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Case 324

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Male patient, 59 years old, presenting weight loss with gradual increase of the abdominal volume and uncharacteristic abdominal pain in the past 3 years, without alteration of the intestinal habit. At the physical examination: patient in good conditions, with muscle loss in the upper and lower limbs and voluminous ascites (grade 3). Paracentesis revealed gelatinous and mucinous ascites at the cytologic examination. Increase of CEA and Ca19-9 was found in the laboratory tests. Abdominal and pelvis CT was requested.

Considering the clinical history and the provided images, what is the most likely diagnosis?

a) Peritoneal carcinomatosis

25%

b) Peritoneal tuberculosis

25%

c) Pseudomyxoma peritonei

25%

d) Spontaneous bacterial peritonitis

25%
   

Image analysis

Image 1 - Analysis: Abdominal computed tomography (CT), axial view, at the T10 level, after intravenous administration of iodinated contrast, arterial phase. Peritoneal implants. Presence of thick fluid compressing the liver surface (red arrow), also evidenced in the left hypochondrium (yellow arrow).

 

 

Image 2 - Analysis: Abdominal and pelvis CT, axial view, at the L5 level, after intravenous administration of iodinated contrast, arterial phase. Homogeneous material accumulation, of low density, contrast-enhanced, consistent with thick fluid (red circle), displacing the adjacent intestinal loops (yellow arrows).

 

 

Image 3 - Analysis: Abdominal and pelvis CT, coronal reconstruction, mesenteric region, after intravenous administration of iodinated contrast, arterial phase. Peritoneal implants. Homogeneous material accumulation, of low density, non-contrast-enhanced, consistent with thick fluid (red arrows), conditioning impressions in the inferior liver surface - scalloping (blue arrows).

 

 

Image 4 - Analysis: Abdominal and pelvis CT, paramedian sagittal reconstruction, after intravenous administration of iodinated contrast, arterial phase. Homogeneous material accumulation, of low density, in the peritoneal cavity, non-contrast-enhanced, consistent with thick fluid (red circle), compressing the liver surface (blue arrow) and displacing the adjacent intestinal loops (yellow arrows).

Highlights

- Pseudomyxoma peritonei is a rare malignant condition, which is defined as an intraperitoneal accumulation of mucus due to the dissemination of mucinous neoplasm, characterized by the redistribution phenomenon;

- The primary tumor is usually appendiceal, but it can also be derived from the colon and rectum, urachus, pancreas, gallbladder, pyelocaliceal system and ovaries;

- The diagnostic methods are CT and ascitic fluid analysis;

- Treatment can vary from periodic surgical debulking to cytoreductive surgery with intraperitoneal chemotherapy;

- Pseudomyxoma peritonei presents a low degree of malignancy, indolent behavior, absence of distant metastases and long survival.

References

- D'Ippolito G, P. Caldana R. Gastrointestinal - Série Colégio Brasileiro de Radiologia e Diagnóstico por Imagem. 2nd ed. Rio de Janeiro: Elsevier Editora Ltda, 2011.

- Sugarbaker P. Cytoreductive surgery and peri-operative intraperitoneal chemotherapy as a curative approach to pseudomyxoma peritonei syndrome. European Journal of Surgical Oncology (EJSO), 27(3): 239-243, 2001.

- Hinson F, Ambrose N. Pseudomyxoma peritonei. British Journal of Surgery, 85(10): 1332-1339, 1998.

- Carr N. Current concepts in pseudomyxoma peritonei. Annales de Pathologie, 34(1): 9-13, 2014.

- Carr N, Cecil T, Mohamed F, Sobin L, Sugarbaker P, González-Moreno S et al. A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia. The American Journal of Surgical Pathology, 40(1): 14-26, 2016.

- Swanson R, Meyerhardt J. UpToDate [Internet]. Cancer of the appendix and pseudomyxoma peritonei [updated in May 4th, 2018, access in August 4th, 2018]. Available in: https://www.uptodate.com/contents/cancer-of-the-appendix-and-pseudomyxoma-peritonei;

Author

Gabriella Yuka Shiomatsu, 4th year medical student at Universidade Federal de Minas Gerais.

E-mail: gabi.yuka[at]hotmail.com

Supervisors

Rodrigo Gomes da Silva, Colorectal Surgeon and Associated Professor at the Surgery Department of the Medical School of Universidade Federal de Minas Gerais.

E-mail: rodrigogsilva[at]uol.com.br

 

Júlio Guerra Domingues, Substitute Professor at the Anatomy and Image Department of the Medical School of Universidade de Minas Gerais.

E-mail: jgdjulio[at]gmail.com

Reviewers

Vinícius Avelar, Guilherme Carvalho, Prof. José Nelson Mendes Vieira e Profª. Viviane Santuari Parisotto Marino.

Translated by

Gabriella Yuka Shiomatsu, 4th year medical student at Universidade Federal de Minas Gerais.

E-mail: gabi.yuka[at]hotmail.com

Test question

(Selective Process - Plastic Surgery Medical Residency INCA/2016) - Which one is the best alternative that defines Pseudomyxoma peritonei?

a) Pseudomyxoma peritonei is a rare benign process of the peritoneal cavity that typically arises from an ovarian or an appendiceal adenocarcinoma rupture.

25%

b) Pseudomyxoma peritonei is a rare malignant process of the peritoneal cavity that typically arises from an ovarian or appendiceal carcinoma rupture.

25%

c) Pseudomyxoma peritonei is a rare malignant process of the peritoneal cavity that typically arises from an uterine lesion or a gastric adenocarcinoma.

25%

d) Pseudomyxoma peritonei is a rare benign process of the peritoneal cavity that typically arises from an uterine lesion or a gastric adenocarcinoma.

25%

e)

25%
   

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