Appendix cancer historical perspective

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2]

Historical Perspective

Discovery

• Appendix cancer was first described in the published literature by Sir George Thos. Beatson, an English surgeon, in 1913.

Landmark Events in the Development of Treatment Strategies

• Surgical sciences development

• Appendectomy
• Anesthesia
• Sterilization

• Chemotherapy
• Introduction of 5-fluorouracil (5-FU) the first coorectal chemotherapy agent
• Development of new chemotherapy agents like

• Irinotecan
• Oxaliplatin
• Vascular endothelial growth factor receptor inhibitors (bevacizumab)
• Epidermal growth factor receptor inhibitors (cetuximab and panitumumab),
• Aflibercept
• Regorafenib: inhibitor of angiogenic tyrosine kinases (including the VEGF receptors 1,2, and 3),
• Capecitabine or 5-FU with or without a platinum drug

Intraperitoneal chemotherapy including hyperthermic intraperitoneal chemotherapy plus/minus early postoperative intraperitoneal chemotherapy (EPIC)

• introduction of Octereotide analogs to control symptmoes of carcinoid syndrome

• Development of new Imaging modalities

• CT scan, MRI as well as specific imaging modalities such as Somatostatin scintigraphy revolutionized approaching to the patients with appendix cancer

Future horizons

• Genetic studies revolutionized cancer treatment; appendix cancer is not an exception.
• Traditionally appandiceal cancers were approached the same as colorectal cancers. Recent genetic studies demonstrated that appendiceal tumors are clearly differ from colorectal cancers.
  • Presence of mutated TP53 and APC genes were significantly lower in appendiceal cancers compared to colorectal cancers.

• It has been shown that mutation profiles are associated with the patients’ prognosis.
  • Mutations in the TP53 significantly decrease life expectancy in patients with appendix cancer.
  • Regardless of tumor grade, Tp 53 mutations were associated with poorer outcomes.
  • Patients with GNAS mutations had a life expectancy of 10 years after diagnosis.
  • Appendiceal tumors with GNAS mutations rarely develop into high-grade tumors.

Famous Cases

The following are a few famous cases of appendix cancer:

• Celebrated actress, Audrey Hepburn was the most famous victims of appendix cancer, she passed away in 1993.

• Stuart Scott, ESPN sportscast anchor was diagnosed with appendix cancer in 2007 and died 8 years later in 2015.