The cut surface of the appendix revealed mucinous material. The intervening fibroblastic stroma showed dissecting pools of mucin (Fig. There was no evidence of atypia or invasion. Histopathology of the left ovarian mass revealed a mucinous tumor with borderline features composed of multiple mucin filled cysts lined by multilayered mucinous epithelium. The samples were sent for histopathological examination. Total abdominal hysterectomy with bilateral salpingo-oophorectomy, omentectomy, retro peritoneal lymph node dissection was done along with appendectomy. Intraoperatively, the peritoneal cavity was filled with gelatinous material, a large mucinous tumor (with ruptured capsule) replacing the left ovary and an enlarged appendix. Investigations revealed an elevated CEA (923 ng/ml) and mildly elevated CA 125 (51 U/ml). On ultrasonography, a large, multiloculated cystic lesion measuring 15 × 12 × 16 cm was noted in the pelvis. Ī 75-year female presented with abdominal distension and loss of appetite for 2 months. The treatment options range from cytoreductive surgery to hyperthermic intraperitoneal chemotherapy (HIPEC) with varied prognosis. This can be further classified as invasive, low grade or high grade, based on the WHO AJCC 2010 classification. According to the current AJCC and WHO classification, any neoplastic mucinous epithelium in the appendiceal wall and beyond is a mucinous adenocarcinoma. It was classified into disseminated peritoneal adenomucinosis (DPAM) and peritoneal mucinous carcinomatosis (PMCA) based on cellularity, epithelial atypia and mitotic activity which presented much subjective ambiguity. The pathological diagnosis and classification of PMP has posed much debate. The ovaries usually represent secondary involvement. It may also originate from other mucinous neoplasms throughout the gastrointestinal tract. The primary neoplasms producing PMP may be benign or malignant and their origin has been debated, until recent studies have attributed it to primary appendiceal mucinous neoplasms. PMP is a clinical terminology rather than a pathological diagnosis. Ronnett BM, Shmookler BM, Sugarbaker PH et al (1997) Pseudomyxoma peritonei: new concepts in diagnosis, origin, nomenclature, and relationship to mucinous borderline (low malignant potential) tumors of the ovary.Pseudomyxoma peritonei (PMP) is an enigmatic intra-abdominal disease characterized by epithelial implants secreting extracellular mucin resulting in dissecting gelatinous ascites. Roberts DL, O’Dwyer ST, Stern PL, Renehan AG (2015) Global gene expression in pseudomyxoma peritonei, with parallel development of two immortalized cell lines. Journal of clinical oncology: official journal of the American Society of Clinical Oncology 30(20):2449–56 Am J Surg Pathol 40(1):14–26Ĭhua TC, Moran BJ, Sugarbaker PH, Levine EA, Glehen O, Gilly FN, et al (2012) Early- and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. (2016) A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia: The Results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process. Am J Surg Pathol 30:551–559Ĭarr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, Gonzalez-Moreno S, et al. Br J Cancer 95:1258–1264īradley RF, Stewart JH, Russell GB et al (2006) Pseudomyxoma peritonei of appendiceal origin: a clinicopathologic analysis of 101 patients uniformly treated at a single institution, with literature review. Societa Italiana di Terapie Integrate Locoregionali in Oncologia (SITILO), Milan ( )īibi R, Pranesh N, Saunders MP et al (2006) A specific cadherin phenotype may characterise the disseminating yet non-metastatic behaviour of pseudomyxoma peritonei. 5th International Workshop on Peritoneal Surface Malignancy (2006) Peritoneal Surface Oncology Group International (PSOGI).
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