Abstract
Pseudomyxoma peritonei (PMP) is a rare clinical condition, where copious mucinous ascites accumulate in the peritoneal cavity due to dissemination of mucin-producing tumor. Because of this disseminating, yet nonmetastasizing, behavior, PMP attracts much interest from surgical oncologists in that aggressive locoregional therapy can give the opportunity of long survival and even cure. Although extra-abdominal metastasis is exceptionally rare, the lung is the most likely site in such a case. In this paper, the clinical findings and treatment of eleven cases with pulmonary metastasis from PMP were reviewed, including ten cases in the literature and one case which we experienced. The clinical features of PMP cases with pulmonary metastasis were similar to cases without pulmonary metastasis. The histological type was low-grade mucinous neoplasm in most cases. Pulmonary lesions were resected in seven cases in which abdominal lesions were controlled by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy or another therapeutic modality. Disease-free state was maintained in five cases at the end of the follow-up period. However, it should be noted that rapid progression after resection was seen in two cases, suggesting that biological features may have changed by surgical intervention.
Highlights
Pseudomyxoma peritonei (PMP) is a rare clinical condition, where copious mucinous ascites accumulate in the peritoneal cavity due to dissemination of mucin-producing tumor [1]
No biological study was done in previous case reports, the histological types of PMP and pulmonary metastasis were classified as DPAM and low-grade mucinous neoplasm in all cases
As to the two cases which showed rapid progression after Cytoreductive surgery (CRS) and pulmonary resection, it was highly suspected that inflammatory reactions caused by surgical stress and other factors may have changed biological features which cannot be determined by the histological type
Summary
Pseudomyxoma peritonei (PMP) is a rare clinical condition, where copious mucinous ascites accumulate in the peritoneal cavity due to dissemination of mucin-producing tumor [1] It is initiated by perforation of low-grade mucinous appendiceal neoplasm in most cases. Histological findings of the lung nodule were similar with those of appendiceal tumor, showing that low-grade mucinous neoplasm invaded pulmonary. CT examination two months after CRS showed multiple lung nodules, and they progressed rapidly (Figure 3). She underwent laparotomy for intestinal obstruction caused by diffuse abdominal recurrence five months later.
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