstract/2019/3/rjz057/5378632 by Ed 'DeepDyve' Gillespie user on 16 October 2019 4 S. Gussago et al. radiotherapy did not show effects in this neoplasm [1]. An 3. Wang TB, Dai WG, Liu DW, Shi HP, Dong WG. Diagnosis and interesting case report of 2017 described the use of Rapamycin treatment of benign multicystic peritoneal mesothelioma. after primary surgery since the high level of phosphorylation of World J Gastroenterol 2013;19:6689–92. p70S6 kinase, a downstream mTOR target, found in BMPM [8]. 4. Lee CE, Agrawal A. Remote recurrence of benign multicystic No time lapse of follow up is known, with recurrence of this peritoneal mesothelioma. J Obstet Gynaecol Can 2017;39: pathology reported as far as 36 years after surgery. 1042–5. A better knowledge and consequent probably better thera- 5. Khurram MS, Shaikh H, Khan U, Edens J, Ibrar W, Hamza A, peutic approach for BMPM could be gained by a centralization et al. Benign multicystic peritoneal mesothelioma: a rare of cases in National Reference Centers, in order to increase the condition in an uncommon gender. Case Rep Pathol 2017; volume of patients and allow the beginning of targeted studies. 2017:9752908. Even if BMPMs are affected by an extremely low rate of mortal- 6. Dzieniecka M, Kałuży´nski A. Benign multicystic peritoneal ity (one case report) [2], the high risk of recurrence of this neo- mesothelioma (BMPM) – case report and review of the lit- plasm brings an intrinsic moderate–high morbidity, with a erature. Pol J Pathol 2011;62:122–4. considerable need for prolonged follow-up, particularly in 7. Baratti D, Kusamura S, Sironi A, Cabras A, Fumagalli L, young women whom infertility related radiation risk must be Laterza B, et al. Multicystic peritoneal mesothelioma: out- considered. comes and patho-biological features in a multi-institutional series treated by cytoreductive surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC (11). Eur J Surg Oncol CONFLICT OF INTEREST STATEMENT 2010;36:1047–53. None declared. 8. Stallone G, Infante B, Cormio L, Macarini L, Grandaliano G. Rapamycin treatment for benign multicystic peritoneal mesothelioma: a rare disease with a difficult management. REFERENCES Am J Case Rep 2017;18:632–6. 1. Safioleas MC, Constantinos K, Michael S, Konstantinos G, 9. Mehta V, Chowdhary V, Sharma R, Golia Pernicka JS. Constantinos S, Alkiviadis K. Benign multicystic peritoneal Imaging appearance of benign multicystic peritoneal meso- mesothelioma: a case report and review of the literature. thelioma: a case report and review of the literature. Clin World J Gastroenterol 2006;12:5739–42. Imaging 2017;42:133–7. 2. Weiss SW, Tavassoli FA. Multicystic mesothelioma. An ana- 10. Ross MJ, Welch WR, Scully RE. Multilocular peritoneal inclu- lysis of pathologic findings and biologic behavior in 37 sion cysts (so-called cystic mesotheliomas). Cancer 1989;64: cases. Am J Surg Pathol 1988;12:737–46. 1336–46. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Benign Multicystic Peritoneal Mesothelioma (BMPM) as a rare cause of abdominal pain in a young male: case report and review of the literature

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Oxford University Press
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Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2019.
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Abstract

