- Email: [email protected]
Remote Recurrence of Benign Multicystic Peritoneal Mesothelioma
CASE REPORT
Remote Recurrence of Benign Multicystic Peritoneal Mesothelioma Caroline E. Lee, MD; Anita Agrawal, MD Department of Obstetrics and Gynecology, University of Saskatchewan, Saskatoon, SK
Abstract Background: Benign peritoneal cystic mesothelioma (BPCM) is a rare disease entity that arises from mesothelioma cells. We describe a rare case of BPCM recurrence 36 years after its initial presentation. Case: A 62-year-old woman was referred to an outpatient gynaecologic oncology clinic with an incidental finding of multiple pelvic cysts. She had a preceding history of known BPCM treated with extensive debulking surgery. She presented after 36 years of clinical remission. A repeat laparotomy for a debulking surgical procedure confirmed a recurrence of BPCM. Conclusion: Our current case represents a woman with a remote recurrence of BPCM after initial optimal debulking surgery. Her clinical presentation of recurrence after 36 years illustrates the need for long-term follow-up and clinical suspicion in symptomatic patients with previously diagnosed BPCM.
Résumé Contexte : Le mésothéliome kystique bénin du péritoine (MKBP) est une affection rare qui se développe dans les cellules mésothéliales. Nous présentons un rare cas de récurrence survenue 36 ans après la présentation initiale de l’affection. Cas : Une femme de 62 ans a été dirigée vers une consultation externe en gynéco-oncologie en raison de la découverte fortuite de multiples kystes pelviens. Ses antécédents comprenaient un MKBP traité par une importante chirurgie de réduction tumorale. Ces nouveaux kystes ont été découverts 36 ans après la rémission clinique. Une deuxième chirurgie de réduction tumorale par laparotomie a permis de confirmer la récurrence du MKBP. Conclusion : Le cas abordé ici est celui d’une femme présentant une récurrence de MKBP longtemps après avoir subi une première chirurgie de réduction tumorale optimale. Cette récurrence, survenue après 36 ans, montre qu’un suivi à long terme et un soupçon clinique de MKBP sont nécessaires chez les patients symptomatiques ayant un antécédent connu de cette affection.
Key Words: Cystic mesothelioma, peritoneal mesothelioma, recurrence Corresponding Author: Dr. Anita Agrawal, University of Saskatchewan, Saskatoon, SK. [email protected] Competing interests: None declared. Received on February 22, 2017 Accepted on April 25, 2017
Crown Copyright ª 2017 Published by Elsevier Inc. on behalf of The Society of Obstetricians and Gynaecologists of Canada/La Société des obstétriciens et gynécologues du Canada. All rights reserved.
J Obstet Gynaecol Can 2017;-(-):1e4 https://doi.org/10.1016/j.jogc.2017.04.038
INTRODUCTION
B
enign peritoneal cystic mesothelioma (BPCM) is a rare peritoneal tumour arising from the epithelial and mesenchymal components of mesothelial cells. Currently, there are approximately 140 published cases of BPCM, occurring predominantly in women of reproductive age.1e3 BPCM has been described as the result of a reactive or inflammatory process because of its association with endometriosis, previous abdominal operations, and pelvic inflammatory disease.1 Currently, the recommended treatment is complete surgical excision with close follow-up, given a local recurrence rate of 41%e50% occurring 2e29 years after initial presentation.2,4e6 Here, we describe a case report of a patient with a recurrence of BPCM after 36 years of clinical remission. CASE DESCRIPTION
A 62-year-old woman was referred to an outpatient gynaecologic oncology clinic with an incidental finding of multiple pelvic cysts. After a laparotomy for a benign ovarian cyst at age 17, she underwent two additional laparotomies 9 months apart for BPCM. At age 26, she underwent her first laparotomy for multiple pelvic lesions that were confirmed to be BPCM on the pathology report. However, she experienced a sudden acute onset of lower pelvic pain 9 months later related to the development of a large pelvic peritoneal cyst. Repeat laparotomy, total abdominal hysterectomy, and bilateral salpingoophorectomy were completed with the goal of optimal debulking of BPCM. The cysts were found on the pelvic peritoneal surfaces, sigmoid colon, omentum, and -
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CASE REPORT
Figure 1. Multiple cystic pelvic lesions on CT (top) and MRI (bottom) scans.
