Objective
To discuss a case of appendiceal cancer presenting as ovarian cyst rupture.
Case Report
A 31 year old nulliparous female was referred by her GP with a one day history of severe lower abdominal pain and profuse vomiting. A formal Pelvic USS showed an 8.5cm left ovarian cyst and large amount of particulate free fluid in the pelvis thought to be a ruptured ovarian cyst. She underwent an emergency diagnostic laparoscopy which revealed large straw coloured fluid in the abdomen, two large green gelatinous masses over the appendix and the right ovary which was also torted; There was also extensive mucinous deposits over the parietal peritoneum, the abdominal wall and the right diaphragm. An appendicectomy and detorsion of the ovary was performed while the mucinous material was left in situ. Tumour markers returned as normal and histo-cytology showed a low grade appendiceal mucinous neoplasm and pseudomyxoma peritonei. (PMP). A staging CT showed ascites in the abdomen and pelvis with no pulmonary metastasis. The patient has recently undergone cytoreductive surgery(CRS) which includes radical hysterectomy, oophorectomy, right hemicolectomy and anterior resection of the rectum, splenectomy, diaphragm excision, cholecystectomy as well as Hyperthermic Intraperitoneal Chemotherapy(HIPEC). The patient was admitted to intensive care unit for two days and was subsequently discharged with appropriate vaccinations and prophylactic antibiotics, follow up from stoma care nurse and endocrinologist for commencement of hormonal replacement therapy.
Discussion
Pseudomyxoma Peritonei (PMP) is a clinical syndrome secondary to intraperitoneal accumulation of a gelatinous ascites from rupture of a mucinous tumour, most commonly appendiceal cancer. The incidence is approximately 2 in 10,000 laparotomies and the aetiology remains unknown. Patients affected are most commonly asymptomatic. Symptoms when present include non-tender increasing abdominal girth, palpation of ovarian mass during an internal examination and intestinal obstruction which is a late sign. CT may show heterogenous material with scalloping of the liver, spleen and mesentery; thickened undersurface of the diaphragm, and relative sparing and central displacement of the small bowel and mesentery. Prognosis depends on tumour biology, stage of disease and response to treatment with a median survival after surgery of 5.9-6.25 years. Swanson et al 2016 reports improved survival with HIPEC in addition to CRS to 59-81% at 5 years, 49-70% at ten years.
Recommendations/Conclusions
The general gynaecologists should be prepared for unusual findings at diagnostic laparoscopies for abdominal pain and involve relevant specialties as needed.