Benign Multicystic Peritoneal Mesothelioma (BMPM) is a rare benign cystic neoplasm that arise from the peritoneum, typic- ally found in young females (83%), with a high incidence of local recurrence after resection (almost 50% of cases). Fewer than 200 cases have been reported worldwide in 2017. Due to its rarity and the lack of a classical clinical presentation, the diagnosis pre-operatively is challenging; moreover a great part of the literature is composed of case reports and small group studies. Nowadays, guidelines are still not available, and treatment is en bloc resection of the lesion. Some authors suggest the use of Hyperthermic Intraperitoneal Chemotherapy (HIPEC) associated with the surgical approach, even if comparative data on recurrence and complications are lacking. We report a case of BMPM diagnosed in a 40-year-old male who under- went an exploratory laparoscopy and complete excision of the neoformation. Pathology confirmed the presence of a BMPM. Symptoms are non-specific, such as abdominal or pelvic pain INTRODUCTION and mass, but this entity may be found incidentally at laparot- Benign Multicystic Peritoneal Mesothelioma (BMPM) is an omy or within a hernia sac, grossly composed of multiple cysts exceedingly rare benign cystic intra-abdominal neoplasy that measuring up to 15 cm or more in diameter, microscopically arise from the peritoneum [1], typically found in females of lined by mesothelial cells. Due to its rarity, preoperative diagno- child-bearing age [2], with a high incidence of local recurrence sis is still a challenging clinical problem [6]. No evidence-based [3] after surgical resection, even after a long silent follow-up [4]. guidelines for treatment are actually available, although a surgi- First described in 1979 by Mennemeyer and Smith, fewer than cal resection is recommended: some authors suggest a combin- 200 cases have been reported worldwide in 2017 [5]. Although ation of cytoreductive surgery and Hyperthermic Intraperitoneal classically described as a benign lesion, with low risk of show Chemotherapy (HIPEC) [7], and new strategies based upon immu- malignant transformation and only after repeated post- nohistochemistry knowledge of the neoplasm may lead to others operative recurrences, a case report described malignancy since pharmaceutical strategies [8]. Benign Multicystic Peritoneal its first manifestation. Pathogenesis of this neoplasm is still Mesothelioma (BMPM) is a rare benign cystic neoplasm that uncertain; association with previous abdominal surgery, endo- arise from the peritoneum, typically found in young females metriosis or pelvic inflammatory disease are described [6]. (83%), with a high incidence of local recurrence after resection Received: January 29, 2019. Accepted: February 12, 2019 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2019. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com 1 Downloaded from https://academic.oup.com/jscr/article-abstract/2019/3/rjz057/5378632 by Ed 'DeepDyve' Gillespie user on 16 October 2019 2 S. Gussago et al. (almost 50% of cases). Fewer than 200 cases have been reported margin on omental fat. A small lesion of the colonic serosa was worldwide in 2017. Due to its rarity and the lack of a classical treated with a prophylactic continuous suture with Vycril 2-0. clinical presentation the diagnosis pre-operatively is challen- The postoperative course was uneventful ant the patient was ging, moreover a great part of the literature is composed of case discharged after 2 days without complications. reports and small group studies. Nowadays, guidelines are still On gross examination a soft, yellowish tissue specimen was not available, and treatment is en bloc resection of the lesion. seen, measuring about 13 × 9 × 4 cm, composed of multiple Some authors suggest the use of HIPEC associated with the sur- cysts, up to 1 cm in diameter, containing a clear fluid. It was gical approach, even if comparative data on recurrence and fixed in 10% formalin, embedded in paraffin, and the sections complications are lacking. We report a case of BMPM diagnosed were evaluated by hematoxylin and eosin stain. Histologically, it in a 40-year-old male who underwent an exploratory laparos- copy and complete excision of the neoformation. Pathology confirmed the presence of a BMPM. CASE REPORT A 40-year-old Caucasian male, a past medical history of smok- ing, complaining from 5 years of occasional abdominal discom- fort started having an acute, stinging pain in the right upper abdomen. A blood sample was taken that showed a smoldering increase of the transaminases and C-reactive protein of 22 mg/ L. He was investigated in the outpatient setting with an abdom- inal ultrasonography that revealed a polycystic lesion of 9 cm of diameter in the right flank. He was than referred to our Hospital, where the physical examination was completely silent. A contrast enhanced CT scan of the abdomen revealed a polylobate hypodense formation, with multiple thin internal septa which are enriched after contrast (dimension 83 × 43 × 61 mm) (Figs 1 and 2). Radiologist posed differential diagnosis among peritoneal lymphangiomatosis, enteric duplication cyst and—even if less likely in a male without history of past abdom- Figure 2: Contrast enhanced axial image that show the presence of a polylobate inal surgery—benign multicystic peritoneal mesothelioma. hypodense formation, with multiple thin internal septa. After multidisciplinary discussion we proceeded to an explora- tory laparoscopy that confirmed the presence of multiple cystic lesions of the peritoneum, some interesting the omentum, with a weak adherence with the ascending colon (Figs 3 and 4). We performed a complete excision of the neoformation, with free Figure 3: Intraoperative image showing the multicystic mass. Figure 1: Computed tomography image results: (1) contrast enhanced sagittal reconstruction showing the right flank neoplasm. Figure 4: Intraoperative image showing the multicystic mass. Downloaded from https://academic.oup.com/jscr/article-abstract/2019/3/rjz057/5378632 by Ed 'DeepDyve' Gillespie user on 