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

World J Surg Oncol. 2022 Sep 28;20(1):317. doi: 10.1186/s12957-022-02784-y.

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.

Keywords: Cytoreduction; Desmoid-type fibromatosis; Hyperthermic intraperitoneal chemotherapy; Malignant peritoneal mesothelioma; Mesothelioma.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Female
  • Fibromatosis, Aggressive* / pathology
  • Humans
  • Mesothelioma* / pathology
  • Mesothelioma* / surgery
  • Mesothelioma, Malignant*
  • Neoplasm Recurrence, Local
  • Peritoneal Neoplasms* / diagnosis
  • Peritoneal Neoplasms* / surgery
  • Rare Diseases