Appendix cancer
Encyclopedia
Appendix cancer or appendiceal cancer
are rare malignancies of the vermiform appendix
.
, but not all appendiceal carcinoids are malignant. Malignant features include size over (1 to 2 cm), presence in lymph nodes, or invasion of tissues adjacent to the appendix.
Adenocarcinoma
s are more common primary malignancies of the appendix than carcinoids. Even so, adenocarcinoma originating in the appendix is rare, and usually resembles colonic adenocarcinoma microscopically. The signet ring cell carcinoma
form carries a worse prognosis. Most mucinous tumors of the appendix are benign mucinous cystadenoma
s. The rare malignant form, mucinous cystadenocarcinoma
is identified by invasion through the wall of the appendix or epithelial cells in mucus
found in the peritoneum
. Appendiceal tumors can present with peritoneal seeding, resulting in peritoneal carcinomatosis or Pseudomyxoma peritonei
(PMP).
Gastrointestinal stromal tumor
s are rare tumors with malignant potential. Primary lymphoma
s can occur in the appendix. Breast cancer
, colon cancer, and tumors of the female genital tract may metastasize to the appendix.
A Hong Kong case series of 1492 appendectomies identified 17 neoplasms. Eight were carcinoids without specification of malignant features. Three cases of adenocarcinoma, and one each of cystadenocarcinoma, psedomyxoma peritoneii, and metastasic carcinoma were identified. The remaining tumors were benign.
.
Pseudomyxoma peritonei
treatment includes cytoreductive surgery
which includes the removal of visible tumor and affected essential organs within the abdomen and pelvis. The peritoneal cavity is infused with heated chemotherapy
known as HIPEC in an attempt to eradicate residual disease. The surgery may or may not be preceded or followed with intravenous chemotherapy.
was diagnosed with appendix cancer, and died of the disease in 1993.
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
are rare malignancies of the vermiform appendix
Vermiform appendix
The appendix is a blind-ended tube connected to the cecum , from which it develops embryologically. The cecum is a pouchlike structure of the colon...
.
Types
The most common tumor of the appendix is carcinoidCarcinoid
Carcinoid is a slow-growing type of neuroendocrine tumor, originating in the cells of the neuroendocrine system.In 2000, the World Health Organization redefined "carcinoid", but this new definition has not been accepted by all practitioners. This has led to some complexity in distinguishing...
, but not all appendiceal carcinoids are malignant. Malignant features include size over (1 to 2 cm), presence in lymph nodes, or invasion of tissues adjacent to the appendix.
Adenocarcinoma
Adenocarcinoma
Adenocarcinoma is a cancer of an epithelium that originates in glandular tissue. Epithelial tissue includes, but is not limited to, the surface layer of skin, glands and a variety of other tissue that lines the cavities and organs of the body. Epithelium can be derived embryologically from...
s are more common primary malignancies of the appendix than carcinoids. Even so, adenocarcinoma originating in the appendix is rare, and usually resembles colonic adenocarcinoma microscopically. The signet ring cell carcinoma
Signet ring cell carcinoma
Signet ring cell carcinoma is an epithelial malignancy characterized by the histologic appearance of signet ring cells.It is a form of adenocarcinoma.-Histologic appearance:...
form carries a worse prognosis. Most mucinous tumors of the appendix are benign mucinous cystadenoma
Cystadenoma
Cystadenoma refers to a type of cystic adenoma.When malignant, it is called cystadenocarcinoma.-Classification:When not otherwise specified, the ICD-O coding is 8440/0...
s. The rare malignant form, mucinous cystadenocarcinoma
Mucinous cystadenocarcinoma
Mucinous cystadenocarcinoma is a type of tumor in the cystadenocarcinoma grouping.It can occur in the breast....
is identified by invasion through the wall of the appendix or epithelial cells in mucus
Mucus
In vertebrates, mucus is a slippery secretion produced by, and covering, mucous membranes. Mucous fluid is typically produced from mucous cells found in mucous glands. Mucous cells secrete products that are rich in glycoproteins and water. Mucous fluid may also originate from mixed glands, which...
found in the peritoneum
Peritoneum
The peritoneum is the serous membrane that forms the lining of the abdominal cavity or the coelom — it covers most of the intra-abdominal organs — in amniotes and some invertebrates...
. Appendiceal tumors can present with peritoneal seeding, resulting in peritoneal carcinomatosis or Pseudomyxoma peritonei
Pseudomyxoma peritonei
Pseudomyxoma Peritonei is an uncommon tumor known for its production of mucin in the abdominal cavity. If left untreated, mucin will eventually build up to the point where it compresses vital structures: the colon, the liver, kidneys,stomach, spleen, pancreas, etc.Unlike most cancers, this disease...
(PMP).
Gastrointestinal stromal tumor
Gastrointestinal stromal tumor
A gastrointestinal stromal tumor is one of the most common mesenchymal tumors of the gastrointestinal tract...
s are rare tumors with malignant potential. Primary lymphoma
Lymphoma
Lymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage...
s can occur in the appendix. Breast cancer
Breast cancer
Breast cancer is cancer originating from breast tissue, most commonly from the inner lining of milk ducts or the lobules that supply the ducts with milk. Cancers originating from ducts are known as ductal carcinomas; those originating from lobules are known as lobular carcinomas...
, colon cancer, and tumors of the female genital tract may metastasize to the appendix.
Epidemiology
A study of primary malignacies in the United States found a rate of 0.12 cases per 1,000,000 population per years. Carcinoids that were not identified as malignant were not included in this data. Carcinoid is found in roughly 1 in 300 appendectomies for acute appendicitis.A Hong Kong case series of 1492 appendectomies identified 17 neoplasms. Eight were carcinoids without specification of malignant features. Three cases of adenocarcinoma, and one each of cystadenocarcinoma, psedomyxoma peritoneii, and metastasic carcinoma were identified. The remaining tumors were benign.
Treatment
The treatment for tumors varies. Small carcinoids (<2 cm) without features of malignancy may be treated by appendectomy if complete removal is possible. Other carcinoids and adenocarcimoas may require right hemicolectomyColectomy
Colectomy consists of the surgical resection of any extent of the large intestine .-History:Sir William Arbuthnot-Lane was one of the early proponents of the usefulness of total colectomies, although his overuse of the procedure called the wisdom of the surgery into question.-Indications:Some of...
.
Pseudomyxoma peritonei
Pseudomyxoma peritonei
Pseudomyxoma Peritonei is an uncommon tumor known for its production of mucin in the abdominal cavity. If left untreated, mucin will eventually build up to the point where it compresses vital structures: the colon, the liver, kidneys,stomach, spleen, pancreas, etc.Unlike most cancers, this disease...
treatment includes cytoreductive surgery
Debulking
Debulking is the surgical removal of part of a malignant tumour which cannot be completely excised, so as to enhance the effectiveness of radiation or chemotherapy. It is used only in specific malignancies, as generally partial removal of a tumor is not considered a worthwhile intervention...
which includes the removal of visible tumor and affected essential organs within the abdomen and pelvis. The peritoneal cavity is infused with heated chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
known as HIPEC in an attempt to eradicate residual disease. The surgery may or may not be preceded or followed with intravenous chemotherapy.
Famous cases
Actress Audrey HepburnAudrey Hepburn
Audrey Hepburn was a British actress and humanitarian. Although modest about her acting ability, Hepburn remains one of the world's most famous actresses of all time, remembered as a film and fashion icon of the twentieth century...
was diagnosed with appendix cancer, and died of the disease in 1993.