Malignant peritoneal mesothelioma in a 16-year-old girl: presentation of a rare disease

Our plain-English summary of Malignant peritoneal mesothelioma in a 16-year-old girl: presentation of a rare disease, by I.B. Brecht, A. Agaimy, M. Besendörfer, et al (Klinische Pädiatrie, 2012).

The study

• Authors: I.B. Brecht, A. Agaimy, M. Besendörfer, R. Carbon, F.C. Thiel, O. Rompel, D. Osinski, T. Langer, M. Metzler, W. Holter
• Journal: Klinische Pädiatrie
• Published: 2012;224(3):170-3
• Type of article: Case report
• Cited by: 9 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Malignant peritoneal mesothelioma is extremely rarely seen in young patients.A 16 year-old girl presented with appendicitis-like acute abdominal pain. Intra-operatively, multiple confluent peritoneal nodules were seen on the entire greater omentum and in the pelvis infiltrating the uterus and both ovaries. Biopsies were obtained and interpreted as serous ovarian carcinoma. Radical surgical resection and hyperthermic intraperitoneal chemotherapy -(HIPEC) with carboplatin was performed and followed by 2 cycles of carboplatin/paclitaxel. Histological reevaluation showed characteristic features of epithelioid peritoneal mesothelioma and ruled out serous ovarian cancer. Therapy was continued with 6 cycles of pemetrexed/cisplatin.3 months after end of chemotherapy vital tumor tissue was found in the recess behind the liver, which could be resected completely. The patient is currently disease-free 17 months after initial diagnosis.Malignant peritoneal mesothelioma in young female patients might be under-recognized and possibly misdiagnosed as ovarian serous carcinoma in some cases. International and interdisciplinary cooperation is necessary in order to provide evidence based guidelines for diagnosis and treatment in the future.

© Georg Thieme Verlag KG Stuttgart · New York.

In plain English

This case report from University Hospital Erlangen, Germany, described a 16-year-old girl with malignant peritoneal mesothelioma. She had sudden lower right abdominal pain, and surgery for suspected appendicitis found many tumor nodules on the greater omentum and both ovaries. Two pathologists first diagnosed serous adenocarcinoma arising from the ovaries. She then had her ovaries, fallopian tubes, uterus, omentum, part of the abdominal lining and lymph nodes removed (10 of 53 nodes contained tumor). She also received heated chemotherapy in the abdomen during surgery and two cycles of carboplatin and paclitaxel. Re-examination of the tissue with additional staining (immunohistochemistry) changed the diagnosis to epithelial-type primary peritoneal mesothelioma, and treatment switched to six cycles of pemetrexed and cisplatin. Three months after chemotherapy ended, a 0.6 cm tumor between the liver and kidney was found and completely removed. Seventeen months after diagnosis she was in complete remission, but she was infertile and needed estrogen replacement. The article did not describe any history of asbestos exposure for her. The authors concluded that this disease in young females might be under-recognized and sometimes misdiagnosed as ovarian serous carcinoma.

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 16-year-old girl, treated at University Hospital Erlangen, Germany (dates of treatment not given)
• Exposure: Not reported: the article gives no history of asbestos or other exposure for this patient
• Main results:
  • The local pathologist and a second-opinion pathologist first diagnosed a moderately to poorly differentiated serous adenocarcinoma arising from the ovaries
  • After radical surgery, 10 of 53 para-aortic and pelvic lymph nodes showed metastatic deposits
  • On re-evaluation, immunohistochemistry excluded serous carcinoma, and the diagnosis was revised to primary peritoneal mesothelioma of the epithelial type, confirmed by central referral pathology
  • Three months after the end of therapy, biopsy showed vital mesothelioma tissue between the liver and kidney. The tumor measured 0.6 cm and was removed completely.
  • At 17 months after diagnosis, the patient was still in complete remission
  • Treatment left the patient infertile because of the hysterectomy, and with hypergonadotropic hypogonadism after removal of both ovaries, requiring estrogen replacement
• The authors' conclusion: The authors concluded that malignant peritoneal mesothelioma in young female patients might be under-recognized and possibly misdiagnosed as ovarian serous carcinoma in some cases. They called for international, interdisciplinary cooperation to develop evidence-based guidelines.
• Limitations the authors state:
  • The authors stated that the final prognosis is difficult to predict because reliable survival data for pediatric patients do not exist
  • The authors stated it is difficult to judge the role of chemotherapy in this case

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: Peritoneal Mesothelioma, Omentum, Carcinoma, Surgical Resection, HIPEC, Carboplatin, Epithelioid, Ovarian Cancer.

Related studies

• The Challenge of Defining Treatment Standards for a Rare Disease: Malignant Peritoneal Mesothelioma — H.L. Kindler (2016)
• What made hyperthermic intraperitoneal chemotherapy an effective curative treatment for peritoneal surface malignancy: A 25-year experience with 1,125 procedures — G. Passot, D. Vaudoyer, et al. (2016)
• A novel nomogram for peritoneal mesothelioma predicts survival — N.P. Schaub, M. Alimchandani, et al. (2013)
• The IASLC Mesothelioma Staging Project: Improving Staging of a Rare Disease Through International Participation — H. Pass, D. Giroux, et al. (2016)
• Primary pericardial mesothelioma and asbestos exposure: a rare fatal disease — D.M.M. Freitas, R.L. Ramos, et al. (2017)
• Clinical presentation of peritoneal mesothelioma — Y.I. Acherman, L.S. Welch, et al. (2003)

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