diaphragmatic surface. Again, the same diagnosis was confirmed on the pathology report. Postoperatively, she had been prescribed hormone replacement therapy. She maintained a state of clinical remission for 36 years. Shortly after discontinuing her hormone replacement therapy, radiologic recurrence was suspected because of an incidental finding of multiple pelvic cysts during investigations for cholestatic symptoms. CT and MRI scans revealed multiple cystic pelvic lesions along the left common iliac vein, right internal iliac artery, right adnexa, pelvic midline, rectosigmoid junction, rectum, and urinary bladder (Figure 1). The largest cyst measured 4.4 3.3 cm. The lesions were stable in size over the period of a year. Although the suspicion of malignant transformation of BPCM was low, a debulking operation
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by laparotomy was offered to confirm our working diagnosis of mesothelioma recurrence. Significant adhesions were encountered during the laparotomy as a result of her previous surgical procedures. The findings described on her CT scan and MRI were confirmed, and all visible disease was excised (Figure 2). Her final pathology report confirmed the diagnosis of benign mesothelial cysts (Figure 3). Histologic examination revealed benign nuclei and a lack of papillary architecture. DISCUSSION
As described in the literature, BPCM manifested in our patient with extensive involvement of the peritoneal surfaces including the surfaces of the pelvic and abdominal viscera (bladder and bowel) and the retroperitoneum.
Remote Recurrence of Benign Multicystic Peritoneal Mesothelioma
Figure 2. Multiple cystic lesions with a thin connective layer containing clear serous fluid.
BPCM is often discovered incidentally but can occasionally manifest with non-specific abdominal pain, abdominal distension, early satiety, and constipation.1,2 Lesions are described as occurring as a confluent mass of multiple translucent cysts or in isolation, studding peritoneal surfaces in a discontinuous fashion.1 Cysts range from a few millimetres to more than 20 cm in size and are often filled with serous fluid that is clear or straw-coloured.1,4 Unlike pleural mesotheliomas, BPCM has a favourable prognosis but with a propensity for local recurrence.4e6 However, rare cases of malignant transformation have been reported.7 The etiology of BPCM has been widely debated, but the prevailing theory is that BPCM forms as a response to a reactive process.1 However, other investigators believe that BPCM arises from a neoplastic process because of the slow but progressive growth of untreated lesions with the potential for malignant transformation.7 The current predominant treatment is complete resection of BPCM lesions, but the ideal management of BPCM continues to be highly debated.2,4,5 Hormonal therapy in the form of antiestrogen agents or gonadotropin agonists has been used successfully to reduce BPCM lesions, thus providing support for the theory that BPCM may depend on female sex hormones.4 Other investigators have used a
combination of cytoreduction surgery and hyperthermic intraperitoneal chemotherapy with good results.2 Given the rarity of BPCM, the debate regarding ideal management is unlikely to be resolved until more extensive literature is published. CONCLUSION
Our patient represents a unique case of BPCM in which remote recurrence followed optimal surgical management of the condition. Her current presentation was her third recurrence of BPCM, occurring more than 30 years after the initial presentation. After her second debulking operation, she was prescribed hormone replacement therapy and remained in clinical remission. However, recurrence was discovered on imaging and coincided with the time period of hormonal therapy discontinuation, although the time of recurrence is not definite. Cysts may have been present long before she discontinued the hormones, especially given that the cysts remained stable in size and number for almost a year. The current presentation may lend some opposition to the theory of hormonal dependence of BPCM. Our case also highlights the importance of long-term follow-up to monitor for remote recurrence even after optimal debulking surgery for BPCM.
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CASE REPORT
Figure 3. Histologic images representing benign mesothelioma cysts as seen by the appearance of benign nuclei and lack of papillary architecture.
REFERENCES 1. Weiss SW, Tavassoli FA. Multicystic mesothelioma: an analysis of pathologic findings and biologic behavior in 37 cases. Am J Surg Pathol 1988;12:37e46. 2. Chua TC, Yan TD, Deraco M, et al. Multi-institutional experience of diffuse intra-abdominal multicystic peritoneal mesothelioma. Br J Surg 2011;98:60e4. 3. Psoinos CM, Kandil D, Piperdi B, et al. Multicystic peritoneal mesothelioma in an octogenarian: diagnosis, natural history, and treatment. Int J Surg Pathol 2012;20:92e6. 4. Sawh RN, Malpica A, Deavers MT, et al. Benign cystic mesothelioma of the peritoneum: a clinicopathologic study of 17 cases and immunohistochemical
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analysis of estrogen and progesterone receptor status. Hum Pathol 2003;34:369e74. 5. 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:e70e3. 6. Iversen OH, Hovig T, Brandtzaeg P. Peritoneal, benign, cystic mesothelioma with free-floating cysts, re-examined by new methods: a case report. APMIS 1988;96:123e7. 7. Gonzalez-Moreno S, Yan H, Alcorn KW, et al. Malignant transformation of “benign” cystic mesothelioma of the peritoneum. J Surg Oncol 2002;79:243e51.
