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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.
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PURPOSE: Risk-reducing surgery for cancer prevention in solid tumors is a pressing clinical topic because of the increasing availability of germline genetic testing. We examined the short- and long-term outcomes of risk-reducing total gastrectomy (RRTG) and its lesser-known impacts on health-related quality of life (QOL) in individuals with hereditary diffuse gastric cancer syndrome. METHODS: Individuals who underwent RRTG as part of a single-institution natural history study of hereditary gastric cancers were examined. Clinicopathologic details, acute and chronic operative morbidity, and health-related QOL were assessed. Validated questionnaires were used to determine QOL scores and psycho-social-spiritual measures of healing. RESULTS: One hundred twenty-six individuals underwent RRTG because of a pathogenic or likely pathogenic germline CDH1 variant between October 2017 and December 2021. Most patients (87.3%; 110/126) had pT1aN0 gastric carcinoma with signet ring cell features on final pathology. Acute (<30 days) postoperative major morbidity was low (5.6%; 7/126) and nearly all patients (98.4%) lost weight after total gastrectomy. At 2 years after gastrectomy, 94% (64/68) of patients exhibited at least one chronic complication (ie, bile reflux, dysphagia, and micronutrient deficiency). Occupation change (23.5%), divorce (3%), and alcohol dependence (1.5%) were life-altering consequences attributed to total gastrectomy by some patients. In patients with a median follow-up of 24 months, QOL scores decreased at 1 month after gastrectomy and returned to baseline by 6-12 months. CONCLUSION: RRTG is associated with life-changing adverse events that should be discussed when counseling patients with CDH1 variants about gastric cancer prevention. The risks of cancer-prevention surgery should not only be judged in the context of likelihood of death due to disease if left untreated, but also based on the real consequences of organ removal.