Desmoid-type fibromatosis in the setting of malignant peritoneal mesothelioma: a case report of two rare diseases

Our plain-English summary of Desmoid-type fibromatosis in the setting of malignant peritoneal mesothelioma: a case report of two rare diseases, by S.N. Gregory, A.A. Satterwhite, H.R. Alexander, et al (World Journal of Surgical Oncology, 2022).

The study

• Authors: S.N. Gregory, A.A. Satterwhite, H.R. Alexander, A.M. Blakely
• Journal: World Journal of Surgical Oncology
• Published: 2022;20(1):317
• Type of article: Case report
• Cited by: 1 article, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Background: Desmoid-type fibromatosis (DTF) is a rare benign lesion that usually arises from the abdominal wall or extremities and rarely from the mesentery or intrabdominal organs. Malignant peritoneal mesothelioma is also a rare, yet aggressive disease. To our knowledge, this is the first case report of desmoid-type fibromatosis in the setting of malignant peritoneal mesothelioma.

Case Presentation: An early 30-year-old female was referred to our center for large intra-abdominal mass concerning for recurrent malignant peritoneal mesothelioma after previous cytoreductive surgery with hyperthermic intraperitoneal chemotherapy and adjuvant chemotherapy. Further investigation revealed a large mesenteric mass, which was resected en bloc with the cecum and terminal ileum. Pathologic findings confirmed a surprising diagnosis of desmoid-type fibromatosis.

Conclusions: No adjuvant therapy was offered to this patient due to negative tumor margins; however, close follow-up will be provided for recurrence of both malignant peritoneal mesothelioma and desmoid-type fibromatosis, which can be differentiated in the future via biopsy in this patient.

© 2022. The Author(s).

In plain English

This article is a case report about one patient. A Hispanic woman in her early 30s was diagnosed with peritoneal mesothelioma, a cancer of the lining of the abdomen, in 2017. In 2018 she had cytoreductive surgery with heated chemotherapy (mitomycin C) delivered into the abdomen, for well-differentiated epithelioid mesothelioma. She had no signs of recurrence for 2 years. She then developed fevers, abdominal pain and a mass with multiple nodules in the mesentery, the tissue that holds the bowel in place. The mass measured 10.5 by 10.7 cm on MRI and was only partly removed. After five cycles of cisplatin and pemetrexed, the mass was essentially stable. In July 2021 it was removed together with the cecum and the end of the small bowel. Tissue tests, including positive nuclear beta-catenin staining, showed desmoid-type fibromatosis rather than returning mesothelioma. The authors describe this as a benign but sometimes locally aggressive tumor. The surgical margins were negative, so no further therapy was given. The patient was alive with no signs of recurrence. The authors concluded that the case illustrates the use of immunohistochemistry to tell these similar rare conditions apart. The article does not discuss asbestos.

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 (one patient)
• Who was studied: One Hispanic woman in her early 30s, first seen in 2017 with peritoneal mesothelioma. She was treated in the USA from 2018 and later at the National Cancer Institute, Bethesda, Maryland, with surgery in July 2021.
• Exposure: The article does not report asbestos or any other exposure. It concerns the patient's diagnosis, treatment and pathology.
• Main results:
  • In 2018 the patient had cytoreductive surgery with heated intraperitoneal chemotherapy (HIPEC) using mitomycin C, for well-differentiated epithelioid mesothelioma.
  • After 2 years without recurrence, she had fevers, abdominal pain and a palpable abdominal mass. MRI showed a lesion measuring 10.5 by 10.7 cm and multiple mesenteric nodules. The mass was only partly excised.
  • After five cycles of neoadjuvant cisplatin and pemetrexed, CT in June 2021 showed the mesenteric mass essentially stable (9.3 by 5.7 by 7.3 cm).
  • The mass was removed in July 2021 together with the cecum and terminal ileum. Pathology showed spindle cells in a collagenous stroma, with nuclear beta-catenin, calretinin and CK7 positive. This gave a new diagnosis of desmoid-type fibromatosis.
  • The tumor margins were negative after this surgery, so no adjuvant therapy was offered.
  • At the time of writing, the patient was alive and under surveillance for both peritoneal mesothelioma and desmoid-type fibromatosis, with no signs of recurrence.
• The authors' conclusion: The authors concluded that desmoid-type fibromatosis can simulate mesothelioma. They wrote that this case illustrates the use of immunohistochemistry to tell similar rare conditions apart, so that patients receive correct treatment. They stated that, to their knowledge, no earlier case reports described this tumor in a patient who also had malignant peritoneal mesothelioma.

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, Mesentery, Cytoreductive Surgery, HIPEC, Chemotherapy, Adjuvant Therapy, Biopsy.

Related studies

• Immunoreactivity for calretinin and keratins in desmoid fibromatosis and other myofibroblastic tumors: a diagnostic pitfall — S. Barak, Z. Wang, et al. (2012)
• Malignant peritoneal mesothelioma in a 16-year-old girl: presentation of a rare disease — I.B. Brecht, A. Agaimy, et al. (2012)
• Rapamycin Treatment for Benign Multicystic Peritoneal Mesothelioma: A Rare Disease with a Difficult Management — G. Stallone, B. Infante, et al. (2017)
• The Challenge of Defining Treatment Standards for a Rare Disease: Malignant Peritoneal Mesothelioma — H.L. Kindler (2016)
• Benign multicystic peritoneal mesothelioma: a rare cause of intra-abdominal cystic disease — B. Campbell, D. Mehanna, et al. (2017)
• Patterns of peritoneal dissemination and response to systemic chemotherapy in common and rare peritoneal tumours treated by cytoreductive surgery: study protocol of a prospective, multicentre, observational study — A. Bhatt, P. Rousset, et al. (2021)

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