A rare case of giant extra-ovarian mucinous cystadenoma arising from sigmoid mesocolon

A rare case of giant extra-ovarian mucinous cystadenoma arising from sigmoid mesocolon An 80-year-old female presented with one month history of acutely worsening abdominal distention and pain, without fea- tures of bowel obstruction. A giant intra-abdominal simple cyst, separate from the ovaries, was seen on imaging. Initial haematological and biochemical investigations, including tumour markers, were normal. At laparotomy, the cystic tumour was discovered to arise from the sigmoid mesocolon and was resected en bloc. Histopathology revealed the tumour to be a benign extra-ovarian mucinous cystadenoma, which is a neoplasm of ovarian origin that can arise from extra-ovarian sites, including the mesentery. Extra-ovarian mucinous cystadenoma arising specifically from the mesentery are very rare intra- abdominal neoplasms with malignant potential despite its benign appearance on investigations. This case aims to raise awareness of this condition and to highlight its diagnostic approach and surgical management. INTRODUCTION Cases of mucinous cystadenoma arising specifically from the Mucinous cystic neoplasm (MCN) are surface epithelial tumours mesentery are rare with only 17 cases previously reported in the lit- of ovarian origin that can arise from extra-ovarian sites including erature [3]. There are no known risk factors aside from age (mean = pancreas, appendix, hepatobiliary tract and rarely the mesentery 32 ± 13) and gender (predominantly female) [3]. The pathogenesis and retroperitoneum [1–4]. Generally, the vast majority of ovarian of extra-ovarian MCNs remains incompletely understood, and is MCNs are benign or histologically borderline tumours. They typic- furtherconfoundedbytheir discoveryintwo men[3]. Some of the ally occur in mid adult life [3] and are rare before puberty or after more widely accepted theories that have been proposed include menopause [5]. The most common subtype of MCN is mucinous mucinous metaplasia in pre-existing mesothelial cysts, invaginated cystadenoma, which is typically large, multi-loculated and filled peritoneum along the course of ovarian descent, coelomic metapla- with gelatinous fluid rich in glycoproteins [5, 6]. The cyst wall of sia of epithelial cells and neoplastic differentiation of epithelial cells extra-ovarian MCNs is encapsulated by a fibrous capsule, lined by from a secondary extragenital Mullerian system [1, 7–10]. mucin-secreting epithelium and goblet cells, and associated with Unlike its ovarian counterpart, extra-ovarian mucinous cysta- an underlying sub-epithelial ovarian like stroma (OLS) [1, 6]. The denoma arising from the mesentery has malignant potential with OLS stains strongly for alpha-smooth muscle actin (alpha-SMA) 42% identified as such in 17 cases [3]. and vimentin and weakly for desmin. Both oestrogen and proges- We present a case report of a giant benign extra-ovarian terone receptors are expressed in the nuclei of OLS cells [4]. mucinous cystadenoma arising from the sigmoid mesocolon. Received: February 6, 2018. Accepted: February 14, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. 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 Downloaded from https://academic.oup.com/jscr/article-abstract/2018/3/rjy038/4924898 by Ed 'DeepDyve' Gillespie user on 16 March 2018 2 A. Tan et al. Figure 1: CT abdomen with IV and oral contrast; (A) coronal and (B) sagittal. CASE REPORT An 80-year-old female presented to our health service with one month history of acutely worsening abdominal distention and pain, on a background of six months of non-specificgastro- intestinal symptoms which was investigated with an abdominal ultrasound that did not detect any abnormality. Relevant medical history includes vaginal hysterectomy secondary to fibroids, type 2 diabetes mellitus, hypothyroidism, hypercholesterolaemia and hypertension. On history, the main symptoms were abdominal distention and pain with no features of bowel obstruction. The vital signs were within normal limits. On examination, the abdomen was grossly distended with focal tenderness in the upper abdomen. There was a palpable cystic mass which appears of pelvic ori- Figure 2: Midline laparotomy