Background: Pseudomyxoma peritonei (PMP) is a rare condition characterized by mucinous tumors, disseminated intra-peritoneal implants, and mucinous ascites. So far its diagnosis remains challenging to most clinicians.
Case Presentation: A 55-year-old male patient had suffered from acute onset of abdominal pain and abdominal distension for one day prior to his admission. Physical examination revealed tenderness over the right lower quadrant of the abdomen without diffuse muscle guarding. A large amount of ascites was identified by abdominal computed tomography (CT) scan. Paracentesis showed the appearance of sticky mucinous ascites. He underwent laparotomy under the impression of pseudomyxoma peritonei. There was a lot of mucinous ascites, one appendiceal tumor and multiple peritoneal implants disseminated from the subphrenic space to the recto-vesicle pouch. Pseudomyxoma Peritonei caused by mucinous adenocarcinoma of appendiceal origin, was confirmed by histopathology. We performed an excision of the appendiceal tumor combined with copious irrigation and debridement. After the operation, he received 10 cycles of systemic chemotherapy with FOLFOX4 regimen, without specific morbidity. Follow-up of abdominal CT and colonoscopy at post-operative 17 months showed excellent response without evidence of local recurrence or distal metastasis. He made an uneventful recovery (up to the present) for 21 months after the operation.
Conclusion: This case report emphasizes the possible new role of systemic chemotherapy in the treatment of patients with this rare clinical syndrome.
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http://dx.doi.org/10.1186/1477-7819-6-118 | DOI Listing |
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.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!