APPENDICEAL PSUDOMIXOMA PERITONEI IN PREGNANCY: a CASE REPORT

Cancer during pregnancy is a rare but challenging event, with an estimated incidence of 1 in 1000. The management of cancer during pregnancy requires a multidisciplinary approach and a balanced concern for maternal survival and fetal health. Pseudomyxoma peritonei (PMP) in 90% of cases begins as a slow-growing tumor in the appendix, which starts producing mucin and can then break through the wall of the appendix, spreading throughout the peritoneum. PMP is extremely rare, with an annual incidence of approximately 1: 1000000. Presently, the gold standard treatment is represented by cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy (HIPEC). This approach has allowed a 5 year overall survival rate of 73% and a disease free survival rate of 56%. A 38 year old woman, with a previous cesarean section for breech presentation, was found, during the routine first trimester ultrasound, to have a complex right ovarian mass, measuring 7 × 5x4 cm, with abnormal vascularization. A diagnostic laparoscopy was planned at 16 weeks + 4 days, as this procedure is nowadays considered safe during pregnancy. Right adnexectomy, peritoneal washing plus biopsy of a retrocecal adhesion were performed. The extemporaneus pathology report revealed a mucinous cystadenoma, the surgeons therefore decided not to perform other surgical actions. Unfortunately, the final pathology report diagnosed ovarian infiltration of a well-differentiated mucinous adenocarcinoma of probable appendiceal origin, with pseudomixoma fields, and a positive washing. A multidisciplinary counseling was made to the patient and her relatives, and they chose to delay definitive treatment after an early delivery, which was planned at 33 weeks + 5 days, after RDS prophylaxis. The patient underwent cesarean section, as she had already received a cesarean section and so it would have been impossible to medically induce labour, and she delivered a male baby of 2540 g. Two months later, the patient underwent laparotomic right hemicolectomy with regional lymphadenectomy, appendicectomy, peritonectomy, omentectomy, hysterectomy, bilateral salpingo-oophorectomy followed by HIPEC with cisplatin and mitomycin C at 41.5 °c over 60 minutes. A complete cancer resection was achieved, with no macroscopic residual tumor. The final pathology report diagnosed a well differentiated adenocarcinoma of the appendix, stage pT4apN1. The patient was then treated with 8 cycles of 5-flouorouracil, leucovorin and oxaliplatin. At the 2 years follow up, both the patient and her child are in good condition, and she is disease-free at the last instrumental examination.