Malignant Mesothelioma of the Peritoneum in Women: A Clinicopathologic Study of 164 Cases

Our plain-English summary of Malignant Mesothelioma of the Peritoneum in Women: A Clinicopathologic Study of 164 Cases, by A. Malpica, E.D. Euscher, M.L. Marques-Piubelli, et al (The American Journal of Surgical Pathology, 2021).

The study

• Authors: A. Malpica, E.D. Euscher, M.L. Marques-Piubelli, M.C. Ferrufino-Schmidt, R.N. Miranda, R. Sams, R.E. Royal, K.P.S. Raghav, K.F. Fournier, P. Ramalingam
• Journal: The American Journal of Surgical Pathology
• Published: 2021;45(1):45-58
• Cited by: 23 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Malignant mesothelioma of the peritoneum in women is an uncommon tumor. In this study, we present the clinicopathologic features of 164 such cases seen in our institution over a period of 42 years (1974-2016). Clinical information, pathologic findings, immunohistochemical results, and follow-up were recorded. Hematoxylin and eosin-stained slides were reviewed in all cases. Patients ranged in age from 3 to 85 years, median: 49 years. Most patients presented with abdominal/pelvic pain, although some were asymptomatic, presented with paraneoplastic syndromes or cervical lymphadenopathy. Overall, 9% of patients had a history of direct or indirect exposure to asbestos. In total, 31% and 69% of patients had either a personal or family history of other tumors; most of these tumors are currently recognized as part of a syndrome. Genetic testing information was available in 5 patients: BAP-1 germline mutation (1), type 2 neurofibromatosis (1), Lynch syndrome (1), McCune-Albright syndrome (1), no BAP-1 or TP53 mutation (1). Most cases had gross and microscopic features typical of malignant mesothelioma of the peritoneum in women; however, some had confounding features such as gelatinous appearance, signet ring or clear cells, and well-differentiated papillary mesothelioma-like areas. Calretinin and WT-1 were the markers more frequently expressed, and up to 23% of the cases showed PAX-8 expression. Patients' treatments predominantly included: chemotherapy, cytoreductive surgery, and hyperthermic intraperitoneal chemotherapy. On multivariate analysis, the predominance of deciduoid cells, nuclear grade 3, and the absence of surgical treatment were associated with worse overall survival (OS). For all patients, the 3- and 5-year OS were 74.3% and 57.4%, respectively. The 3- and 5-year OS for patients treated with cytoreductive surgery, and hyperthermic intraperitoneal chemotherapy were 88.9% and 77.8%, respectively.

In plain English

This retrospective study described 164 women and girls with malignant mesothelioma of the peritoneum (the lining of the abdomen). Cases came from The University of Texas MD Anderson Cancer Center, 1974 to 2016. The authors reviewed tissue slides, medical records and follow-up. Patients were aged 3 to 85 years (median 49). Direct or indirect asbestos exposure was documented in 11 of 122 patients (9%); no talc use was documented. Thirty-eight (31.1%) had a personal history of other tumors (the article gives the denominator as both 124 and 122), and 79 of 114 had a family history of tumors. In 73 of 147 referred or consult cases, the first diagnosis differed from the final one. For all patients, survival was 74.3% at 3 years and 57.4% at 5 years. With cytoreductive surgery plus heated abdominal chemotherapy (HIPEC), the results section gave 91.8% and 85.3%, and the abstract gave 88.9% and 77.8%. Mostly deciduoid cells, nuclear grade 3 and no surgery were associated with worse survival. The authors concluded that with current treatments this cancer does not have the dismal prognosis previously reported. They noted that treatment was not controlled.

