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Pseudomyxoma Perito...
Pseudomyxoma Peritonei : Aspects of Natural History, Learning Curve, Treatment Outcome and Prognostic Factors
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- Andréasson, Håkan (author)
- Uppsala universitet,Kolorektalkirurgi
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- Mahteme, Haile, Docent (thesis advisor)
- Uppsala universitet,Kolorektalkirurgi
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- Påhlman, Lars, Professor (thesis advisor)
- Uppsala universitet,Kolorektalkirurgi
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- Moran, Brendan, Professor (opponent)
- Pelican Cancer Foundation, Basingstoke Colorectal, The Hampshire Clinic Basing Road
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(creator_code:org_t)
- ISBN 9789155486518
- Uppsala : Acta Universitatis Upsaliensis, 2013
- English 76 s.
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Series: Digital Comprehensive Summaries of Uppsala Dissertations from the Faculty of Medicine, 1651-6206 ; 896
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Abstract
Subject headings
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- Pseudomyxoma peritonei (PMP) is a rare disease characterized by mucinous peritoneal metastasis (PM). Different loco-regional treatment strategies, i.e. debulking surgery and cytoreductive surgery (CRS) in combination with hyperthermic intraperitoneal chemotherapy (HIPEC), have changed the prognosis for these patients. CRS is an aggressive surgical procedure with a long learning curve. PMP exists in different types; how many depends on which classification is used.The aims of this thesis were to investigate the time-frame of PMP development from an isolated appendiceal neoplasm, examine the learning process for CRS, evaluate the differences in treatment outcome between debulking surgery and CRS in combination with HIPEC, to evaluate a more detailed PMP classification and to investigate particularly interesting new cysteine-histidine (PINCH) protein as a prognostic factor for PMP.Retrospectively 26 PMP patients were identified as having had an appendectomy with a neoplasm in the appendix but with no evidence of PM at the appendectomy. They were treated for PMP within a median of 13.1 months (3.8-95.3) after the appendectomy. No difference was seen between the types of PMP regarding the time to a clinically significant development of PMP and how much tumour was found at treatment. CRS is a highly invasive treatment and stabilization in the learning curve was seen after 220±10 procedures. Patients treated with CRS+HIPEC had a better 5-year overall survival (OS) than patients treated with debulking surgery, 74% vs. 40%. CRS increased the rate of complete cytoreduction from 25% in patients treated with debulking surgery to 72%. The new four-grade PMP classification showed very good inter-rater agreement between two independent pathologists and a difference in survival rates was observed between the different grades. A positive PINCH staining was recorded in 83% of the tumours and that was associated with poorer survival.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Kirurgi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Surgery (hsv//eng)
Keyword
- Pseudomyxoma peritonei
- Natural history
- Learning curve
- Cytoreductive surgery
- Debulking surgery
- Treatment outcome
- Prognostic factors
- PINCH
Publication and Content Type
- vet (subject category)
- dok (subject category)
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