showing cyst. gin, however, the gynaecological examination was normal. Routine laboratory investigations were normal. Tumour markers, including CA 125, CA 15-3, CA 19-9 and CEA, were all within normal limits. A contrast-enhanced CT scan of the abdomen and pelvis was performed, which revealed a large intra-peritoneal cyst measuring 21 × 25 × 26 cm , containing simple fluid (Fig. 1). Therewereafew thin septations along the periphery with no appreciable nodular or soft tissue components within. The origin of the cyst was not able to be determined due to gross displacement of surrounding viscera. There was associated splaying of the bowel loops without resultant bowel obstruction. No pathologically enlarged abdominal, pelvic or inguinal lymph nodes were noted and there was no vascular com- promise. On pelvic ultrasound, the majority of the lesion appeared simple cystic in nature. There was no obvious internal colour flow. Both ovaries were identified and appeared separate from the cyst. Figure 3: Resected cyst. A laparotomy was performed to resect the cyst for diagnos- tic and therapeutic purposes (Figs 2 and 3). The cyst was noted to originate from the sigmoid mesocolon. No other viscera DISCUSSION required resection. The patient made an uneventful recovery. Macroscopically, there was an intact cystic structure contain- Cases of extra-ovarian mucinous cystadenoma arising from the ing clear yellowish serous fluid. The internal surface was smooth mesentery are very rare. Due to its rarity and typical benign with wall thickness of up to 10 mm. On microscopy, the cyst was appearance as a simple cyst, extra-ovarian mucinous cystade- lined by a single layer of columnar cells with apical mucin and noma is a diagnostic challenge to clinicians and may not be had surrounding ovarian stroma. There was no evidence of malig- pre-operatively recognized as a neoplasm with malignant nancy. The cystic tumour was confirmed to be a benign mucinous potential. Through our case presentation, we offer a diagnostic cystadenoma of the ovary arising from the sigmoid mesocolon. and surgical approach to its management. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/3/rjy038/4924898 by Ed 'DeepDyve' Gillespie user on 16 March 2018 A rare case of giant extra-ovarian mucinous cystadenoma 3 Imaging provides the pre-operative differential for extra-ovarian REFERENCES MCNs. On CT, the tumour is typically a well-circumscribed, uni- 1. Metaxas G, Tangalos A, Pappa P, Papageorgiou I. Mucinous loculated cystic lesion containing simple fluid which does not cystic neoplasms of the mesentery: a case report and enhance with contrast. Occasionally, internal septations or periph- review of the literature. World J Surg Oncol 2009;7:47. eral calcification of the capsule can be seen [3]. As MCNs can be 2. Mourra N, Werbrouck A, Arrive L. A large intra-abdominal large, CT is able to assess for anatomical relationship between the cystic mass arising from the mesocolon. Diagnosis: Mucinous tumour and surrounding structures [11]. On ultrasound, MCNs cystadenoma of the mesocolon with high-grade dysplasia. share similar features as a simple cyst: well-defined, uni-loculated Gastroenterology 2011;141:1160. cyst containing slightly echoic fluid. Pelvic ultrasound may also be 3. Cauchy F, Lefevre JH, Mourra N, Parc Y, Tiret E, Balladur P. used to determine whether the MCN arises from the ovary. Mucinous cystadenoma of the mesocolon, a rare entity fre- Haematological and biochemistry investigations are often quently presenting with features of malignity: two case reports unremarkable. Tumour markers such as CA 19-9 may be ele- and review of the literature. Clin Res Hepatol Gastroenterol 2012; vated in ovarian and pancreatic MCNs but not in those arising 36:e12–6. from the mesentery [3]. Therefore, there is no clear evidence on 4. Shiono S, Suda K, Nobukawa B, Arakawa A, Yamasaki S, the diagnostic utility of pre-operative tumour marker. Sasahara N, et al. Pancreatic, hepatic, splenic, and mesenteric Pre-operative aspiration of the cystic fluid for cytological ana- mucinous cystic neoplasms (MCN) are lumped together as lysis is not recommended for two reasons. In the 17 cases of extra ovarian MCN. Pathol Int 2006;56:71–7. extra-ovarian mucinous cystadenoma arising from the mesentery, 5. Kumar V, Abbas A, Aster J. Robbins & Cotran Pathologic Basis malignancy (high grade dysplasia and cystadenocarcinoma) was of Disease. 