Intra-Operative Diagnosis of Benign Multicystic Peritoneal Mesothelioma: A Case Report of Rare Entity and Lessons Learned

Our plain-English summary of Intra-Operative Diagnosis of Benign Multicystic Peritoneal Mesothelioma: A Case Report of Rare Entity and Lessons Learned, by C.C. Wu, A. Bassi, A. Onitilo, et al (Curēus, 2024).

The study

• Authors: C.C. Wu, A. Bassi, A. Onitilo, R. Sharma
• Journal: Curēus
• Published: 2024;16(5):e60664
• Type of article: Case report
• Cited by: 5 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Benign multicystic peritoneal mesothelioma (BMPM), also known as multicystic peritoneal mesothelioma (MCPM), is a rare cystic neoplasm arising from the mesothelium lining of the abdominal and pelvic peritoneum. This entity has been disproportionately described in women of reproductive age. Both the etiology and pathogenesis of the condition are not well understood. Preoperative diagnosis is challenging as differentials are varied and include endometriosis, lymphangioma, pseudomyxoma peritonei, cystic adenomatoid tumor, and malignant peritoneal mesothelioma. Management options include cytoreductive surgery (CRS) with or without heated intraperitoneal chemotherapy (HIPEC). In this case report, we highlight the complexity of preoperative diagnosis, presentation, workup, treatment, and management of BMPM. We report the case of a female patient presenting with abdominal pain and imagining consistent with cystic intra-abdominal lesions. After an inconclusive percutaneous biopsy and a multi-disciplinary tumor board discussion, the patient was offered CRS with HIPEC. Intra-operative frozen section indicated benign epithelial lined cysts. CRS and HIPEC were performed. After a second opinion, the lesions were confirmed by pathology and immunohistochemistry to be BMPM. In this report, we discuss the gold standard of care for patients with BMPM to improve the disease control rate. This pathway is proposed in our study, and, thus, we conclude that BMPM should be considered in the differential diagnosis of patients presenting with symptomatic multiple intraperitoneal cystic lesions.

Copyright © 2024, Wu et al.

In plain English

This article is a case report of one patient with benign multicystic peritoneal mesothelioma (BMPM), a rare cystic tumor of the abdominal lining. It does not mention asbestos or any other exposure. The patient, a 43-year-old woman, had two months of lower abdominal pain. Ultrasound showed a 15.4 cm mass, and a needle biopsy found no mucin or malignancy. Before surgery her CA-125 blood marker was elevated (107.7 U/mL in the text, 84.4 U/mL in the table). At open surgery, many thin-walled, clear-fluid cysts were removed along with several organs. A frozen section taken during surgery showed simple peritoneal cysts, and heated cisplatin chemotherapy was given inside the abdomen. Outside pathologists confirmed BMPM. She left hospital on day 4 without complications and had no symptoms at one-year follow-up. Her CA-125 stayed in the normal range two years on. The authors concluded that BMPM should be considered when diagnosing patients who have symptoms and multiple cystic lesions inside the peritoneal cavity. They highlighted heated chemotherapy at the first operation and called for further research.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Case report (single patient)
• Who was studied: One 43-year-old woman with a possible history of polycystic ovarian syndrome and no previous abdominal surgery or peritoneal inflammation. Location and dates of care are not stated.
• Exposure: Not reported: the article does not mention asbestos or any other environmental or occupational exposure for this patient
• Main results:
  • Before surgery, ultrasound showed a large right adnexal mass with septations and solid components.
  • A percutaneous ultrasound-guided core needle biopsy before surgery did not show mucin or malignancy.
  • The patient's CA-125 was elevated before surgery: 107.7 U/mL in the text (84.4 U/mL in Table 1), against a reference range of 0 to 30.2 U/mL. Her CA 19-9 and CEA were normal.
  • At surgery, multiple cysts were found within the peritoneum. Both ovaries and fallopian tubes, the omentum, appendix and gallbladder were removed, and tumors were resected.
  • The frozen section taken during surgery showed simple peritoneal cysts.
  • Closed-circuit heated intraperitoneal chemotherapy (HIPEC) with cisplatin was given, with a total dwell time of 90 minutes.
  • Final pathology, on second opinion at another institution, confirmed BMPM. The lesion stained positive for calretinin, CK5/6, WT1 and BAP1, and negative for MOC31.
  • Fluorescence in situ hybridization found no homozygous deletion of CDKN2A/B (printed as CKD2NA/B). The authors took this as supporting the benign nature of the disease.
  • The patient was discharged without complications on postoperative day 4.
  • At one-year follow-up the patient had no symptoms, and CT showed resolution of the peritoneal mass.
  • After surgery and HIPEC, the patient's CA-125 fell to 7.0 (no unit given) and remained in the reference range two years after follow-up.
• The authors' conclusion: The authors concluded that BMPM should be considered in the differential diagnosis of patients presenting with symptomatic multiple intraperitoneal cystic lesions. They also noted that BMPM's cause is poorly understood and that there is no generally accepted treatment. Because surgery alone has a high recurrence rate, they highlighted HIPEC at the first operation. They called for further research on optimal management.

Topics

Medical terms in this study, explained in our Lexicon: Peritoneal Mesothelioma, Neoplasm, Mesothelium, Peritoneum, Etiology, Adenomatoid Tumor, Cytoreductive Surgery, Chemotherapy.

Related studies

• Benign multicystic peritoneal mesothelioma: a rare cause of intra-abdominal cystic disease — B. Campbell, D. Mehanna, et al. (2017)
• Multicystic mesothelioma: Operative and long-term outcomes with cytoreductive surgery and hyperthermic intra peritoneal chemotherapy — E. Nizri, D. Baratti, et al. (2018)
• Intra-abdominal benign multicystic peritoneal mesothelioma — I. Jouvin, A. Dohan, et al. (2014)
• Intra-abdominal cystic lymphangioma of the mesocolon sigmoids: a rare entity in adult patient woman — A. Guachilema Ribadeneira, Á.R.T. Monard, et al. (2020)
• Benign Multicystic Peritoneal Mesothelioma: A Rare Condition in an Uncommon Gender — M.S. Khurram, H. Shaikh, et al. (2017)
• Benign Multicystic Peritoneal Mesothelioma (BMPM) as a rare cause of abdominal pain in a young male: case report and review of the literature — S. Gussago, P. Spina, et al. (2019)

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