Massive mucinous neoplasm of the appendix

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An 84‐year‐old woman was referred to our centre with a 12‐month history of progressive abdominal distension, constipation, early satiety and intermittent vomiting. Twenty‐two years earlier, she had undergone total abdominal hysterectomy with bilateral salpingo‐oophorectomy for mucinous tumour of the ovaries. At the time, the possibility of pseudomyxoma peritonei was raised. She was well subsequently and required no further intervention until her recent presentation.
On examination, she had a grossly distended abdomen. Computed tomography scan demonstrated a massive multiloculated swelling filling the entire abdominopelvic cavity and the features were felt to be in keep with recurrent pseudomyxoma peritonei.
Following multidisciplinary discussion, it was decided to proceed to surgery with a view to cytoreduction and intraoperative chemotherapy (HIPEC) in order to achieve symptom control.
At laparotomy, the dominant finding was a massive tumour arising from the caecum, fixed to the abdominal wall and retroperitoneum via dense adhesions (Fig. 1). The transverse colon and terminal ileum were fixed to the surface of the tumour while there was secondary mucinous ascites in the pelvis and paracolic gutters. The tumour was mobilized and resected en‐bloc with the colon and omentum (Fig. 1). There were no peritoneal deposits; however, in view of the signs of peritoneal mucin dissemination, HIPEC with mitomycin C was given as planned preoperatively.
The patient made an uneventful recovery and was discharged home well 12 days later. At subsequent review, 3 months later, she had made a full recovery and was asymptomatic.
Histopathological analysis showed a low‐grade appendiceal mucinous neoplasm, 29 cm in dimension, with acellular mucin present on the attached mesocolon.
Pseudomyxoma peritonei is a rare condition, characterized by copious mucinous ascites and peritoneal deposits. It is usually secondary to rupture of a mucinous tumour of the appendix.1 Patients often present with disseminated disease many years following initial appendicectomy.2 In the current case, the reverse occurred. Following initial treatment for features of disseminated disease likely from an unrecognized appendix tumour, our patient presented many years later with symptoms due to the primary tumour itself and was successfully treated with a combination of surgery and HIPEC.
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