9th edn. PA: Elsevier, 2014. demonstrated in eight cases (42%) [3]. Spillage of potentially malig- 6. Cibas E, Ducatman B. Cytology: Diagnostic Principles and nant mucin into the peritoneum may cause seeding of malignant Clinical Correlates. 4th edn. PA: Saunders, 2014. cells and development of Pseudomyxoma peritonei. Additionally, 7. Kurtz R, Heimann T, Holt J, Beck R. Mesenteric and retro- cytological analysis of the aspirated fluid provides little diagnostic peritoneal cysts. Ann Surg 1986;1:109–12. value as it frequently fails to reveal the type of epithelial cells lin- 8. Linden PA, Ashley SW. Mucinous cystadenocarcinoma of ing the cyst [12]. Therefore, and for similar reasons, we do not rec- the mesentery. Surgery 2000;127:707–8. ommend intraoperative decompression of the cyst via aspiration. 9. Fujii S, Konishi I, Okamura H, Mori T. Mucinous cystadeno- Given the significant risk of malignancy, en bloc resection of mes- carcinoma of the retroperitoneum: a light and electron enteric MCNs is recommended for suitable surgical candidates. microscopic study. Gynecol Oncol 1986;24:103–12. 10. Tykkä H, Koivuniemi A. Carcinoma arising in a mesenteric ACKNOWLEDGEMENTS cyst. Am J Surg 1975;129:709–11. 11. De Perrot M, Bründler M, Tötsch M, Mentha G, Morel P. The authors are thankful to the patient who had provided writ- Mesenteric cysts. Dig Surg 2000;17:323–8. ten informed consent. 12. Bakker RF, Stoot JH, Blok P, Merkus JW. Primary retroperi- toneal mucinous cystadenoma with sarcoma-like mural CONFLICT OF INTEREST STATEMENT nodule: a case report and review of the literature. Virchows The authors declare that there are no conflicts of interest Arch 2007;451:853–7. regarding the publication of this article. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/3/rjy038/4924898 by Ed 'DeepDyve' Gillespie user on 16 March 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

A rare case of giant extra-ovarian mucinous cystadenoma arising from sigmoid mesocolon

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Abstract

An 80-year-old female presented with one month history of acutely worsening abdominal distention and pain, without fea- tures of bowel obstruction. A giant intra-abdominal simple cyst, separate from the ovaries, was seen on imaging. Initial haematological and biochemical investigations, including tumour markers, were normal. At laparotomy, the cystic tumour was discovered to arise from the sigmoid mesocolon and was resected en bloc. Histopathology revealed the tumour to be a benign extra-ovarian mucinous cystadenoma, which is a neoplasm of ovarian origin that can arise from extra-ovarian sites, including the mesentery. Extra-ovarian mucinous cystadenoma arising specifically from the mesentery are very rare intra- abdominal neoplasms with malignant potential despite its benign appearance on investigations. This case aims to raise awareness of this condition and to highlight its diagnostic approach and surgical management. INTRODUCTION Cases of mucinous cystadenoma arising specifically from the Mucinous cystic neoplasm (MCN) are surface epithelial tumours mesentery are rare with only 17 cases previously reported in the lit- of ovarian origin that can arise from extra-ovarian sites including erature [3]. There are no known risk factors aside from age (mean = pancreas, appendix, hepatobiliary tract and rarely the mesentery 32 ± 13) and gender (predominantly female) [3]. The pathogenesis and retroperitoneum [1–4]. Generally, the vast majority of ovarian of extra-ovarian MCNs remains incompletely understood, and is MCNs are benign or histologically borderline tumours. They typic- furtherconfoundedbytheir discoveryintwo men[3]. Some of the ally occur in mid adult life [3] and are rare before puberty or after more widely accepted theories that have been proposed include menopause [5]. The most common subtype of MCN is mucinous mucinous metaplasia in pre-existing mesothelial cysts, invaginated cystadenoma, which is typically large, multi-loculated and filled peritoneum along the course of ovarian descent, coelomic