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The Desmoid Tumor Research Foundation

September 2022

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

Author Information

Gregory SN (1, 2), Satterwhite AA (1), Alexander HR (2), Blakely AM (3).

(1) Department of Surgical Oncology, National Cancer Institute, National Institutes of Health, 10 Center Drive, Bethesda, MD, 20892, USA. (2) Department of Surgical Oncology, Rutgers Cancer Institute of New Jersey, Rutgers Robert Wood Johnson University Hospital, 195 Little Albany St, New Brunswick, NJ, 08901, USA. (3) Department of Surgical Oncology, National Cancer Institute, National Institutes of Health, 10 Center Drive, Bethesda, MD, 20892, USA. andrew.blakely@nih.gov.

Publication

World J Surg Oncol

Summary

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.

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