[Pseudomyxoma Peritonei Caused by Intraductal Papillary Mucinous Neoplasm-A Three Case Series]
Toru Imagami1, Atsushi Mitsunaka, Nobuyuki Takao
1Dept. of Digestive Surgery and Peritoneal Dissemination Center, Omi Medical Center.
Abstract:
Pseudomyxoma peritonei(PMP)is a rare condition caused by intraductal papillary mucinous neoplasm(IPMN). At our institution, 3 cases, along with literature review, of PMP caused by IPMN were treated with cytoreductive surgery. In case 1, a 70-year-old man was pathologically diagnosed with low-grade PMP with intraductal papillary mucinous carcinoma. Recurrence was discovered 68 months after surgery, and the patient died after 78 months. In case 2, a 69-year-old man was pathologically diagnosed with high-grade PMP with signet-ring cell carcinoma caused by intraductal papillary mucinous carcinoma. The patient died 8 months post-surgery. In case 3, a 77-year-old woman was pathologically diagnosed with low- grade(partially high-grade)PMP with intraductal papillary mucinous adenoma. There was a recurrence 14 months post- surgery. The patient is still alive because of systemic chemotherapy 32 months after surgery.
Insights
Pseudomyxoma peritonei (PMP), a rare condition linked to intraductal papillary mucinous neoplasms (IPMN), was treated with surgery in three cases. Outcomes varied, with one patient surviving long-term with chemotherapy after recurrence.
Area of Science:
- Gastroenterology
- Surgical Oncology
- Oncology
Background:
- Pseudomyxoma peritonei (PMP) is a rare clinical entity.
- Intraductal papillary mucinous neoplasms (IPMN) are a known cause of PMP.
- Cytoreductive surgery is a treatment option for PMP.
Observation:
- Three cases of PMP originating from IPMN were treated with cytoreductive surgery.
- Case 1: Low-grade PMP with intraductal papillary mucinous carcinoma; recurrence at 68 months, death at 78 months.
- Case 2: High-grade PMP with signet-ring cell carcinoma; death at 8 months.
- Case 3: Low-grade (partially high-grade) PMP with intraductal papillary mucinous adenoma; recurrence at 14 months, alive at 32 months with chemotherapy.
Findings:
- Treatment outcomes for PMP secondary to IPMN vary significantly.
- Cytoreductive surgery offers a chance for long-term survival, as demonstrated in Case 3.
- Recurrence is a significant concern, even after successful initial surgery.
Implications:
- Further research is needed to optimize treatment strategies for PMP from IPMN.
- Systemic chemotherapy may play a crucial role in managing recurrent or advanced disease.
- Early diagnosis and surgical intervention are critical for improving patient prognosis.