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: Retrospective clinicopathologic case series from a single institution (pathology review plus clinical records and follow-up, with survival analysis)
• Who was studied: 164 female patients with malignant mesothelioma of the peritoneum from the pathology files of The University of Texas MD Anderson Cancer Center, Houston, 1974 to 2016 (81 referrals, 67 consults, 16 in-house cases)
• Exposure: Asbestos exposure was recorded as a history item only. It was direct (patient-reported, at home or work) or indirect (spouse or first-degree relative reported occupational exposure). No fiber types or doses were given. Talc use was also recorded.
• Main results:
  • Patients were aged 3 to 85 years (n=161; mean 50, median 49).
  • Asbestos exposure (direct or indirect) was documented in 11 (9%) of 122 patients. No talc use was documented in any of 122 patients.
  • 38 patients (31.1%) had a personal history of other neoplasms. The results text gives the denominator as 124, while Table 2 gives 122. The 31.1% figure matches 122.
  • 79 (69.3%) of 114 patients had a family history of other neoplasms.
  • Genetic testing information was available for only 5 patients. Results were: BAP-1 germline mutation (1), type 2 neurofibromatosis (1), Lynch syndrome (1), McCune-Albright syndrome (1), and 1 patient negative for BAP-1 germline and TP53 mutations.
  • Tumors were epithelioid in 132 cases and biphasic in 32. None was purely sarcomatoid.
  • In referral and consult cases, the final diagnosis differed from the initial diagnosis in 73 (49.65%) of 147 cases. Carcinoma (40) was the most common initial diagnosis.
  • PAX-8 was positive in 22.5% of cases tested (9 of 40). WT-1 was positive in all 46 cases tested.
  • For all patients, mean overall survival was 98 months (95% CI 80-115) and median overall survival was 75 months (95% CI 42-107). Overall survival was 74.3% at 3 years and 57.4% at 5 years.
  • In the results section, patients treated with cytoreductive surgery (CRS) and HIPEC had a mean overall survival of 104 months (95% CI 92-117). Their overall survival was 91.8% at 3 years and 85.3% at 5 years.
  • The article's own abstract gives different 3- and 5-year overall survival for the CRS and HIPEC group: 88.9% and 77.8%.
  • On multivariate analysis, three factors were associated with worse overall survival: a predominance of deciduoid cells (P=0.045), nuclear grade 3 (P less than 0.001), and absence of surgical treatment (P=0.044).
  • Elevated serum CA125 was not correlated with overall survival.
• The authors' conclusion: The authors concluded that, with current treatment modalities, malignant mesothelioma of the peritoneum in women does not have the dismal prognosis previously reported.
• Limitations the authors state:
  • Treatment was not controlled. Patients received many different treatments over the 42-year span, many first at other hospitals.
  • The authors could not assess newer antibodies (such as BAP-1, claudin-4, HEG1 and MTAP) or CDKN2A deletion by FISH.
  • The high rate of other tumors may reflect sample size and selection bias, because the study was done in a tertiary cancer hospital.
  • Because the study was retrospective and spanned a long period, genetic testing information was available for only 5 patients.
  • Few cases were tested for GATA-3 (7).

Topics

The National Library of Medicine indexes this study under subjects our site covers: Mesothelioma, Peritoneal mesothelioma.

Medical terms in this study, explained in our Lexicon: Mesothelioma, Peritoneum, Lymphadenopathy, Germline Mutation, Confounding, Well-Differentiated Papillary Mesothelioma, Calretinin, Wilms Tumor 1 (WT-1).

Related studies

• Aberrant Pax-8 expression in well-differentiated papillary mesothelioma and malignant mesothelioma of the peritoneum: a clinicopathologic study — D. Xing, N. Banet, et al. (2018)
• Well-differentiated papillary mesothelioma of the female peritoneum: a clinicopathologic study of 26 cases — A. Malpica, S. Sant'Ambrogio, et al. (2012)
• Papillary tumors of the peritoneum in women: mesothelioma or papillary carcinoma — M. Kannerstein, J. Churg, et al. (1977)
• Primary papillary serous neoplasia of the peritoneum: a clinicopathologic and ultrastructural study of eight cases — U. Raju, G. Fine, et al. (1989)
• Pseudomesotheliomatous carcinoma involving pleura and peritoneum: A clinicopathologic and immunohistochemical study of three cases — I.A. Shah, J.R. Salvatore, et al. (1999)
• Diffuse mesothelioma of the peritoneum: correlation between histological and clinical parameters and survival in 73 patients — S. Liu, P. Staats, et al. (2014)

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