metapla- with gelatinous fluid rich in glycoproteins [5, 6]. The cyst wall of sia of epithelial cells and neoplastic differentiation of epithelial cells extra-ovarian MCNs is encapsulated by a fibrous capsule, lined by from a secondary extragenital Mullerian system [1, 7–10]. mucin-secreting epithelium and goblet cells, and associated with Unlike its ovarian counterpart, extra-ovarian mucinous cysta- an underlying sub-epithelial ovarian like stroma (OLS) [1, 6]. The denoma arising from the mesentery has malignant potential with OLS stains strongly for alpha-smooth muscle actin (alpha-SMA) 42% identified as such in 17 cases [3]. and vimentin and weakly for desmin. Both oestrogen and proges- We present a case report of a giant benign extra-ovarian terone receptors are expressed in the nuclei of OLS cells [4]. mucinous cystadenoma arising from the sigmoid mesocolon. Received: February 6, 2018. Accepted: February 14, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. 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 Downloaded from https://academic.oup.com/jscr/article-abstract/2018/3/rjy038/4924898 by Ed 'DeepDyve' Gillespie user on 16 March 2018 2 A. Tan et al. Figure 1: CT abdomen with IV and oral contrast; (A) coronal and (B) sagittal. CASE REPORT An 80-year-old female presented to our health service with one month history of acutely worsening abdominal distention and pain, on a background of six months of non-specificgastro- intestinal symptoms which was investigated with an abdominal ultrasound that did not detect any abnormality. Relevant medical history includes vaginal hysterectomy secondary to fibroids, type 2 diabetes mellitus, hypothyroidism, hypercholesterolaemia and hypertension. On history, the main symptoms were abdominal distention and pain with no features of bowel obstruction. The vital signs were within normal limits. On examination, the abdomen was grossly distended with focal tenderness in the upper abdomen. There was a palpable cystic mass which appears of pelvic ori- Figure 2: Midline laparotomy showing cyst. gin, however, the gynaecological examination was normal. Routine laboratory investigations were normal. Tumour markers, including CA 125, CA 15-3, CA 19-9 and CEA, were all within normal limits. A contrast-enhanced CT scan of the abdomen and pelvis was performed, which revealed a large intra-peritoneal cyst measuring 21 × 25 × 26 cm , containing simple fluid (Fig. 1). Therewereafew thin septations along the periphery with no appreciable nodular or soft tissue components within. The origin of the cyst was not able to be determined due to gross displacement of surrounding viscera. There was associated splaying of the bowel loops without resultant bowel obstruction. No pathologically enlarged abdominal, pelvic or inguinal lymph nodes were noted and there was no vascular com- promise. On pelvic ultrasound, the majority of the lesion appeared simple cystic in nature. There was no obvious internal colour flow. Both ovaries were identified and appeared separate from the cyst. Figure 3: Resected cyst. A laparotomy was performed to resect the cyst for diagnos- tic and therapeutic purposes (Figs 2 and 3). The cyst was noted to originate from the sigmoid mesocolon. No other viscera DISCUSSION required resection. The patient made an uneventful recovery. Macroscopically, there was an intact cystic structure contain- Cases of extra-ovarian mucinous cystadenoma arising from the ing clear yellowish serous fluid. The internal surface was smooth mesentery are very rare. Due to its rarity and typical benign with wall thickness of up to 10 mm. On microscopy, the cyst was appearance as a simple cyst, extra-ovarian mucinous cystade- lined by a single layer of columnar cells with apical mucin and noma is a diagnostic challenge to clinicians and may not be had surrounding ovarian stroma. There was no evidence of malig- pre-operatively recognized as a neoplasm with malignant nancy. The cystic tumour was confirmed to be a benign mucinous potential. Through our case presentation, we offer a diagnostic cystadenoma of the ovary arising from the sigmoid mesocolon. and surgical approach to its management. