Joshua D. Lansom

Mucinous appendiceal neoplasms (MANs) are rare tumours and the primary cause of pseudomyxoma peritonei. These tumours have a much more benign course than typical colorectal cancers, generally growing for many years before giving any clinical signs. The spectrum of presentations, tumour stages and the underlying cytology is very wide, warranting from the simplest operation like an appendicectomy to the most complicated operation like a complete cytoreduction surgery and hyperthermic intraperitoneal chemotherapy. Fortunately, most patients can be offered a curative treatment, but limiting operative morbidity without compromising oncologic outcomes is the biggest challenge in managing these patients. Histopathology is the cornerstone of decision making for MANs, but is also subject to ongoing debate because of a lack of terminology consensus amongst pathologists. Combined with the rarity of this disease, the multiple histopathologic classification updates of MANs explain the ongoing confusion amongst clinicians in regard to individual optimal treatment. This review will cover the most recent histological classification of MANs and attempt to clarify optimal management of patients with different clinical presentation and histologic combinations.

Pseudomyxoma peritonei (PMP) is the intra-peritoneal accumulation of mucus due to mucinous neoplasia, most often from a ruptured mucinous appendiceal neoplasm. A similar syndrome is caused by appendix cancer and other gastrointestinal malignancies. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) provides long-term survival in selected patients with these conditions. The management of the appendiceal neoplasm prior to development of peritoneal involvement is initially discussed. This is followed by an overview of the management of peritoneal disease caused by appendiceal neoplasms. The principles and basic techniques of CRS and intraperitoneal chemotherapy (both intraoperative and post operative) are then discussed. Survival outcomes from several large studies are summarised. Prognostic factors are also discussed. We report our basic outcome data for the 345 patients with PMP or appendix cancer treated at our institution. Finally, the promising upcoming treatment of mucolytic therapy is discussed. We conclude that appendiceal neoplasms, although rare can cause significant morbidity and mortality. With optimal management long-term survival is possible in the majority of patients. The key to treatment is complete cytoreduction and use of hyperthermic intraperitoneal chemotherapy.

Background: This study aimed to (i) investigate the factors that influence donor and recipient decision making in adult‐to‐adult living donor liver transplantation (AALDLT); (ii) quantify the level of risk that would be acceptable to potential donors; and (iii) determine from whom an individual would be willing to receive a donation.Methods: A self‐administered questionnaire using hypothetical scenarios centred on AALDLT was created and administered to participants recruited from the waiting room of an orthopaedic outpatient clinic at a teaching hospital in Sydney (n = 105). The questionnaire asked participants to consider scenarios in which they either (i) were a potential donor for a family member or close friend or (ii) themselves required a liver transplant.Results: Ninety‐five (90%) participants expressed an in‐principal willingness to consider living organ donation. The factors most important in deciding to be living liver donors were the probability of a good outcome for the recipient, the likelihood of the potential recipient's survival until a deceased donor liver became available and the risk of donor death. Donor death was also rated as the least acceptable donor outcome. Participants expressed a willingness to receive a donation from all proposed donor groups equally.Conclusions: The acceptability of hypothetical living organ donation was very high in the population group studied. Participants were also willing to accept significantly higher risks of complications from organ donation than they would actually be exposed to. Clinicians should feel encouraged to discuss the risks and benefits of living donation frankly with patients and their families.