16 October 2019 BMPM as a rare cause of abdominal pain in a young male 3 consisted of cysts lined by a single layer of flattened to cuboidal intermediate intensity on T2-weighted sequences, with mild mesothelial cells without atypia. Immunohistochemistry using contrast enhancement of the wall [9]. an automated stainer (Ventana, Tucson, AZ) was performed On radiological imaging abdominal lymphangioma is the and all tumor cells showed immunoreactivity for WT1 and most important differential diagnosis, but this neoplasm is more Calretinin. Based on these findings, the final histological diag- typical of pediatric population. The differential diagnosis must nosis was multicystic benign mesothelioma. include cystic adenomatoid tumor and malignant mesothelioma After discussion with Referral Center was suggested an (Figs 5–8). annual follow-up with MRI, and in case of relapse an approach Microscopically, the cysts of BMPM are lined by cuboidal or with cytoreductive surgery and subsequent HIPEC. flattened mesothelial cells. In the latter case, BMPM could simulate the appearance of a cystic lymphangioma, but the for- mer exhibits mesothelial markers, such as calretinin and WT1. DISCUSSION Ross et al. [10] believe that BMPM is probably the result of a peritoneal reactive proliferation, forming multiple peritoneal Even if other case reports described this condition, is particu- inclusion cysts, and not a true neoplasm. The natural history of larly uncommon to find BMPM in young male patients without this disorder, with its great tendency to local recurrence and its a past medical history of intra-abdominal surgery. tumor-like appearance, suggests its neoplastic pathogenesis, Clinical presentation usually includes chronic abdominal but both of the hypothesis can be argued. discomfort and mass-related symptoms like abdominal disten- Surgical resection en bloc of the BMPM is both diagnostic and sion, tenderness or dyspareunia. Physical examination may therapeutic and usually performed with a laparoscopic notice an abdominal mass. At the time of diagnosis, the mean approach, but it is affected by an high rate of recurrence. The diameter of BMPM is 13 cm [3]. Ultrasound of the abdomen usu- extreme rarity of this pathology is the prominent limitation to ally shows anechoic to mildly echogenic, multiseptated cystic an evidence-based approach. Nowadays, guidelines are still not structures in the pelvis composed of some lobulations and available, even if some authors suggest the combination of cysts [9]. cytoreductive surgery and HIPEC [7]. Chemotherapy and On CT scan, BMPM usually appears as low-density, multi- loculated, multicystic, thin-walled lesion. On MRI, BMPM appears hypointense on T1-weighted images and with hyper to Figure 5: Intraoperative image showing the multicystic mass. Figure 7: Immunohistochemically reaction for WT1. Figure 6: Histopathology results: cysts lined by flattened/cuboidal mesothelial cells. Figure 8: Immunohistochemically reaction for Calretinin. Downloaded from https://academic.oup.com/jscr/article-abstract/2019/3/rjz057/5378632 by Ed 'DeepDyve' Gillespie user on 16 October 2019 4 S. Gussago et al. radiotherapy did not show effects in this neoplasm [1]. An 3. Wang TB, Dai WG, Liu DW, Shi HP, Dong WG. Diagnosis and interesting case report of 2017 described the use of Rapamycin treatment of benign multicystic peritoneal mesothelioma. after primary surgery since the high level of phosphorylation of World J Gastroenterol 2013;19:6689–92. p70S6 kinase, a downstream mTOR target, found in BMPM [8]. 4. Lee CE, Agrawal A. Remote recurrence of benign multicystic No time lapse of follow up is known, with recurrence of this peritoneal mesothelioma. J Obstet Gynaecol Can 2017;39: pathology reported as far as 36 years after surgery. 1042–5. A better knowledge and consequent probably better thera- 5. Khurram MS, Shaikh H, Khan U, Edens J, Ibrar W, Hamza A, peutic approach for BMPM could be gained by a centralization et al. Benign multicystic peritoneal mesothelioma: a rare of cases in National Reference Centers, in order to increase the condition in an uncommon gender. Case Rep Pathol 2017; volume of patients and allow the beginning of targeted studies. 2017:9752908. Even if BMPMs are affected by an extremely low rate of mortal- 6. Dzieniecka M, Kałuży´nski A. Benign multicystic peritoneal ity (one case report) [2], the high risk of recurrence of this neo- mesothelioma (BMPM) – case report and review of the lit- plasm brings an intrinsic moderate–high morbidity, with a erature. Pol J Pathol 2011;62:122–4. considerable need for prolonged follow-up, particularly in 7. Baratti D, Kusamura S, Sironi A, Cabras A, Fumagalli L, young women whom infertility related radiation risk must be Laterza B, et al. Multicystic peritoneal mesothelioma: out- considered. comes and patho-biological features in a multi-institutional series treated by cytoreductive surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC (11). Eur J Surg Oncol CONFLICT OF INTEREST STATEMENT 2010;36:1047–53. None declared. 8. Stallone G, Infante B, Cormio L, Macarini L, Grandaliano G. Rapamycin treatment for benign multicystic peritoneal mesothelioma: a rare disease with a difficult management. REFERENCES Am J Case Rep 2017;18:632–6. 1. Safioleas MC, Constantinos K, Michael S, Konstantinos G, 9. Mehta V, Chowdhary V, Sharma R, Golia Pernicka JS. Constantinos S, Alkiviadis K. Benign multicystic peritoneal Imaging appearance of benign multicystic peritoneal meso- mesothelioma: a case report and review of the literature. thelioma: a case report and review of the literature. Clin World J Gastroenterol 2006;12:5739–42. Imaging 2017;42:133–7. 2. Weiss SW, Tavassoli FA. Multicystic mesothelioma. An ana- 10. Ross MJ, Welch WR, Scully RE. Multilocular peritoneal inclu- lysis of pathologic findings and biologic behavior in 37 sion cysts (so-called cystic mesotheliomas). Cancer 1989;64: cases. Am J Surg Pathol 1988;12:737–46. 1336–46.

Journal

Journal of Surgical Case Reports – Oxford University Press

Published: Mar 1, 2019

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