Remote Recurrence of Benign Multicystic Peritoneal Mesothelioma Caroline E. Lee, MD; Anita Agrawal, MD Department of Obstetrics and Gynecology, University of Saskatchewan, Saskatoon, SK
Abstract Background: Benign peritoneal cystic mesothelioma (BPCM) is a rare disease entity that arises from mesothelioma cells. We describe a rare case of BPCM recurrence 36 years after its initial presentation. Case: A 62-year-old woman was referred to an outpatient gynaecologic oncology clinic with an incidental finding of multiple pelvic cysts. She had a preceding history of known BPCM treated with extensive debulking surgery. She presented after 36 years of clinical remission. A repeat laparotomy for a debulking surgical procedure confirmed a recurrence of BPCM. Conclusion: Our current case represents a woman with a remote recurrence of BPCM after initial optimal debulking surgery. Her clinical presentation of recurrence after 36 years illustrates the need for long-term follow-up and clinical suspicion in symptomatic patients with previously diagnosed BPCM.
Résumé Contexte : Le mésothéliome kystique bénin du péritoine (MKBP) est une affection rare qui se développe dans les cellules mésothéliales. Nous présentons un rare cas de récurrence survenue 36 ans après la présentation initiale de l’affection. Cas : Une femme de 62 ans a été dirigée vers une consultation externe en gynéco-oncologie en raison de la découverte fortuite de multiples kystes pelviens. Ses antécédents comprenaient un MKBP traité par une importante chirurgie de réduction tumorale. Ces nouveaux kystes ont été découverts 36 ans après la rémission clinique. Une deuxième chirurgie de réduction tumorale par laparotomie a permis de confirmer la récurrence du MKBP. Conclusion : Le cas abordé ici est celui d’une femme présentant une récurrence de MKBP longtemps après avoir subi une première chirurgie de réduction tumorale optimale. Cette récurrence, survenue après 36 ans, montre qu’un suivi à long terme et un soupçon clinique de MKBP sont nécessaires chez les patients symptomatiques ayant un antécédent connu de cette affection.
Key Words: Cystic mesothelioma, peritoneal mesothelioma, recurrence Corresponding Author: Dr. Anita Agrawal, University of Saskatchewan, Saskatoon, SK. [email protected] Competing interests: None declared. Received on February 22, 2017 Accepted on April 25, 2017
Crown Copyright ª 2017 Published by Elsevier Inc. on behalf of The Society of Obstetricians and Gynaecologists of Canada/La Société des obstétriciens et gynécologues du Canada. All rights reserved.
J Obstet Gynaecol Can 2017;-(-):1e4 https://doi.org/10.1016/j.jogc.2017.04.038
INTRODUCTION
B
enign peritoneal cystic mesothelioma (BPCM) is a rare peritoneal tumour arising from the epithelial and mesenchymal components of mesothelial cells. Currently, there are approximately 140 published cases of BPCM, occurring predominantly in women of reproductive age.1e3 BPCM has been described as the result of a reactive or inflammatory process because of its association with endometriosis, previous abdominal operations, and pelvic inflammatory disease.1 Currently, the recommended treatment is complete surgical excision with close follow-up, given a local recurrence rate of 41%e50% occurring 2e29 years after initial presentation.2,4e6 Here, we describe a case report of a patient with a recurrence of BPCM after 36 years of clinical remission. CASE DESCRIPTION
A 62-year-old woman was referred to an outpatient gynaecologic oncology clinic with an incidental finding of multiple pelvic cysts. After a laparotomy for a benign ovarian cyst at age 17, she underwent two additional laparotomies 9 months apart for BPCM. At age 26, she underwent her first laparotomy for multiple pelvic lesions that were confirmed to be BPCM on the pathology report. However, she experienced a sudden acute onset of lower pelvic pain 9 months later related to the development of a large pelvic peritoneal cyst. Repeat laparotomy, total abdominal hysterectomy, and bilateral salpingoophorectomy were completed with the goal of optimal debulking of BPCM. The cysts were found on the pelvic peritoneal surfaces, sigmoid colon, omentum, and -
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CASE REPORT
Figure 1. Multiple cystic pelvic lesions on CT (top) and MRI (bottom) scans.