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/3/rjy038/4924898 by Ed 'DeepDyve' Gillespie user on 16 March 2018 A rare case of giant extra-ovarian mucinous cystadenoma 3 Imaging provides the pre-operative differential for extra-ovarian REFERENCES MCNs. On CT, the tumour is typically a well-circumscribed, uni- 1. Metaxas G, Tangalos A, Pappa P, Papageorgiou I. Mucinous loculated cystic lesion containing simple fluid which does not cystic neoplasms of the mesentery: a case report and enhance with contrast. Occasionally, internal septations or periph- review of the literature. World J Surg Oncol 2009;7:47. eral calcification of the capsule can be seen [3]. As MCNs can be 2. Mourra N, Werbrouck A, Arrive L. A large intra-abdominal large, CT is able to assess for anatomical relationship between the cystic mass arising from the mesocolon. Diagnosis: Mucinous tumour and surrounding structures [11]. On ultrasound, MCNs cystadenoma of the mesocolon with high-grade dysplasia. share similar features as a simple cyst: well-defined, uni-loculated Gastroenterology 2011;141:1160. cyst containing slightly echoic fluid. Pelvic ultrasound may also be 3. Cauchy F, Lefevre JH, Mourra N, Parc Y, Tiret E, Balladur P. used to determine whether the MCN arises from the ovary. Mucinous cystadenoma of the mesocolon, a rare entity fre- Haematological and biochemistry investigations are often quently presenting with features of malignity: two case reports unremarkable. Tumour markers such as CA 19-9 may be ele- and review of the literature. Clin Res Hepatol Gastroenterol 2012; vated in ovarian and pancreatic MCNs but not in those arising 36:e12–6. from the mesentery [3]. Therefore, there is no clear evidence on 4. Shiono S, Suda K, Nobukawa B, Arakawa A, Yamasaki S, the diagnostic utility of pre-operative tumour marker. Sasahara N, et al. Pancreatic, hepatic, splenic, and mesenteric Pre-operative aspiration of the cystic fluid for cytological ana- mucinous cystic neoplasms (MCN) are lumped together as lysis is not recommended for two reasons. In the 17 cases of extra ovarian MCN. Pathol Int 2006;56:71–7. extra-ovarian mucinous cystadenoma arising from the mesentery, 5. Kumar V, Abbas A, Aster J. Robbins & Cotran Pathologic Basis malignancy (high grade dysplasia and cystadenocarcinoma) was of Disease. 9th edn. PA: Elsevier, 2014. demonstrated in eight cases (42%) [3]. Spillage of potentially malig- 6. Cibas E, Ducatman B. Cytology: Diagnostic Principles and nant mucin into the peritoneum may cause seeding of malignant Clinical Correlates. 4th edn. PA: Saunders, 2014. cells and development of Pseudomyxoma peritonei. Additionally, 7. Kurtz R, Heimann T, Holt J, Beck R. Mesenteric and retro- cytological analysis of the aspirated fluid provides little diagnostic peritoneal cysts. Ann Surg 1986;1:109–12. value as it frequently fails to reveal the type of epithelial cells lin- 8. Linden PA, Ashley SW. Mucinous cystadenocarcinoma of ing the cyst [12]. Therefore, and for similar reasons, we do not rec- the mesentery. Surgery 2000;127:707–8. ommend intraoperative decompression of the cyst via aspiration. 9. Fujii S, Konishi I, Okamura H, Mori T. Mucinous cystadeno- Given the significant risk of malignancy, en bloc resection of mes- carcinoma of the retroperitoneum: a light and electron enteric MCNs is recommended for suitable surgical candidates. microscopic study. Gynecol Oncol 1986;24:103–12. 10. Tykkä H, Koivuniemi A. Carcinoma arising in a mesenteric ACKNOWLEDGEMENTS cyst. Am J Surg 1975;129:709–11. 11. De Perrot M, Bründler M, Tötsch M, Mentha G, Morel P. The authors are thankful to the patient who had provided writ- Mesenteric cysts. Dig Surg 2000;17:323–8. ten informed consent. 12. Bakker RF, Stoot JH, Blok P, Merkus JW. Primary retroperi- toneal mucinous cystadenoma with sarcoma-like mural CONFLICT OF INTEREST STATEMENT nodule: a case report and review of the literature. Virchows The authors declare that there are no conflicts of interest Arch 2007;451:853–7. regarding the publication of this article. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/3/rjy038/4924898 by Ed 'DeepDyve' Gillespie user on 16 March 2018

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Journal of Surgical Case ReportsOxford University Press

Published: Mar 1, 2018

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