Appendiceal cancer

Disease Description (Causes and Symptoms)

• Definition: Appendiceal carcinoma is a malignant tumor that develops in the appendix (the pouch attached to the end of the cecum). (Synonyms: cecal cancer, appendiceal cancer)
• Occurrence pattern (for reference): It is known to occur more commonly in women in their 20s–30s, and in many cases grows slowly and metastasizes relatively infrequently.
• Causes and pathophysiology:
  • Reported standard risk factors include chronic inflammation of the appendiceal mucosa, obstruction of the appendiceal lumen (stasis of contents), and inflammation of lymphoid tissue.
  • Pathologically, there are various histologic types, including mucinous neoplasm, adenocarcinoma, and neuroendocrine tumor (carcinoid). In particular, mucinous lesions may cause mucinous leakage into the peritoneal cavity (pseudomyxoma peritonei).
• Main symptoms:
  • Vomiting, loss of appetite, abdominal pain (initially around the navel or in the upper abdomen → later migrating to the right lower abdomen), fever, chills
  • Right lower abdominal tenderness and rebound tenderness (pain when the hand is removed) may occur, clinically overlapping with appendicitis
  • May also be discovered incidentally without symptoms
  • As the disease progresses, localized or generalized peritonitis due to perforation, spread within the abdominal cavity, and metastasis to other organs (peritoneum, lymph nodes, liver, ovaries, lungs, etc.) may occur

Treatment Methods and Precautions

• Diagnostic principles:
  • Confirmation through imaging tests (abdominal computed tomography (CT)), blood tests (including tumor markers), colonoscopy and, if necessary, biopsy; in some cases, diagnostic surgery
• Standard treatment (general principles):
  • Localized lesion: Surgical removal first (appendectomy or consideration of right hemicolectomy depending on the size and location of the lesion)
  • Mucinous peritoneal spread (pseudomyxoma peritonei) or extensive metastasis within the abdominal cavity: Consider cytoreductive surgery together with hyperthermic intraperitoneal chemotherapy (HIPEC)
  • For metastatic or lymph-node-involved adenocarcinoma, systemic chemotherapy (such as colorectal cancer chemotherapy regimens) may be used
  • Neuroendocrine tumors are individualized according to the pathological grade, with options including surgery, endocrine therapy, targeted therapy, or chemotherapy
• Adjunctive and alternative treatments:
  • Chemotherapy, targeted therapy, and, selectively, radiation therapy may be considered depending on the pathological type, stage, and the patient's overall condition
• Lifestyle management and precautions:
  • Follow the physician's instructions regarding fasting and medication management before and after surgery; immediately visit a medical institution if symptoms of wound infection or peritonitis (high fever, severe abdominal pain, abdominal rigidity, etc.) occur
  • Perforation and peritonitis increase mortality (reference range: 0.1%–5%) and the risk of complications
• Prognosis:
  • Prognosis varies greatly depending on the pathological type and stage and whether there is peritoneal spread. When peritoneal metastasis is present, long-term treatment (surgery + HIPEC, etc.) is required, and there is a risk of recurrence
• Complications:
  • Appendiceal perforation, localized or generalized peritonitis, intra-abdominal metastasis (peritoneum, liver, ovaries, etc.), and postoperative intestinal adhesions and functional impairment

Checklist Before an Outpatient Visit (Items to Bring and Records)

• Documents/items to bring:
  • List of currently used medications (including prescription drugs, OTC medicines, herbal medicines, and supplements)
  • Copies of previous test results (CT, ultrasound, blood tests, endoscopy findings, pathology results, etc.)
  • Allergy history (medications, anesthesia, etc.)
  • History of surgery and anesthesia and major underlying conditions (heart disease, lung disease, diabetes, etc.)
• Recommended symptom records:
  • Time of symptom onset and progression (whether the location of pain has changed, presence of fever, frequency of vomiting)
  • Nature and intensity of pain (pain score), changes in appetite and bowel movements
  • Recent weight changes and systemic symptoms (fatigue, jaundice, etc.)
  • For women, genital symptoms and menstrual history (considering the possibility of ovarian involvement)
• Information helpful to provide to healthcare professionals during the outpatient visit:
  • Whether antibiotics or steroids have been taken recently
  • Family history (gastrointestinal cancers, hereditary tumor syndromes, etc.)

Examples of General Questions to Ask the Doctor

• What is the exact pathological diagnosis of my lesion (histologic type and grade)?
• How is my stage determined, and what is the prognosis (survival rate and risk of recurrence)?
• What treatment options do you recommend (extent of surgery, chemotherapy, etc.), and what are the advantages and disadvantages of each treatment?
• What are the major side effects of surgery or chemotherapy, and what is the recovery period?
• Is there concern about fertility or gynecologic effects, and what options are available if I wish to preserve fertility?

Examples of Disease-Specific Questions

• If the pathology result shows a mucinous neoplasm, what is the risk of developing pseudomyxoma peritonei, and what recommendations are available for its prevention and treatment?
• If invasive adenocarcinoma or lymph-node involvement is found on biopsy, how is it determined whether right hemicolectomy is necessary or appendectomy alone is sufficient?
• If there is peritoneal metastasis, what are the indications and prognosis for cytoreductive surgery + HIPEC?

(Bringing the above items to your appointment can help with consultation and establishing a diagnostic plan.)