From the Guidelines
Appendectomy is the initial treatment of choice for an appendiceal mucinous neoplasm, with right hemicolectomy considered for tumors larger than 2 cm or those with serosal breach, cellular atypia, or invasion. The management of appendiceal mucinous neoplasms depends on the size and characteristics of the tumor. According to the guidelines for the management of gastroenteropancreatic neuroendocrine tumors 1, classical neuroendocrine tumors (NETs) less than 1 cm in size can usually be managed by appendicectomy, while for tumors greater than 2 cm in size or for goblet cell tumors of any size, right hemicolectomy is the treatment of choice.
For tumors between 1-2 cm in size, any of the following mandates right hemicolectomy: serosal breach by tumor, cellular atypia, or invasion. It is essential to note that the evidence from the study on ovarian cancer 1 is not directly applicable to the management of appendiceal mucinous neoplasms, as it focuses on mucinous carcinomas of the ovary.
Key considerations in the management of appendiceal mucinous neoplasms include:
- Tumor size and classification
- Presence of serosal breach, cellular atypia, or invasion
- Complete surgical removal to prevent rupture and spread of mucin into the peritoneal cavity
- Regular follow-up with imaging studies to monitor for recurrence
- Prognosis varies significantly based on tumor grade and extent of spread.
In clinical practice, the primary goal is to prioritize morbidity, mortality, and quality of life, and thus, prompt surgical intervention with appendectomy and consideration of right hemicolectomy for more advanced cases is recommended 1.
From the Research
Diagnosis and Treatment of Appendiceal Mucinous Neoplasms
When a patient has an appendiceal mucinous neoplasm, several key points are indicated:
- The disease is rare and heterogeneous, with a rising incidence 2
- Treatment is based on stage and histology, with low-grade tumors typically treated surgically and high-grade tumors requiring more aggressive treatment, including debulking surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) 2, 3
- The disease can present atypically, making diagnosis challenging, and may involve the spread of mucinous tumor throughout the peritoneum 3, 4
- Imaging features, including the presence of extra-appendiceal mucin, are important for diagnosis and treatment planning 5
- A comprehensive approach to diagnosis and treatment is necessary, involving a multidisciplinary team of healthcare professionals, including gastroenterologists, pathologists, surgeons, and oncologists 2, 6
Key Considerations
Some key considerations when dealing with appendiceal mucinous neoplasms include:
- The importance of individualized treatment based on patient-specific parameters, such as comorbidities and performance status 3
- The need for aggressive cytoreductive surgery and intraperitoneal chemotherapy in cases of disseminated disease 3
- The role of systemic chemotherapy and targeted therapies in the treatment of appendiceal mucinous neoplasms 3, 2
- The importance of nurse education and patient care in the management of these complex patients 4