Primary peritoneal cancer
Encyclopedia
Primary peritoneal cancer or carcinoma is also known as: serous surface papillary carcinoma, primary peritoneal carcinoma, extra-ovarian serous carcinoma, primary serous papillary carcinoma, psammomacarcinoma. It was historically classified under "carcinoma of unknown primary" (CUP). Primary peritoneal cancer (PPC, or PPCa) is a cancer
of the cells
lining the peritoneum
, or abdominal cavity.
Some studies indicate that up to 15% of serous ovarian cancers are thought to be actually primary peritoneal carcinomas in origin.
Histomorphological
and molecular biological characteristics suggest that serous carcinoma
s, which include ovarian serous carcinoma, uterine serous carcinoma, Fallopian tube serous carcinoma, cervical serous carcinoma, and primary peritoneal serous carcinoma really represent one entity.
is thought to be of mesonephric origin. With the overall point being that normal ovarian and peritoneal tissue is derived from the mesonephros. On the contrary, fallopian tube epithelium, endometrium and endocervix are related to paramesonephros (Müllerian duct). Surprisingly, epithelial ovarian cancer and primary peritoneal cancer are histologically similar to the Mullerian epithelium; not their embryonal origin, the mesonephros
. Either a metaplasia has occurred or Mullerian remnants have been left behind in coelemic epithelium, which have turned oncogenic.
1/2 has been described. Furthermore, women with BRCA1/2 mutation have a 5% risk of developing primary peritoneal cancer even after prophylactic oophorectomy
.
Primary peritoneal carcinoma shows similar rates of tumor suppressor gene
dysfunction (p53
, BRCA
, WT1
) as ovarian cancer and can also show an increased expression of HER-2/neu.
An association with vascular endothelial growth factor
has been observed.
, which is epithelial ovarian cancer.
The median survival of primary peritoneal carcinomas is usually shorter by 2–6 months time when compared with serous ovarian cancer. Studies show median survival varies between 11.3–17.8 months. One study reported 19-40 month median survival (95% CI) with a 5 year survival of 26.5%.
Elevated albumin levels have been associated with a more favorable prognosis.
caused by exposure to asbestos
.
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...
of the cells
Cell (biology)
The cell is the basic structural and functional unit of all known living organisms. It is the smallest unit of life that is classified as a living thing, and is often called the building block of life. The Alberts text discusses how the "cellular building blocks" move to shape developing embryos....
lining 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...
, or abdominal cavity.
Some studies indicate that up to 15% of serous ovarian cancers are thought to be actually primary peritoneal carcinomas in origin.
Histomorphological
Histopathology
Histopathology refers to the microscopic examination of tissue in order to study the manifestations of disease...
and molecular biological characteristics suggest that serous carcinoma
Serous carcinoma
In pathology, serous carcinoma is an epithelial malignancy that arises from the lining of a cavity that produces a serum-like fluid .Serous lined cavities include the peritoneum, pericardium and pleural space and tunica vaginalis....
s, which include ovarian serous carcinoma, uterine serous carcinoma, Fallopian tube serous carcinoma, cervical serous carcinoma, and primary peritoneal serous carcinoma really represent one entity.
Theory
Ovarian and peritoneal epithelium share common embryonal origin, the coelomic epithelium (mesodermal origin). Coelomic epitheliumCoelomic epithelium
Coelomic epithelium refers to the epithelium that lines the surface of the body wall and abdominal organs. It constitutes the outermost layer of the male and female gonads, thus forming the germinal epithelium of the female or of the male. It is also called the germinal epithelium of Waldeyer or...
is thought to be of mesonephric origin. With the overall point being that normal ovarian and peritoneal tissue is derived from the mesonephros. On the contrary, fallopian tube epithelium, endometrium and endocervix are related to paramesonephros (Müllerian duct). Surprisingly, epithelial ovarian cancer and primary peritoneal cancer are histologically similar to the Mullerian epithelium; not their embryonal origin, the mesonephros
Mesonephros
The mesonephros is one of three excretory organs that develop in vertebrates. It serves as the main excretory organ of aquatic vertebrates and as a temporary kidney in reptiles, birds, and mammals. The mesonephros is included in the Wolffian body after Caspar Friedrich Wolff who described it in 1759...
