Pseudomyxoma Peritonei.

BACKGROUND: Pseudomyxoma peritonei is a rare tumorous disease with various grades of malignancy and is characterized by production of mucinous and gelatinous masses. Development of pseudomyxoma peritonei is usually associated with rupture of appendiceal mucinous tumors and other mucinous tumors of the gastrointestinal tract or ovaries. Pseudomyxoma peritonei is usually divided into three types: low-grade, high-grade, and high-grade with signet ring cells. Staging of the disease is determined by the peritoneal cancer index. Clinical findings are highly variable depending on disease staging. The typical finding of “jelly belly” syndrome worsens with disease progression. The diagnosis is based on the pre-operative cure by imaging methods, especially computed tomography. METHODS: The Sugarbaker method involves maximal removal of tumorous masses, so-called cytoreductive surgery, and perioperative intraabdominal application of hot cytostatics (hyperthermic intraperitoneal chemotherapy) with the aim of achieving maximal liquidation of tumorous processes. RESULTS: Our results are comparable with previous published data and confirm high effectivness of this method. The results show statistically very significant extention of overall survival, disease free interval with acceptable lethality 0-12 % and morbidity 27-56%. These results promote this method as the gold standard of treatment of pseudomyxoma peritonei in selected patients.

Malignant Peritoneal Tumors - Introduction.

This article provides an introduction to peritoneal tumors, which are the subject of a series of review papers published in Issue 5 (2019) of Klinicka onkologie. Many malignant peritoneal tumors are characterized by production of mucinous and gelatinous masses, multiple peritoneal disability, so-called peritoneal carcinomatosis, and various grades of malignancy depending on their origin, staging, and histological type. Malignant peritoneal tumors are rare and their clinical symptomatology is nonspecific and varies according to the extent of disability. Diagnosis, particularly in the initial asymptomatic stages, is very complicated and often impossible, and tumors are often diagnosed by chance during other operations. Malignant peritoneal tumors were regarded as incurable and lethal for a long time; however, this view has changed over the past three decades. The Sugarbaker method, a combination of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy, was introduced in the 1990s. Postoperative cytostatic lavage is usually performed in specific cases. Classifications for the extent of disease and completeness of cytoreduction were established. Studies repeatedly confirmed the efficacy of this treatment for peritoneal malignancy. The combination of an aggressive surgical approach and intraperitoneal chemotherapy not only enhances quality of life, but also prolongs progression-free survival and overall survival in selected patients. Specialized centers for treatment of peritoneal malignancy were established based on results from the Czech Republic and around the world. These centers provide complex care, including specific surgical interventions and follow-up, for selected patients with primary and secondary peritoneal malignancy.