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Abdominal hernia and the unexpected final diagnosis

Abdominal hernia and the unexpected final diagnosis Mucinous neoplasm of the appendix are rare entities, among these, mucinous cystadenomas contribute to 31–34%. Cystadenomas often produce extensive dilatation of the appendix with epithelial atypia. Spontaneous perforation often occurs in 20%, leading to mucin distribution throughout the peritoneal cavity. Half of the patients are completely asymp- tomatic and are detected as an incidental diagnosis, others symptoms include a palpable mass, hernias, weight loss, peri- tonism or even intestinal obstruction. We present a case of a 71-year-old female, she presented with a palpable mass in the upper abdomen. A ventral hernia was the most likely diagnosis and hernioplasty was planned. At surgery, a mucinous mass was discovered and mucous material was found free en the peritoneal cavity. Also, a perforated appendix and a mass in the cecum was found. Patient underwent full recovery. Pathology reported pseudomyxoma peritonei as the final diagnosis. INTRODUCTION could be missed. Pseudomyxoma peritonei is a rare disease Abdominal ventral hernias are usually a fairly easy diagnose that characterizes by the production and accumulation of with a relative straight-forward treatment. However, in rare mucous material in the abdomen. This mucous material can cases, some patients will have an undiagnosed pathology that accumulate through an abdominal defect and could simulate Figure 1: (A) Mucinous mass attached to the subcutaneous tissue. (B) Mucinous mass attached to the omentum that protrudes from the abdominal wall defect. (C) Right colon, with a perforated appendix and a mass in the cecum. Received: March 14, 2018. Accepted: April 26, 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/2018/5/rjy099/4995821 by Ed 'DeepDyve' Gillespie user on 21 June 2018 2 J.M. Cevallos et al. symptoms indistinguishable from a hernia. We present a case After exhaustive revision of the peritoneal cavity, a 3 × 3 × 1 of a 79-year-old female patient, she presented to surgical con- cm mass in the cecum was also discovered, that compromised sultation with a mass in the abdomen. Ventral hernia was the a thick appendix base and was covered with mucous material most likely diagnosis and surgery was planned. Mucous mater- (Fig. 1C). The rest if the appendix was digested and mucous ial in the abdomen and a mass in the cecum was discovered. material was found instead. Pseudomyxoma peritonei was the final diagnosis. The surgical decision was straight forward; the subcutane- ous mass removed along with a part of the greater omentum. Also since the cecum was compromised, and the appendix was CASE REPORT digested. A right hemicolectomy was performed and an ileoco- Patient is a 79-year-old female patient with past medical his- lic anastomosis was performed with autosutures. During the tory of hypertension, diabetes, hemorrhoidectomy and left total procedure, an adhesion between the transverse colon and the left knee replacement surgery. She presented to surgical con- inferior vena cava was tractioned, and a severe bleeding sultation after having experienced a year of a mass in the upper occurred. However, due to prompt intervention, the lesion was abdomen. After physical examination, ventral hernia was the identified and controlled (Fig. 2). A drain was left near the cava most likely diagnose, and surgery was planned. laceration, the aponeurotic defect was closed without a mesh, and During the procedure, a 15 × 10 × 5cm mucinous mass was the remainder of the surgery continued without complications discovered that was firmly attached to the subcutaneous tissue Pathology described a low grade disseminated mucinous (Fig. 1A) that protruded from the peritoneal cavity through 6 × 6 cystadenoma of the appendix, the serosa of the cecum was cm aponeurotic defect (Fig. 1B). The mass was also attached to congestive and infiltrated with mucous material (Fig. 3A), the the greater omentum and mucous material was found free in base of the appendix was perforated (Fig. 3B) and was covered the peritoneal cavity, most of it in the pelvic hole. Also, multi- with mucous-hemorrhagic material (Fig. 3C). Pseudomyxoma ples adhesions were discovered that covered the transverse peritonei was the final diagnosis. colon and the liver, ovaries appeared normal. From there, the patient had good clinical development. Bowel sound and