Four years later (2003), the patient began experiencing obstructive symptoms again due to recurrent disease and he had a further extensive debulking surgery in January 2004. Since then he remained well and was reviewed regularly in the outpatient clinic. Debulking surgery with small bowel resections was performed following this diagnosis at the Royal University Hospital of Saskatchewan. However, at the time of laparoscopy, a large gelatinous mass was found and the diagnosis of PMP was confirmed by histopathology. A laparoscopy was performed for tissue diagnosis of a possible lymphoma. A computed tomographic (CT) scan of his abdomen in January 2000 demonstrated a large abdominal mass. He had undergone multiple investigations and was thought to have underlying Crohn's disease. He was asymptomatic until the fall of 1999 when he started complaining of recurrent small bowel obstruction and weight loss. Although both underwent surgery and one had adjuvant chemotherapy, a protracted clinical course with chronicity and relapse of the disease was a common feature.Ĭase 1 This is a 42 years old male with a history of a ruptured appendiceal mucinous neoplasm that was removed 23 years ago. We report two cases of delayed PMP in men associated with appendiceal lesions with a long latent period in one case. Advanced abdominal disease leading to intestinal obstruction accounts for majority of the patients' morbidity and mortality. 10% of patients die of PMP within 5.5 years of their initial presentation while others experience recurrent and/or residual disease. There is increasing recognition that the two variants DPAM and PMCA are different with the DPAM remaining localized to the abdomen without metastatic behavior and the PCMA behaving like a mucinous (colloid) carcinoma with metastatic and invasive potential. The disease may be localized in the right lower quadrant initially and then become more generalized with mucinous peritoneal, serosal and ommental implants. PMP can occur years (range from 5–35 years) later after the initial presentation of an appendiceal event and, therefore, accurate diagnosis prior to surgery is often delayed and inaccurate. These primary sites are usually associated with the malignant variant-PMCA of PMP. Other possible primary sites include colorectum, gallbladder, pancreas, urachus, urinary bladder, breast and lung, but these are uncommon. Though uncommon in men, these cases are virtually all associated with a lesion in the appendix. Pseudomyxoma peritonei (PMP) is more commonly seen in women who usually present with increasing abdominal girth and this tends to be related to an underlying ovarian lesions which are usually mucinous tumors that can be associated with a teratoma. Cases without epithelium are regarded as mucinous ascites. Viable epithelial glandular cells must be identified within the mucin pools by histological analysis to diagnose PMP. Some authors also require the presence of diffuse mucinous implants for this diagnosis. A definitive diagnosis of PMP requires the presence of a) mucinous neoplastic cells/epithelium, and b) mucinous ascites – diffuse intraabdominal mucin. Other terminologies to reflect this spectrum of biological behavior ranges from disseminated peritoneal adeno mucinosis ( DPAM) – the benign variant to peritoneal mucinous carcinom atosis ( PMCA) – the malignant variant. PMP is a broad descriptive term embracing a wide spectrum of biological behavior of neoplasms from the benign to the borderline to the frankly malignant lesion. This results in multifocal peritoneal, serosal and ommental implants admixed with copious amounts of mucin accumulation within the abdomen and pelvis resulting in the belly full of jelly – "the jelly belly". The "myxomatous" appearance is attributed to the associated fibroblastic and vascular proliferation that is probably incited by the mucin. Pseudomyxoma peritonei (PMP) first described by Werth is an uncommon and poorly understood disease characterized by abundant extracellular mucin in the peritoneum.
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