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An Invasive Treatment of Pseudomyxoma Peritonei with Intrathoracic Involvement
Rami Ababneh1, Pompiliu Piso2, Hans-Stefan Hofmann2
1"Prof. Dr. Ion Chiricuta" Institute of Oncology, Cluj-Napoca, Romania.
Abstract:
Pseudomyxoma peritonei (PP) is a rare disease characterized by the presence of mucinous ascites and low and progressive accumulation of peritoneal implants. We report the case of a 44-year-old man presented with ascites, imaging evaluations suggesting the diagnosis of gelatinous peritoneal carcinomatosis. The patient underwent laparoscopy with extensive cytoreductive surgery combined with hyperthermic intraoperative peritoneal chemotherapy (HIPEC). Microscopic features confirmed the diagnosis of low-grade PP. Nine months later the patients developed left pleural effusion and cytological examination releaved the intrathoracic extension of PP. The treatment consisted in extensive intrathoracic cytoreductive surgery in combination with hyperthermic intrathoracic chemotherapy perfusion (HITOC). Further surgery was requiered due to intra-abdominal recurrence and finally, the patient developed hepatic and pulmonary metastases treated by systemic chemotherapy, with good tolerability and complete response.
Insights
Pseudomyxoma peritonei (PP) is a rare condition causing mucinous ascites. This case highlights successful multimodal treatment including cytoreductive surgery and hyperthermic chemotherapy for advanced PP with intrathoracic and metastatic spread.
Area of Science:
- Oncology
- Surgical Oncology
- Gastroenterology
Background:
- Pseudomyxoma peritonei (PP) is a rare malignancy characterized by mucinous ascites and peritoneal implants.
- It often presents insidiously with symptoms related to abdominal distension and discomfort.
Purpose of the Study:
- To report a case of advanced pseudomyxoma peritonei with intrathoracic and metastatic spread.
- To illustrate the multimodal treatment approach including cytoreductive surgery and hyperthermic chemotherapy.
Main Methods:
- Laparoscopic cytoreductive surgery with hyperthermic intraoperative peritoneal chemotherapy (HIPEC).
- Intrathoracic cytoreductive surgery with hyperthermic intrathoracic chemotherapy perfusion (HITOC) for pleural extension.
- Systemic chemotherapy for hepatic and pulmonary metastases.
Main Results:
- Initial diagnosis of low-grade PP confirmed post-surgery.
- Successful treatment of intrathoracic extension with HITOC.
- Complete response to systemic chemotherapy for distant metastases.
Conclusions:
- Pseudomyxoma peritonei can present with extensive intra-abdominal, intrathoracic, and distant metastatic disease.
- Aggressive cytoreductive surgery combined with HIPEC and HITOC, followed by systemic chemotherapy, can achieve a complete response in advanced PP.
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