. Either a metaplasia has occurred or Mullerian remnants have been left behind in coelemic epithelium, which have turned oncogenic.
Genetic causes
Although the precise causes are not known, a link with certain variants of BRCABRCA
BRCA can refer to one of several things:*BRCA1 and BRCA2, a pair of genes involved in breast cancer. See BRCA mutation*British Radio Car Association – an organisation governing all types of radio controlled car racing in the United Kingdom....
1/2 has been described. Furthermore, women with BRCA1/2 mutation have a 5% risk of developing primary peritoneal cancer even after prophylactic oophorectomy
Oophorectomy
Oophorectomy is the surgical removal of an ovary or ovaries. The surgery is also called ovariectomy, but this term has been traditionally used in basic science research describing the surgical removal of ovaries in laboratory animals...
.
Primary peritoneal carcinoma shows similar rates of tumor suppressor gene
Tumor suppressor gene
A tumor suppressor gene, or anti-oncogene, is a gene that protects a cell from one step on the path to cancer. When this gene is mutated to cause a loss or reduction in its function, the cell can progress to cancer, usually in combination with other genetic changes.-Two-hit hypothesis:Unlike...
dysfunction (p53
P53
p53 , is a tumor suppressor protein that in humans is encoded by the TP53 gene. p53 is crucial in multicellular organisms, where it regulates the cell cycle and, thus, functions as a tumor suppressor that is involved in preventing cancer...
, BRCA
BRCA
BRCA can refer to one of several things:*BRCA1 and BRCA2, a pair of genes involved in breast cancer. See BRCA mutation*British Radio Car Association – an organisation governing all types of radio controlled car racing in the United Kingdom....
, WT1
WT1
Wilms tumor protein is a protein that in humans is encoded by the WT1 gene.- Function :This gene encodes a transcription factor that contains four zinc finger motifs at the C-terminus and a proline / glutamine-rich DNA-binding domain at the N-terminus...
) as ovarian cancer and can also show an increased expression of HER-2/neu.
An association with vascular endothelial growth factor
Vascular endothelial growth factor
Vascular endothelial growth factor is a signal protein produced by cells that stimulates vasculogenesis and angiogenesis. It is part of the system that restores the oxygen supply to tissues when blood circulation is inadequate....
has been observed.
Prognosis and treatment
Prognosis and treatment is the same as for the most common type of ovarian cancerOvarian cancer
Ovarian cancer is a cancerous growth arising from the ovary. Symptoms are frequently very subtle early on and may include: bloating, pelvic pain, difficulty eating and frequent urination, and are easily confused with other illnesses....
, which is epithelial ovarian cancer.
The median survival of primary peritoneal carcinomas is usually shorter by 2–6 months time when compared with serous ovarian cancer. Studies show median survival varies between 11.3–17.8 months. One study reported 19-40 month median survival (95% CI) with a 5 year survival of 26.5%.
Elevated albumin levels have been associated with a more favorable prognosis.
Mesothelioma
This cancer involves mesothelial cells and thus could be medically described as mesothelioma, a rare form of cancerCancer
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...
caused by exposure to asbestos
Asbestos
Asbestos is a set of six naturally occurring silicate minerals used commercially for their desirable physical properties. They all have in common their eponymous, asbestiform habit: long, thin fibrous crystals...
.
External links
- Description at National Health Service (England)National Health Service (England)The National Health Service or NHS is the publicly funded healthcare system in England. It is both the largest and oldest single-payer healthcare system in the world. It is able to function in the way that it does because it is primarily funded through the general taxation system, similar to how...
- Description at American Cancer SocietyAmerican Cancer SocietyThe American Cancer Society is the "nationwide community-based voluntary health organization" dedicated, in their own words, "to eliminating cancer as a major health problem by preventing cancer, saving lives, and diminishing suffering from cancer, through research, education, advocacy, and...