flatus were present from the third postoperative day. Which is why sips of liquids were initiated, attaining good oral tolerance, and low plus serous production of the abdominal drain. Soft diet was initiated and the drain was removed. The patient fully recovered from surgery. On follow up controls patient is well and was referred to the oncologist. DISCUSSION Mucinous neoplasm of the appendix are rare entities, contribut- ing to only 0.2–0.7% of the appendiceal pathologies [1], and have a wide spectrum of clinical behavior, while invasive adenocar- cinoma with malignant features is usually deadly, there are other mucinous neoplastic lesions that do not demonstrate invasive features, but still have the potential to spread, recur and metastasize, a clinical entity known as PMP [2]. Mucinous cystadenoma starts in the appendix with trans- formation of the appendiceal goblet cells and subsequent form- ation of a mucinous tumor [6]. This mucin accumulates and eventually perforate the appendix in ~20% [5]. When there is a perforation, mucin and cells escape in the peritoneal cavity, these cells lack surface adhesion molecules, exfoliate easily and passively circulate in the peritoneal cavity. They can Figure 2: Inferior vena cava lesion repaired. deposit in the omentum, diaphragm and cul-de-sac [3]. This Figure 3: (A) Cecum wall, congestive and infiltrated with mucous material. (B) Appendix base, with atypia of goblet cells. (C) Mucous material covering the appendix base. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy099/4995821 by Ed 'DeepDyve' Gillespie user on 21 June 2018 Abdominal hernia and the unexpected final diagnosis 3 accumulating mucin increases the intra-abdominal pressure, and REFERENCES compress visceral organs [1, 3], it can herniate across an abdom- 1. Rymer B, Forsythe A, Husada G. Mucocoele and mucinous inal defect and simulate symptoms indistinguishable from a her- tumours of the appendix: a review of the literature. Int J Surg nia in ~14% [8]. It can also promote inflammation with fibrotic 2015;18:132e135. response with the development of bowel obstruction [1, 3]. 2. Rouzbahman M, Chetty R. Mucinous tumours of appendix Clinical manifestations may be non-specific, and varied. and ovary: an overview and evaluation of current practice. From undistinguishable appendicitis to asymptomatic patients J Clin Pathol 2014;67:193–7. [8], it is also an unexpected finding in about 2 of every 10.000 3. Ramaswamy V. Pathology of mucinous appendiceal tumors laparotomies [4]. In our case, patient presented with an appar- and pseudomyxoma peritonei. Indian J Surg Oncol 2016;7: ent abdominal hernia. 258–67. PMP appears in the seventh decade of life and is more com- 4. Touloumis Z, Galyfos G, Kavouras N, Menis M, Lavant L. mon in females, 94% of cases of PMP develop from a mucinous Aggressive pseudomyxoma peritonei: a case report with an tumor of the appendix [3], but it could appear from tumors unusual clinical presentation. Case Rep Oncol Med 2013;2013: from the stomach, gallbladder, colorectal and others [1, 3]. The best therapeutic approach should always be radical sur- 5. Anania G, Giaccari S, Solfrini G, Scagliarini L, Vedana L. gery, followed by cytoreductive surgery and hyperthermic Appendicular mucocele: two case reports and literature intraperitoneal chemotherapy [5, 7]. Since most PMPs, appears review. G Chir 2015;36:276–9. from the appendix it should always be removed [3]. As it hap- 6. Esquivel J, Sugarbaker H. Clinical presentation of the pseudo- pened in our case. myxoma peritonei syndrome. Br J Surg 2000;87:1414–8. If the PMP is discovered at the time of hernia surgery, the 7. Delhorme B, Severac F, Averous G, Glehen O, Passot G. safest but most radical approach should be performed, appen- Cytoreductive surgery and hyperthermic intraperitoneal dix must be removed, samples must be taken, closure of the chemotherapy for pseudomyxoma peritonei of appendicu- aponeurotic defect must be performed and the patient must be lar and extra-appendicular origin. Br J Surg 2018;105: referred to an oncologic center. 668–76. 8. Campbell P, Dawson S, Wali J, Kenny B, Whiteside MC. CONFLICT OF INTEREST STATEMENT Pseudomyxoma peritonei presenting as inguinal hernia. Ulster Med J 2009;78:189–90. None declared. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy099/4995821 by Ed 'DeepDyve' Gillespie user on 21 June 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Abdominal hernia and the unexpected final diagnosis

