Mucinous appendiceal neoplasms with or without pseudomyxoma peritonei: a review

Abstract

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.

Appendiceal Neoplasms: Diagnosis, Management and Follow-up

“…A high dose of heated chemotherapeutic agents is perfused intraoperatively throughout the abdomen to eradicate any residual microscopic cancer cells. Therefore, when a complete or near complete cytoreduction is obtained (when all tumour nodules bigger than 0.25 cm have been removed), HIPEC is added to treat microscopic residual tumour cells [44].…”

Multidisciplinary management of rare cancers: Recurrent neuroendocrine carcinoma of the appendix

“…For mucinous adenocarcinoma and neuroendocrine tumours confined to the appendix, right hemicolectomy is recommended in the first instance 1,2 . When margins are involved or when there is tumour perforation and peritoneal spread, referral to a peritoneal surface malignancy centre is recommended 3 . Recently, the role of cytoreductive surgery (CRS) and heated intraperitoneal chemotherapy (HIPEC) in non‐mucinous neoplasms—including mixed adenoneuroendocrine carcinomas—has been examined with findings showing that while mucinous and non‐mucinous appendiceal tumours share a locoregional dissemination pattern, prognosis is worse following CRS and HIPEC for non‐mucinous neoplasms 4 .…”

“…1,2 When margins are involved or when there is tumour perforation and peritoneal spread, referral to a peritoneal surface malignancy centre is recommended. 3 Recently, the role of cytoreductive surgery (CRS) and heated intraperitoneal chemotherapy (HIPEC) in non-mucinous neoplasms-including mixed adenoneuroendocrine carcinomas-has been examined with findings showing that while mucinous and non-mucinous appendiceal tumours share a locoregional dissemination pattern, prognosis is worse following CRS and HIPEC for nonmucinous neoplasms. 4 In light of these recommendations, the management of patients with rare tumours relies on expert opinion so that decisions made are patient appropriate.…”

Oral administration of bromelain and acetylcysteine in pseudomyxoma peritonei (PMP) caused by low-grade appendiceal mucinous neoplasm (LAMN): a case report

“…Mucinous neoplasms of the appendix such as low-grade appendiceal mucinous neoplasm (LAMN) are the primary cause of PMP. If LAMN leads to rupture of the appendix, it may be followed by PMP with diffuse peritoneal dissemination [ 1 ].…”