Relapse of the pseudomyxoma peritonei after a cytoreductive surgery with peritonectomy and HIPEC, 10-year follow-up - case report with a literature review
Background: Pseudomyxoma peritonei is a rare clinical condition characterized by mucin-secreting epithelial cells which lead to formation of jelly-like structures within the peritoneal cavity and the accumulation of mucinous ascites. Most commonly it arises from the intra-abdominal spread of appendiceal mucinous tumors. Few therapeutic options exist, but a combination of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy, as described by Sugarbaker, stands for the treatment of choice in many tertiary centers nowadays.
Case study: We present a 62-year-old female patient who was initially presented as acute appendicitis. Later pathohistological diagnosis of Pseudomyxoma peritonei just confirmed intraoperative suspicions. Definitive diagnosis was followed with a complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. After a 10 year relapse-free follow up, she presented with colon adenocarcinoma and a recurrence of jelly-like incapsulated structures within the abdominal cavity.
Conclusion: In patients diagnosed with pseudomyxoma peritonei, according to current findings, best results are achieved using complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. However, recurrences still do occur and there is no real consensus regarding their optimal treatment.
Smeenk RM, van Velthuysen ML, Verwaal VJ, Zoetmulder FA. Appendiceal neoplasms and pseudomyxoma peritonei: a population based study. Eur J Surg Oncol 2008;34:196–201.
Mukherjee A, Parvaiz A, Cecil TD, Moran BJ. Pseudomyxoma peritonei usually originates from the appendix: a review of the evidence. Eur J Gynaecol Oncol 2004;25:411–414.
Ferreira CR, Carvalho JP, Soares FA, Siqueira SA, Carvalho FM. Mucinous ovarian tumors associated with pseudomyxoma peritonei of adenomucinosis type: immunohistochemical evidence that they are secondary tumors. Int J Gynecol Cancer 2008;18:59–65.
Moran BJ, Cecil TD. The etiology, clinical presentation, and management of pseudomyxoma peritonei. Surg Oncol Clin N Am 2003;12:585–603.
Sugarbaker PH. Pseudomyxoma peritonei. A cancer whose biology is characterized by a redistribution phenomenon. Ann Surg 1994;219:109–111.
Hinson FL, Ambrose NS. Pseudomyxoma peritonei. Br J Surg 1998;85:1332–1339.
Stewart JH 4th, Shen P, Levine EA. Intraperitoneal hyperthermic chemotherapy for peritoneal surface malignancy: current status and future directions. Ann Surg Oncol 2005;12:765–777.
Miner T.J., Shia J., Jaques D.P., Klimstra D.S., Brennan M.F., Coit D.G. Long-term survival following treatment of pseudomyxoma peritonei: an analysis of surgical therapy. Ann Surg 2005;241:300–308.
Spratt J.S., Adcock R.A., Muskovin M., Sherrill W., McKeown J. Clinical delivery system for intraperitoneal hyperthermic chemotherapy. Cancer Res 1980;40:256–260.
Sugarbaker PH, Kern K, Lack E. Malignant Pseudomyxoma peritonei of colonic origin. Natural history and presentation of a curative approach to treatment. Dis Colon Rectum 1987;30:1772–1779.
Moran B, Baratti D, Yan T, Kusamura S, Deraco M. Consensus statement on the loco-regional treatment of appendiceal mucinous neoplasms with peritoneal dissemination (pseudomyxoma peritonei). J Surg Oncol 2008;98:277–282.
Elias D, Honoré C, Ciuchendéa, R. et al. Peritoneal pseudomyxoma: results of a systematic policy of complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Br J Surg 2008;95:1164–1171.
Sugarbaker PH. Cytoreductive surgery and peri-operative intraperitoneal chemotherapy as a curative approach to pseudomyxoma peritonei syndrome. Eur J Surg Oncol J Eur Soc Surg Oncol Br Assoc Surg Oncol 2001;27:239–243.
Güner Z, Schmidt U, Dahlke MH, Schlitt HJ, Klempnauer J, Piso P. Cytoreductive surgery and intraperitoneal chemotherapy for pseudomyxoma peritonei. Int J Colorectal Dis 2005;20:155–160.
Loungnarath R, Causeret S, Bossard N et al. Cytoreductive surgery with intraperitoneal chemohyperthermia for the treatment of pseudomyxoma peritonei: a prospective study. Dis Colon Rectum 2005;48:1372–1379.
Youssef H, Newman C, Chandrakumaran K, Mohamed F, Cecil TD, Moran BJ. Operative findings, early complications, and long-term survival in 456 patients with pseudomyxoma peritonei syndrome of appendiceal origin. Dis Colon Rectum 2011;54:293–299.
Ahmed S, Stewart JH, Shen P, et al. Outcomes with cytoreductive surgery and HIPEC for peritoneal metastasis. J Surg Oncol 2014;110:575-584.
Chua TC, Moran BJ, Sugarbaker PH, et al. 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. J Clin Oncol 2012;30:2449-2456.
Sugarbaker PH, Ronnett BM, Archer A, et al. Pseudomyxoma peritonei syndrome. Adv Surg 1996;30:233–280.
Delhorme JB, Honoré C, Benhaim L, Dumont F, Dartigues P, Dromain C, et al. Long-term survival after aggressive treatment of relapsed serosal or distant pseudomyxoma peritonei. Eur J Surg Oncol 2017;43:159-167.
Golse N, Bakrin N, Passot G, et al. Iterative procedures combining cytoreductive surgery with hyperthermic intraperitoneal chemotherapy for peritoneal recurrence: postoperative and long-term results. J Surg Oncol 2012;106:197–203.
Smeenk RM, Verwaal VJ, Antonini N, Zoetmulder FAN. Progression of pseudomyxoma peritonei after combined modality treatment: management and outcome. Ann Surg Oncol 2007;14:493–499.
Klaver YLB, Chua TC, Verwaal VJ, de Hingh IHJT, Morris DL. Secondary cytoreductive surgery and peri-operative intraperitoneal chemotherapy for peritoneal recurrence of colorectal and appendiceal peritoneal carcinomatosis following prior primary cytoreduction. J Surg Oncol 2013;107:585–590.
Yan TD, Bijelic L, Sugarbaker PH. Critical analysis of treatment failure after complete cytoreductive surgery and perioperative intraperitoneal chemotherapy for peritoneal dissemination from appendiceal mucinous neoplasms. Ann Surg Oncol 2007;14:2289–2299.
Bijelic L, Yan TD, Sugarbaker PH. Treatment failure following complete cytoreductive surgery and perioperative intraperitoneal chemotherapy for peritoneal dissemination from colorectal or appendiceal mucinous neoplasms. J Surg Oncol 2008;98:295–299.
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