Appendix cancer

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Tumors of the appendix; Appendiceal cancer; Cancer of the appendix

Overview

Appendix cancer (also known as appendiceal cancer) is a rare malignancy of the vermiform appendix. The most common type of appendix cancer is carcinoid tumor. Appendix cancers often present with peritoneal seeding, resulting in peritoneal carcinomatosis or pseudomyxoma peritonei. Appendix cancer may be classified according to WHO classification into 4 groups: epithelial tumors, non-epithelial tumors, secondary tumors, and hyperplastic (metaplastic) polyp. Common causes of appendix cancer may include genetic syndromes (eg. familial adenomatous polyposis, hereditary non-polyposis colorectal cancer) and chronic inflammatory diseases (eg. ulcerative colitis, Crohn's disease). The estimated prevalence of appendix cancer is approximately 0.12 cases per 100,000 individuals in the United States.

Historical Perspective

• Appendix cancer was first described by Gagne, a French Physician, in 1969.

Classification

• Appendix cancer may be classified according to WHO classification into 4 groups:

• Epithelial tumors
• Non-epithelial tumors
• Secondary tumors
• Hyperplastic (metaplastic) polyp

• The table below summarizes the different types of appendix cancer according to the WHO classification

WHO histological classification Tumors of the appendix Adapted from WHO/IARC
Epithelial tumors
Adenoma Tubular Villous Tubulovillous Serrated Carcinoma Adenocarcinoma Mucinous adenocarcinoma Signet-ring cell carcinoma Small cell carcinoma Undifferentiated carcinoma Carcinoid (well differentiated endocrine neoplasm) Tubular carcinoid Goblet cell carcinoid (mucinous carcinoid) Mixed carcinoid-adenocarcinoma Others
Non-epithelial tumors
Neuroma Lipoma Leiomyoma Gastrointestinal stromal tumor Leiomyosarcoma Kaposi sarcoma Others
Secondary tumors
Metastasis (eg. Primary of urogenital tract, breast, lung)
Hyperplastic polyp

Pathophysiology

• The pathogenesis of appendix cancer is characterized by a chronic inflammatory process.
• The KRAS gene mutation has been associated with the development of appendix cancer.
• On gross pathology, well-demarcated mass, less than 1 cm, and gray or yellowish color, and deformed appendix are characteristic findings of appendix cancer.
• On microscopic histopathological analysis findings will depend on the subtype of appendicular cancer.
• Common histopathological findings, may include:

• Cystic structures
• Angiolymphatic invasion
• Well differentiated and mucinous

• 
  Appendiceal carcinoid
• 
  Appendiceal carcinoid
• 
  Appendiceal carcinoid with necrosis
• 
  Carcinoid synaptophysin
• 
  Appendiceal tumor

Causes

• Common causes of appendix cancer may include:

• Hereditary syndromes

• Familial adenomatous polyposis
• Hereditary non-polyposis colorectal cancer

• Chronic inflammatory diseases

• Ulcerative colitis
• Crohn's disease

Differentiating Appendix Cancer from Other Diseases

• Appendix cancer must be differentiated from other diseases that cause abdominal pain, nausea, vomiting such as:

• Appendicitis
• Typhlitis
• Infectious terminal ileitis
• Ileocecal enteric duplication cyst

Epidemiology and Demographics

• The estimated prevalence of appendix cancer is approximately 0.12 cases per 100,000 individuals in the United States.
• The estimated prevalence of adenocarcinoma of the appendix is 0.2 cases per 100,000 individuals worldwide.

Age

• The median age at diagnosis of patients with appendix cancer is 65 years.
• Appendix cancer is more commonly observed among patients aged 50 to 60 years old.
• Appendix cancer is more commonly observed among adults and elderly patients.

Gender

• Males are more commonly affected with appendix cancer than females.

Race

• There is no racial predilection for appendix cancer.

Risk Factors

• Common risk factors in the development of appendix cancer are family history of cancer, long-standing ulcerative colitis, and chronic inflammatory bowel diseases.

Natural History, Complications and Prognosis

• The majority of patients with appendix cancer remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with appendix cancer may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of appendix cancer include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with appendix cancer is approximately [#%].

Diagnosis

Diagnostic Criteria

• The diagnosis of appendix cancer is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms

• Appendix cancer is usually asymptomatic.
• Symptoms of appendix cancer may include the following:

• [symptom 1]
• [symptom 2]
• [symptom 3]
• [symptom 4]
• [symptom 5]
• [symptom 6]

Physical Examination

• Patients with appendix cancer usually appear [general appearance].
• Physical examination may be remarkable for:

• [finding 1]
• [finding 2]
• [finding 3]
• [finding 4]
• [finding 5]
• [finding 6]

Laboratory Findings

• There are no specific laboratory findings associated with appendix cancer.

• A [positive/negative] [test name] is diagnostic of appendix cancer.
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of appendix cancer.
• Other laboratory findings consistent with the diagnosis of appendix cancer include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

• There are no [imaging study] findings associated with appendix cancer.

• [Imaging study 1] is the imaging modality of choice for appendix cancer.
• On [imaging study 1], appendix cancer is characterized by [finding 1], [finding 2], and [finding 3].
• [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

• Appendix cancer may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• There is no treatment for appendix cancer; the mainstay of therapy is supportive care.

• The mainstay of therapy for appendix cancer is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for appendix cancer.
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of appendix cancer.
• [Surgical procedure] can only be performed for patients with [disease stage] appendix cancer.

Prevention

• There are no primary preventive measures available for appendix cancer.

• Effective measures for the primary prevention of appendix cancer include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with appendix cancer are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].