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Culture and Imaging of Ex Vivo Organotypic Pseudomyxoma Peritonei Tumor Slices from Resected Human Tumor Specimens
Published on: December 9, 2022
Pseudomyxoma Peritonei Arising from Urachal Mucinous Neoplasms: a Case Series and Updated Literature Review
Niccolo Allievi1, Vasanth Mark Samuel1, Norman Carr1
1Peritoneal Malignancy Insitute, Hampshire Hospitals NHS Foundation Trust, Basingstoke, UK.
Abstract:
To report a case series of patients with pseudomyxoma peritonei (PMP) from urachal mucinous neoplasm (UMN) treated with CRS and HIPEC at a high-volume referral centre, along with an updated literature review. Retrospective review of cases treated between 2000 and 2021. A literature review using MEDLINE and Google Scholar databases was performed. Clinical presentation of PMP from UMN is heterogeneous, and common symptoms are abdominal distension, weight loss, fatigue and haematuria. At least one tumour marker among CEA, CA 19.9, and CA 125 was elevated in the six cases reported, and 5/6 had a preoperative working diagnosis of urachal mucinous neoplasm suspected on detailed cross-sectional imaging. Complete cytoreduction was achieved in five cases, while one patient underwent maximal tumour debulking. Histological findings mirrored the findings of PMP from appendiceal mucinous neoplasms (AMN). Overall survival ranged between 43 and 141 months after complete cytoreduction. On literature review, 76 cases have been reported to date. Complete cytoreduction is associated with good prognosis for patients with PMP from UMN. A definitive classification system is still not available.
Supplementary Information:
The online version contains supplementary material available at 10.1007/s13193-022-01694-5.
Insights
Pseudomyxoma peritonei (PMP) from urachal mucinous neoplasm (UMN) is rare but treatable. Complete cytoreduction surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) offers good prognosis for PMP patients.
Area of Science:
- Oncology
- Surgical Oncology
- Gastroenterology
Background:
- Pseudomyxoma peritonei (PMP) is a rare malignancy.
- Urachal mucinous neoplasm (UMN) is an uncommon cause of PMP.
- Treatment outcomes for PMP from UMN are not well-established.
Purpose of the Study:
- To report a case series of PMP from UMN treated with CRS and HIPEC.
- To review the current literature on PMP from UMN.
- To evaluate the efficacy and prognosis of CRS and HIPEC for UMN-derived PMP.
Main Methods:
- Retrospective review of patients treated between 2000-2021.
- Literature search using MEDLINE and Google Scholar.
- Analysis of clinical presentation, imaging, tumor markers, surgical outcomes, and survival data.
Main Results:
- Six cases of PMP from UMN were identified.
- Common symptoms included abdominal distension, weight loss, fatigue, and hematuria.
- Five patients achieved complete cytoreduction with CRS and HIPEC, with survival ranging from 43-141 months.
- Histological findings were similar to PMP from appendiceal mucinous neoplasms.
Conclusions:
- PMP from UMN is a rare but treatable condition.
- Complete cytoreduction via CRS and HIPEC is associated with a favorable prognosis.
- Further research is needed for a definitive classification system.
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