Appendix Cancer (including Carcinoid Tumors)
Overview
Appendix cancer is a rare form of gastrointestinal cancer that begins in the appendix, a small pouch attached to the colon. It includes a variety of tumor types, ranging from slow-growing carcinoid tumors to aggressive adenocarcinomas.
Types include:
- Carcinoid tumors (neuroendocrine tumors)
- Appendiceal adenocarcinoma
- Mucinous neoplasms (can lead to pseudomyxoma peritonei)
- Signet ring cell carcinoma (rare and aggressive)
Symptoms
- Abdominal pain (often resembling appendicitis)
- Bloating or increase in abdominal girth
- Changes in bowel habits
- Ascites (fluid in abdomen)
- In some cases, asymptomatic and discovered incidentally
Causes & Risk Factors
- Often unknown
- Genetic syndromes (e.g., MEN1, Lynch syndrome)
- Age >40
- More common in females for some tumor types (e.g., mucinous neoplasms)
Diagnosis
- CT scan or ultrasound (often after acute appendicitis)
- Colonoscopy (may detect synchronous colon lesions)
- Blood tests: tumor markers (CEA, CA 19-9, chromogranin A)
- Surgical pathology after appendectomy
Treatment Options
- Surgery: appendectomy, right hemicolectomy, or cytoreductive surgery
- HIPEC (hyperthermic intraperitoneal chemotherapy) for peritoneal spread
- Systemic chemotherapy (for adenocarcinoma or high-grade tumors)
- Targeted therapy or somatostatin analogs (for NETs)
Prognosis
- Varies by type and stage
- Carcinoid tumors: excellent prognosis
- Mucinous and adenocarcinoma types: intermediate to poor, depending on spread
Living with this Cancer Type
- Long-term monitoring with imaging and markers
- Nutritional support (especially after HIPEC)
- Management of carcinoid syndrome if present
- Emotional and peer support groups
Prevention & Screening
- No routine screening
- Genetic counseling for high-risk families
- Surveillance for patients with syndromes like MEN1 or Lynch
FAQs
Q: How is appendix cancer found?
A: Often during or after surgery for appendicitis or imaging for abdominal issues.
Q: Is it the same as colon cancer?
A: No, it is biologically and clinically distinct, though both involve the lower GI tract.
Q: What is pseudomyxoma peritonei?
A: A condition where mucinous tumor cells spread into the abdominal cavity and produce jelly-like fluid.
Resources
- Appendix Cancer Pseudomyxoma Peritonei Research Foundation (ACPMP)
- American Society of Clinical Oncology
- ClinicalTrials.gov
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