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Appendiceal neoplasm

Definition
Appendiceal neoplasms are abnormal growths that occur in the appendix. They encompass a range of tumors, including low-grade appendiceal mucinous neoplasm, high-grade appendiceal mucinous neoplasm, and mucinous adenocarcinoma.
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Pathophysiology
The pathophysiology of appendiceal neoplasms is not fully understood, but it is believed to involve genetic mutations that lead to the uncontrolled growth and division of cells in the appendix.
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Epidemiology
The incidence of appendiceal neoplasms in the US is estimated at 0.93 per 100,000 person-years from 2000 to 2017.
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Disease course
Clinically, appendiceal neoplasms often present with non-specific symptoms such as abdominal pain, changes in bowel habits, and weight loss. In some cases, they may be discovered incidentally during imaging or surgery for other conditions.
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Prognosis and risk of recurrence
The prognosis of appendiceal neoplasms can vary widely and is influenced by factors such as the stage of the disease at diagnosis and the specific type of neoplasm.
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Key sources
The following summarized guidelines for the evaluation and management of appendiceal neoplasm are prepared by our editorial team based on guidelines from the National Comprehensive Cancer Network (NCCN 2021) and the American Society of Colon and Rectal Surgeons (ASCRS 2019).
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Guidelines

1.Diagnostic investigations

Clinical examination: elicit a complete history and perform physical examination in patients with appendiceal neoplasms.
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  • Diagnostic imaging

  • Laboratory tests

  • Preoperative assessment

2.Diagnostic procedures

Colonoscopy: perform colonoscopy in patients with confirmed or suspected appendiceal neoplasms.
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  • Peritoneal cytology

3.Medical management

Management of advanced/metastatic disease: as per NCCN 2021 guidelines, observe with imaging or offer octreotide or lanreotide in asymptomatic patients with locoregionally advanced or metastatic appendiceal NETs with low tumor burden.
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4.Therapeutic procedures

Hyperthermic intraperitoneal chemotherapy: consider administering intraperitoneal chemotherapy to reduce peritoneal disease recurrence in selected patients with appendiceal epithelial neoplasms.
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5.Surgical interventions

Indications for surgery: as per NCCN 2021 guidelines, perform simple appendectomy in patients with appendiceal NETs ≤ 2 cm.
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6.Follow-up and surveillance

Follow-up assessment: as per NCCN 2021 guidelines, obtain follow-up assessment in patients with NETs after resection:
Situation
Guidance
12 weeks to 12 months
History and physical examination
Biochemical markers (24-hour urine or serum 5-HIAA, testing for Cushing's syndrome) as clinically indicated
Abdominal +/- pelvic multiphase CT or MRI as clinically indicated
1-10 years (every 1-2 years)
History and physical examination
Biochemical markers (24-hour urine or serum 5-HIAA, testing for Cushing's syndrome) as clinically indicated
Abdominal +/- pelvic multiphase CT or MRI
> 10 years
Obtain surveillance as clinically indicated
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