Pseudomyxoma peritonei was diagnosed in 3 men aged 38, 66 and 54 years with weight loss and distension of the abdomen. Pseudomyxoma peritonei results from seeding of the peritoneal cavity with mucus-producing epithelium. The disease is traditionally characterized by accumulation of huge amounts of mucinous ascites, relatively long survival and absence of distant, extraperitoneal metastases. Mostly, the primary tumour is an appendicular adenoma or adenocarcinoma. Sometimes, the primary tumor is localized in the ovaries. Extensive surgical debulking with postoperative intraperitoneal chemotherapy appears to be the treatment of choice.
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Appl Immunohistochem Mol Morphol
January 2025
Department of Pathology.
Histologic grade is a key predictor for pseudomyxoma peritonei (PMP) of appendiceal origin that is used to guide clinical management. However, some tumors demonstrate disease behavior that deviates from their histologic grade. A recent study suggested that TP53, GNAS, and RAS mutation analysis could stratify tumors into distinct molecular groups with different prognosis.
View Article and Find Full Text PDFCureus
December 2024
Department of Surgery, Kitakyushu City Yahata Hospital, Kitakyushu, JPN.
A low-grade appendiceal mucinous neoplasm (LAMN) is a rare condition, occurring in 0.08-4.1% of appendectomy cases.
View Article and Find Full Text PDFMedicine (Baltimore)
November 2024
Department of Myxoma, Aerospace Center Hospital, Beijing, China.
The necessity of prophylactic cytoreductive surgery (PCRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) for low-grade appendiceal mucinous neoplasms (LAMN) after complete removal is still controversial. This study aims to determine the role of PCRS + HIPEC and identify optimal strategies for managing these patients. One hundred fifty-nine patients who sought medical advice at Aerospace Center Hospital were retrospectively analyzed from January 2011 to December 2021.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Division of Thoracic Surgery, Mayo Clinic, Rochester, Minnesota.
Pleural extension of pseudomyxoma peritonei is rare, and treatment demands multidisciplinary care. Perioperative management during cytoreductive surgery and hyperthermic intrathoracic chemotherapy challenges anesthesiology and surgical teams in unique ways. Hemodynamic, arrhythmogenic, ventilatory, fluid balance, acid-base, and nephroprotection issues are important considerations.
View Article and Find Full Text PDFCancers (Basel)
December 2024
General Surgery Department, Università Cattolica del Sacro Cuore, 00168 Rome, Italy.
Advances in cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) and pressurized intraperitoneal aerosol chemotherapy (PIPAC) have improved outcomes for selected patients with peritoneal surface malignancies (PSMs). This retrospective study analyzed 743 PSM patients treated at Fondazione Policlinico Universitario Agostino Gemelli from January 2016 to February 2024. The primary aim was to assess median overall survival (mOS), median disease-free survival (mDFS), and median progression-free survival (mPFS) stratified by tumor origin.
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