Title: Pseudomyxoma Peritonei
Authors: Dr Nikita Gandotra, Dr Abhinav Sharma
DOI: http://dx.doi.org/10.18535/jmscr/v3i9.32
Pseudomyxoma peritonei syndrome is characterized by mucinous ascites and mucinous tumor disseminated on peritoneal surfaces. The disease almost always originates from a perforated appendiceal epithelial tumor. Appendix tumors are unusual, accounting for 0.4% of all of the gastrointestinal tract malignancies. Although rare, the spectrum of malignant disease is complex and has lead to confession in accurate description of the natural history of these tumors. Consequently, many errors in diagnosis and treatment have occurred. Perhaps the most glaring error in management occurs in women who have ovarian tumors as a result of a perforated primary mucinous tumor of the appendix. Appendiceal tumors present with peritoneal seeding in a majority of patients. Dissemination to lymph nodes or to liver is extremely unusual. Advanced treatments of peritoneal carcinomatosis or peritoneal adenomucinosis have changed these survival rates from zero to approximately 80% for all patients.The term “mucocele” of the appendix refers to an accumulation of mucus within an abnormally distended appendiceal lumen, regardless of its cause. Mucoceles of the appendix are rare, appearing in 0.2-0.3 % of surgical specimens. A mucocele of the appendix can be caused by: obstruction of the appendiceal lumen, mucosal hyperplasia, mucinous cystadenoma, mucinous cystadenocarcinoma(4). Whatever the cause, obstruction of the lumen and accumulation of yellow mucous within the appendiceal lumen results. Majority of patients with mucocele are asymptomatic. Most mucocele present with acute or chronic right lower quadrant pain (64%) but patients have presented with intussussception gastrointestinal bleeding, intermittent colicky pain, abdominal masses, secondary infection and urologic symptoms.
Abstract