ABSTRACT
Background: Mesothelioma is a rare, aggressive disease originating from mesothelial cells and carries a poor prognosis. Mesothelioma may arise from the pleura, pericardium, or peritoneum. Peritoneal mesothelioma (PM) usually spreads in a diffuse manner; however, a localized unifocal form of PM may occur. Literature on unifocal mesothelioma remains scarce. Case Description: Herein, we highlight a case of localized epithelioid PM in an 81-year-old gentleman with the unique challenges faced during management. The pelvic mass was 7 cm, well-circumscribed, and hyper-vascular with fibrous attachments to the abdominal wall. The patient had a peritoneal cancer index (PCI) of 4 on initial diagnostic laparoscopy. Diagnosis was confirmed by histology. Resection of the mass with a partial omentectomy was performed. Months later, the patient developed recurrence detected on follow-up imaging in the peri-splenic region. The patient underwent cytoreductive surgery (CRS) and heated intraperitoneal chemotherapy (HIPEC) for 60 minutes using mitomycin C and cisplatin followed by an uneventful recovery. Our case report is followed by a review of literature on disease pathophysiology, treatment options, and recently promising immunotherapy approaches. Conclusions: CRS and HIPEC remains the standard treatment regimen for patients with PM. Nonetheless, a more nuanced approach might be indicated in specific patients with localized unifocal PM. Disease distribution and burden may impact the decision on surgical management in selected patients.
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.