Pseudomyxoma peritonei: a clinical case of this poorly understood condition

Oliveira, Ana Maria de; Rodrigues, Catarina; Borges, Alexys; Martins, Alexandra; Santos, Sofia Loureiro dos; Pires, Francisco Rocha; Araújo, João Mascarenhas

doi:10.2147/ijgm.s51504

Cited by 4 publications

(11 citation statements)

References 21 publications

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“…To make a definitive diagnosis of PMP, the presence of mucinous neoplastic cells and epithelial glandular cells should be demonstrated histologically in the pathologic specimen, and diffuse mucinous material and implants in the abdomen should also be present clinically. If epithelial cells are absent, the term “mucinous ascites” may be more appropriate [ 13 , 19 ]. Mucin is produced by appendiceal goblet cells and has tight junctions with the surrounding stroma.…”

Section: Discussionmentioning

confidence: 99%

“…PMP typically affects female patients, and its most distinctive clinical manifestation is abdominal distension and discomfort, which can give rise to a “Jelly Belly” appearance characterized by diffuse abdominal swelling. In some cases, milder forms of the disease may present with abdominal pain without accompanying abdominal swelling, which can potentially be mistaken for acute appendicitis [ 12 , 14 , 16 , 19 ]. Due to increased intra-abdominal pressure, PMP may sometimes present as a newly developed hernia or uterine prolapse, and some patients may be incidentally diagnosed with PMP during surgery for these aforementioned reasons.…”

Section: Discussionmentioning

confidence: 99%

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Pseudomyxoma peritonei: The struggle of a lifetime and the hope of a cure - a rare diagnosis with review of the literature

Askar

2024

North Clin Istanbul

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Pseudomyxoma peritonei is a rare pathological condition characterized by mucinous tumor tissue implants on the peritoneal surface. Although the cause of Pseudomyxoma peritonei has been extensively studied, the prevailing agreement is that it stems from mucinous tumors that occur in the ovaries or appendix. The tumor tissue typically remains localized to the peritoneum and does not exhibit extraperitoneal spread. Patients with Pseudomyxoma peritonei may present with symptoms such as abdominal pain, bloating, loss of appetite, and shortness of breath. Computerized Tomography is commonly used for diagnostic purposes. The treatment of Pseudomyxoma peritonei typically involves surgical evacuation of the tumoral tissue, followed by cytoreduction and Hyperthermic Intraperitoneal Chemotherapy. While effective treatment options are available, some patients may require repeated surgeries over an extended period. This paper reports on a case study of a patient with a history of recurrent Pseudomyxoma peritonei, necessitating multiple surgical interventions over a decade. The paper concludes with a review of the relevant literature.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Pseudomyxoma peritonei: The struggle of a lifetime and the hope of a cure - a rare diagnosis with review of the literature

Askar

2024

North Clin Istanbul

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“…The diagnosis of PMP, a rare clinical syndrome, is difficult[ 20 , 21 ]. Commonly, the presenting symptom is increasing abdominal girth.…”

Section: Discussionmentioning

confidence: 99%

Pseudomyxoma peritonei originating from intestinal duplication: A case report and review of the literature

Han

Zhou

et al. 2021

WJCC

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BACKGROUND Pseudomyxoma peritonei (PMP) is a rare mucinous neoplasm with a relatively low incidence of 1 to 2 per million individuals. It is typically characterized by a type of gelatinous ascites named “jelly belly”. Most cases of PMP occur in association with ruptured primary mucinous tumors of the appendix (90%). Periodically, PMP can originate from mucinous carcinomas at other sites, including the colorectum, gallbladder, and pancreas. However, unusual origin can occur, as noted in this case report. CASE SUMMARY A 52-year-old woman had an unusual derivation of PMP from intestinal duplication. The patient complained of abdominal distension and increasing abdominal girth. Abdominal contrast-enhanced computed tomography showed a mass in the greater omentum located on the left side of the abdomen, likely to be a cystic mass of peritoneal origin. A PMP diagnosis was presumed based on the specific signs of the mass with flocculent and stripe-like echoes in ultrasound images. Ultrasound-guided percutaneous aspiration suggested a high likelihood of PMP. Once the PMP diagnosis was recognized, identification of the origin of the primary tumor was indicated. Thus, an exploratory laparoscopy was performed. In the absence of a primary tumor of appendix origin, the diagnosis of a low-grade mucinous neoplasm of intestinal duplication origin was finally confirmed by histopathology. CONCLUSION PMP is secondary to mucinous carcinomas of the appendix mostly. This case resulted from an unusual derivation from intestinal duplication.

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“…It is characterized by mucin production in the abdominal cavity and, if left untreated, can compress adjacent vital organs. The term “PMP” was first given by Werth in 1884, and the current incidence is estimated to be one to two cases per million per year [ 1 ]. With a mean incidence at the age of 53 years, PMP is more prevalent in women (male-to-female ratio, 9:11) [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Pseudomyxoma peritonei leading to “jelly belly” abdomen: a case report and review of the literature

Garg,

Peer

et al. 2024

J Med Case Reports

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Background Pseudomyxoma peritonei is an infrequent condition with a global annual incidence of only one to two cases per million people. Mucinous neoplasms, widespread intraperitoneal implants, and mucinous ascites characterize it. Currently, most clinicians misdiagnose this condition, which leads to delayed management. Case presentation A 44-year-old North Indian female presented with a 1.5-month history of an abdominal lump. Physical examination revealed a sizeable abdominopelvic mass at 36 weeks. Contrast-enhanced computed tomography showed a massive multiloculated right ovarian cystic mass measuring 28 × 23 × 13 cm with mild ascites and elevated carcinoembryonic antigen levels (113.75 ng/ml). A provisional diagnosis of ovarian mucinous neoplasm was made, for which the patient underwent laparotomy. Intraoperatively, there were gross mucinous ascites, along with a large, circumscribed, ruptured right ovarian tumor filled with gelatinous material. The appendicular lump was also filled with mucinous material along with the omentum, ascending colon, right lateral aspect of the rectum, splenic surface, and small bowel mesentery. Cytoreductive surgery was performed along with an oncosurgeon, including total abdominal hysterectomy with bilateral salpingoophorectomy, omentectomy, right hemicolectomy, lower anterior resection, ileo-transverse stapled anastomosis with proximal ileal loop diversion stoma, excision of multiple peritoneal gelatinous implants, and peritoneal lavage. Histopathology and immunohistochemistry confirmed the presence of intestinal-type mucinous carcinoma. Postoperatively, the patient was given six cycles of chemotherapy. She tolerated it without any specific morbidity and had an uneventful recovery. Postoperative follow-up at 15 months revealed normal tumor marker levels and abdominal computed tomography findings and no signs suggestive of local recurrence or distal metastases. Conclusions Pseudomyxoma peritonei is a rare disease that is frequently misdiagnosed in the preoperative phase. Therefore, radiologists and clinicians should maintain a high index of suspicion for accurate diagnosis and multidisciplinary management.

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Pseudomyxoma peritonei: a clinical case of this poorly understood condition

Cited by 4 publications

References 21 publications

Pseudomyxoma peritonei: The struggle of a lifetime and the hope of a cure - a rare diagnosis with review of the literature

Pseudomyxoma peritonei: The struggle of a lifetime and the hope of a cure - a rare diagnosis with review of the literature

Pseudomyxoma peritonei originating from intestinal duplication: A case report and review of the literature

Pseudomyxoma peritonei leading to “jelly belly” abdomen: a case report and review of the literature

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