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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) 2018.
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2042-8812
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10.1093/jscr/rjy099
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Abstract

Mucinous neoplasm of the appendix are rare entities, among these, mucinous cystadenomas contribute to 31–34%. Cystadenomas often produce extensive dilatation of the appendix with epithelial atypia. Spontaneous perforation often occurs in 20%, leading to mucin distribution throughout the peritoneal cavity. Half of the patients are completely asymp- tomatic and are detected as an incidental diagnosis, others symptoms include a palpable mass, hernias, weight loss, peri- tonism or even intestinal obstruction. We present a case of a 71-year-old female, she presented with a palpable mass in the upper abdomen. A ventral hernia was the most likely diagnosis and hernioplasty was planned. At surgery, a mucinous mass was discovered and mucous material was found free en the peritoneal cavity. Also, a perforated appendix and a mass in the cecum was found. Patient underwent full recovery. Pathology reported pseudomyxoma peritonei as the final diagnosis. INTRODUCTION could be missed. Pseudomyxoma peritonei is a rare disease Abdominal ventral hernias are usually a fairly easy diagnose that characterizes by the production and accumulation of with a relative straight-forward treatment. However, in rare mucous material in the abdomen. This mucous material can cases, some patients will have an undiagnosed pathology that accumulate through an abdominal defect and could simulate Figure 1: (A) Mucinous mass attached to the subcutaneous tissue. (B) Mucinous mass attached to the omentum that protrudes from the abdominal wall defect. (C) Right colon, with a perforated appendix and a mass in the cecum. Received: March 14, 2018. Accepted: April 26, 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/2018/5/rjy099/4995821 by Ed 'DeepDyve' Gillespie user on 21 June 2018 2 J.M. Cevallos et al. symptoms indistinguishable from a hernia. We present a case After exhaustive revision of the peritoneal cavity, a 3 × 3 × 1 of a 79-year-old female patient, she presented to surgical con- cm mass in the cecum was also discovered, that compromised sultation with a mass in the abdomen. Ventral hernia was the a thick appendix base and was covered with mucous material most likely diagnosis and surgery was planned. Mucous mater- (Fig. 1C). The rest if the appendix was digested and mucous ial in the abdomen and a mass in the cecum was discovered. material was found instead. Pseudomyxoma peritonei was the final diagnosis. The surgical decision was straight forward; the subcutane- ous mass removed along with a part of the greater omentum. Also since the cecum was compromised, and the appendix was CASE REPORT digested. A right hemicolectomy was performed and an ileoco- Patient is a 79-year-old female patient with past medical his- lic anastomosis was performed with autosutures. During the tory of hypertension, diabetes, hemorrhoidectomy and left total procedure, an adhesion between the transverse colon and the left knee replacement surgery. She presented to surgical con- inferior vena cava was tractioned, and a severe bleeding sultation after having experienced a year of a mass in the upper occurred. However, due to prompt intervention, the lesion was abdomen. After physical examination, ventral hernia was the identified and controlled (Fig. 2). A drain was left near the cava most likely diagnose, and surgery was planned. laceration, the aponeurotic defect was closed without a mesh, and During the procedure, a 15 × 10 × 5cm mucinous mass was the remainder of the surgery continued without complications discovered that was firmly attached to the subcutaneous tissue Pathology described a low grade disseminated mucinous (Fig. 1A) that protruded from the peritoneal cavity through 6 × 6 cystadenoma of the appendix, the serosa of the cecum was cm aponeurotic defect (Fig. 1B). The mass was also attached to congestive and infiltrated with mucous material (Fig. 3A), the the greater omentum and mucous material was found free in base of the appendix was perforated (Fig. 3B) and was covered the peritoneal cavity, most of it in the pelvic hole. Also, multi- with mucous-hemorrhagic material (Fig. 3C). Pseudomyxoma ples adhesions were discovered that covered the transverse peritonei was the final diagnosis. colon and the liver, ovaries appeared normal. From there, the