diaphragmatic surface. Again, the same diagnosis was confirmed on the pathology report. Postoperatively, she had been prescribed hormone replacement therapy. She maintained a state of clinical remission for 36 years. Shortly after discontinuing her hormone replacement therapy, radiologic recurrence was suspected because of an incidental finding of multiple pelvic cysts during investigations for cholestatic symptoms. CT and MRI scans revealed multiple cystic pelvic lesions along the left common iliac vein, right internal iliac artery, right adnexa, pelvic midline, rectosigmoid junction, rectum, and urinary bladder (Figure 1). The largest cyst measured 4.4 3.3 cm. The lesions were stable in size over the period of a year. Although the suspicion of malignant transformation of BPCM was low, a debulking operation
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by laparotomy was offered to confirm our working diagnosis of mesothelioma recurrence. Significant adhesions were encountered during the laparotomy as a result of her previous surgical procedures. The findings described on her CT scan and MRI were confirmed, and all visible disease was excised (Figure 2). Her final pathology report confirmed the diagnosis of benign mesothelial cysts (Figure 3). Histologic examination revealed benign nuclei and a lack of papillary architecture. DISCUSSION
As described in the literature, BPCM manifested in our patient with extensive involvement of the peritoneal surfaces including the surfaces of the pelvic and abdominal viscera (bladder and bowel) and the retroperitoneum.
Remote Recurrence of Benign Multicystic Peritoneal Mesothelioma
Figure 2. Multiple cystic lesions with a thin connective layer containing clear serous fluid.
BPCM is often discovered incidentally but can occasionally manifest with non-specific abdominal pain, abdominal distension, early satiety, and constipation.1,2 Lesions are described as occurring as a confluent mass of multiple translucent cysts or in isolation, studding peritoneal surfaces in a discontinuous fashion.1 Cysts range from a few millimetres to more than 20 cm in size and are often filled with serous fluid that is clear or straw-coloured.1,4 Unlike pleural mesotheliomas, BPCM has a favourable prognosis but with a propensity for local recurrence.4e6 However, rare cases of malignant transformation have been reported.7 The etiology of BPCM has been widely debated, but the prevailing theory is that BPCM forms as a response to a reactive process.1 However, other investigators believe that BPCM arises from a neoplastic process because of the slow but progressive growth of untreated lesions with the potential for malignant transformation.7 The current predominant treatment is complete resection of BPCM lesions, but the ideal management of BPCM continues to be highly debated.2,4,5 Hormonal therapy in the form of antiestrogen agents or gonadotropin agonists has been used successfully to reduce BPCM lesions, thus providing support for the theory that BPCM may depend on female sex hormones.4 Other investigators have used a
combination of cytoreduction surgery and hyperthermic intraperitoneal chemotherapy with good results.2 Given the rarity of BPCM, the debate regarding ideal management is unlikely to be resolved until more extensive literature is published. CONCLUSION
Our patient represents a unique case of BPCM in which remote recurrence followed optimal surgical management of the condition. Her current presentation was her third recurrence of BPCM, occurring more than 30 years after the initial presentation. After her second debulking operation, she was prescribed hormone replacement therapy and remained in clinical remission. However, recurrence was discovered on imaging and coincided with the time period of hormonal therapy discontinuation, although the time of recurrence is not definite. Cysts may have been present long before she discontinued the hormones, especially given that the cysts remained stable in size and number for almost a year. The current presentation may lend some opposition to the theory of hormonal dependence of BPCM. Our case also highlights the importance of long-term follow-up to monitor for remote recurrence even after optimal debulking surgery for BPCM.
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CASE REPORT
Figure 3. Histologic images representing benign mesothelioma cysts as seen by the appearance of benign nuclei and lack of papillary architecture.
REFERENCES 1. Weiss SW, Tavassoli FA. Multicystic mesothelioma: an analysis of pathologic findings and biologic behavior in 37 cases. Am J Surg Pathol 1988;12:37e46. 2. Chua TC, Yan TD, Deraco M, et al. Multi-institutional experience of diffuse intra-abdominal multicystic peritoneal mesothelioma. Br J Surg 2011;98:60e4. 3. Psoinos CM, Kandil D, Piperdi B, et al. Multicystic peritoneal mesothelioma in an octogenarian: diagnosis, natural history, and treatment. Int J Surg Pathol 2012;20:92e6. 4. Sawh RN, Malpica A, Deavers MT, et al. Benign cystic mesothelioma of the peritoneum: a clinicopathologic study of 17 cases and immunohistochemical
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analysis of estrogen and progesterone receptor status. Hum Pathol 2003;34:369e74. 5. 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:e70e3. 6. Iversen OH, Hovig T, Brandtzaeg P. Peritoneal, benign, cystic mesothelioma with free-floating cysts, re-examined by new methods: a case report. APMIS 1988;96:123e7. 7. Gonzalez-Moreno S, Yan H, Alcorn KW, et al. Malignant transformation of “benign” cystic mesothelioma of the peritoneum. J Surg Oncol 2002;79:243e51.