patient had good clinical development. Bowel sound and flatus were present from the third postoperative day. Which is why sips of liquids were initiated, attaining good oral tolerance, and low plus serous production of the abdominal drain. Soft diet was initiated and the drain was removed. The patient fully recovered from surgery. On follow up controls patient is well and was referred to the oncologist. DISCUSSION Mucinous neoplasm of the appendix are rare entities, contribut- ing to only 0.2–0.7% of the appendiceal pathologies [1], and have a wide spectrum of clinical behavior, while invasive adenocar- cinoma with malignant features is usually deadly, there are other mucinous neoplastic lesions that do not demonstrate invasive features, but still have the potential to spread, recur and metastasize, a clinical entity known as PMP [2]. Mucinous cystadenoma starts in the appendix with trans- formation of the appendiceal goblet cells and subsequent form- ation of a mucinous tumor [6]. This mucin accumulates and eventually perforate the appendix in ~20% [5]. When there is a perforation, mucin and cells escape in the peritoneal cavity, these cells lack surface adhesion molecules, exfoliate easily and passively circulate in the peritoneal cavity. They can Figure 2: Inferior vena cava lesion repaired. deposit in the omentum, diaphragm and cul-de-sac [3]. This Figure 3: (A) Cecum wall, congestive and infiltrated with mucous material. (B) Appendix base, with atypia of goblet cells. (C) Mucous material covering the appendix base. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy099/4995821 by Ed 'DeepDyve' Gillespie user on 21 June 2018 Abdominal hernia and the unexpected final diagnosis 3 accumulating mucin increases the intra-abdominal pressure, and REFERENCES compress visceral organs [1, 3], it can herniate across an abdom- 1. Rymer B, Forsythe A, Husada G. Mucocoele and mucinous inal defect and simulate symptoms indistinguishable from a her- tumours of the appendix: a review of the literature. Int J Surg nia in ~14% [8]. It can also promote inflammation with fibrotic 2015;18:132e135. response with the development of bowel obstruction [1, 3]. 2. Rouzbahman M, Chetty R. Mucinous tumours of appendix Clinical manifestations may be non-specific, and varied. and ovary: an overview and evaluation of current practice. From undistinguishable appendicitis to asymptomatic patients J Clin Pathol 2014;67:193–7. [8], it is also an unexpected finding in about 2 of every 10.000 3. Ramaswamy V. Pathology of mucinous appendiceal tumors laparotomies [4]. In our case, patient presented with an appar- and pseudomyxoma peritonei. Indian J Surg Oncol 2016;7: ent abdominal hernia. 258–67. PMP appears in the seventh decade of life and is more com- 4. Touloumis Z, Galyfos G, Kavouras N, Menis M, Lavant L. mon in females, 94% of cases of PMP develop from a mucinous Aggressive pseudomyxoma peritonei: a case report with an tumor of the appendix [3], but it could appear from tumors unusual clinical presentation. Case Rep Oncol Med 2013;2013: from the stomach, gallbladder, colorectal and others [1, 3]. The best therapeutic approach should always be radical sur- 5. Anania G, Giaccari S, Solfrini G, Scagliarini L, Vedana L. gery, followed by cytoreductive surgery and hyperthermic Appendicular mucocele: two case reports and literature intraperitoneal chemotherapy [5, 7]. Since most PMPs, appears review. G Chir 2015;36:276–9. from the appendix it should always be removed [3]. As it hap- 6. Esquivel J, Sugarbaker H. Clinical presentation of the pseudo- pened in our case. myxoma peritonei syndrome. Br J Surg 2000;87:1414–8. If the PMP is discovered at the time of hernia surgery, the 7. Delhorme B, Severac F, Averous G, Glehen O, Passot G. safest but most radical approach should be performed, appen- Cytoreductive surgery and hyperthermic intraperitoneal dix must be removed, samples must be taken, closure of the chemotherapy for pseudomyxoma peritonei of appendicu- aponeurotic defect must be performed and the patient must be lar and extra-appendicular origin. Br J Surg 2018;105: referred to an oncologic center. 668–76. 8. Campbell P, Dawson S, Wali J, Kenny B, Whiteside MC. CONFLICT OF INTEREST STATEMENT Pseudomyxoma peritonei presenting as inguinal hernia. Ulster Med J 2009;78:189–90. None declared. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy099/4995821 by Ed 'DeepDyve' Gillespie user on 21 June 2018

Journal

Journal of Surgical Case ReportsOxford University Press

Published: May 14, 2018

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