Surface epithelial-stromal tumor
Encyclopedia
Surface epithelial-stromal tumor
s are a class of ovarian neoplasm
s that may be benign
or malignant
. Neoplasms in this group are thought to be derived from the ovarian
surface epithelium
(modified peritoneum
) or from ectopic endometrial or Fallopian tube
(tubal) tissue. This group of tumors accounts for the majority of all ovarian tumors. Serum CA-125
is often elevated but is only 50% accurate so it is not a useful tumor marker to assess the progress of treatment.
, the presence of papillary processes, and the location of the epithelial elements. Microscopic
pathological
features determine whether a surface epithelial-stromal tumor is benign
, borderline
, or malignant
(evidence of malignancy and stromal invasion). Borderline tumors are of uncertain malignant potential.
This group consists of serous, mucinous
, endometrioid
, clear cell
, and brenner (transitional cell) tumors, though there are a few mixed, undifferentiated and unclassified types.
Components can include:
The chance of malignancy of the tumor increases with the amount of solid areas present, including both papillary structures and any necrotic tissue present.
The five year survival rate of borderline and malignant tumors confined to the ovaries are 100% and 70% respectively. If the peritoneum is involved, these rates become 90% and 25%.
While the 5-year survival rates of borderline tumors are excellent, this should not be seen as evidence of cure, as recurrences can occur many years later.
s:
. As primary ovarian mucinous tumors are usually unilateral (in one ovary), the presentation of bilateral mucinous tumors requires exclusion of a non-ovarian origin.
s account for approximately 20% of all ovarian cancers and are mostly malignant (endometroid carcinomas). They are made of tubular glands bearing a close resemblance to benign or malignant endometrium. 15-30% of endometrioid carcinomas occur in individuals with carcinoma of the endometrium, and these patients have a better prognosis. They appear similar to other surface epithelial-stromal tumors, with solid and cystic areas. 40% of these tumors are bilateral, when bilateral, metastases is often present.
s are characterized by large epithelial cells with abundant clear cytoplasm and may be seen in association with endometriosis or endometrioid carcinoma of the ovary, bearing a resemblance to clear cell carcinoma of the endometrium. They may be predominantly solid or cystic. If solid, the clear cells tend to be arranged in sheets or tubules. In the cystic variety, the neoplastic cells make up the cyst lining.
.
For advanced cancer of this histology, the US National Cancer Institute
recommends a method of chemotherapy
that combines intravenous (IV) and intraperitoneal (IP) administration. Preferred chemotherapeutic agents include a platinum
drug with a taxane
.
are the pleural
cavity (33%), the liver
(26%), and the lung
s (3%).
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
s are a class of ovarian neoplasm
Ovarian 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....
s that may be benign
Benign
A benign tumor is a tumor that lacks the ability to metastasize. Common examples of benign tumors include moles and uterine fibroids.The term "benign" implies a mild and nonprogressive disease. Indeed, many kinds of benign tumors are harmless to human health...
or malignant
Malignant
Malignancy is the tendency of a medical condition, especially tumors, to become progressively worse and to potentially result in death. Malignancy in cancers is characterized by anaplasia, invasiveness, and metastasis...
. Neoplasms in this group are thought to be derived from the ovarian
Ovary
The ovary is an ovum-producing reproductive organ, often found in pairs as part of the vertebrate female reproductive system. Ovaries in anatomically female individuals are analogous to testes in anatomically male individuals, in that they are both gonads and endocrine glands.-Human anatomy:Ovaries...
surface epithelium
Epithelium
Epithelium is one of the four basic types of animal tissue, along with connective tissue, muscle tissue and nervous tissue. Epithelial tissues line the cavities and surfaces of structures throughout the body, and also form many glands. Functions of epithelial cells include secretion, selective...
(modified 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 from ectopic endometrial or Fallopian tube
Fallopian tube
The Fallopian tubes, also known as oviducts, uterine tubes, and salpinges are two very fine tubes lined with ciliated epithelia, leading from the ovaries of female mammals into the uterus, via the utero-tubal junction...
(tubal) tissue. This group of tumors accounts for the majority of all ovarian tumors. Serum CA-125
CA-125
CA-125 also known as mucin 16 or MUC16 is a protein that in humans is encoded by the MUC16 gene. MUC16 is a member of the mucin family glycoproteins...
is often elevated but is only 50% accurate so it is not a useful tumor marker to assess the progress of treatment.
Classification
Epithelial-stromal tumors are classified on the basis of the epithelial cell type, the relative amounts of epithelium and stromaStroma of ovary
The stroma of the ovary is a peculiar soft tissue, abundantly supplied with blood vessels, consisting for the most part of spindle-shaped cells with a small amount of ordinary connective tissue....
, the presence of papillary processes, and the location of the epithelial elements. Microscopic
Microscope
A microscope is an instrument used to see objects that are too small for the naked eye. The science of investigating small objects using such an instrument is called microscopy...
pathological
Pathology
Pathology is the precise study and diagnosis of disease. The word pathology is from Ancient Greek , pathos, "feeling, suffering"; and , -logia, "the study of". Pathologization, to pathologize, refers to the process of defining a condition or behavior as pathological, e.g. pathological gambling....
features determine whether a surface epithelial-stromal tumor is benign
Benign
A benign tumor is a tumor that lacks the ability to metastasize. Common examples of benign tumors include moles and uterine fibroids.The term "benign" implies a mild and nonprogressive disease. Indeed, many kinds of benign tumors are harmless to human health...
, borderline
Ovarian 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....
, or malignant
Malignant
Malignancy is the tendency of a medical condition, especially tumors, to become progressively worse and to potentially result in death. Malignancy in cancers is characterized by anaplasia, invasiveness, and metastasis...
(evidence of malignancy and stromal invasion). Borderline tumors are of uncertain malignant potential.
This group consists of serous, mucinous
Mucinous tumor
Mucinous tumors are part of the surface epithelial-stromal tumor group of ovarian neoplasms, and account for 12-15% of all ovarian tumors.Approximately 75% are benign, 10% are borderline and 15% are malignant....
, endometrioid
Endometrioid tumor
Endometrioid tumors are a class of tumor characterized by a resemblance to endometrium/ endometrial carcinoma, and over a third of cases have focal squamous differentiation.-Ovary:...
, clear cell
Clear cell adenocarcinoma
Clear cell adenocarcinoma or mesonephroma is a rare type of adenocarcinoma. It usually affects cells in the female genital tract. Vaginal clear cell adenoma is common in women in their late teens and early 20's whose mothers took diethylstilbestrol during pregnancy.Some of these cancers may not...
, and brenner (transitional cell) tumors, though there are a few mixed, undifferentiated and unclassified types.
Serous tumors
- These tumors vary in size from small and nearly imperceptible to large, filling the abdominal cavity.
- BenignBenignA benign tumor is a tumor that lacks the ability to metastasize. Common examples of benign tumors include moles and uterine fibroids.The term "benign" implies a mild and nonprogressive disease. Indeed, many kinds of benign tumors are harmless to human health...
, borderline, and malignantMalignantMalignancy is the tendency of a medical condition, especially tumors, to become progressively worse and to potentially result in death. Malignancy in cancers is characterized by anaplasia, invasiveness, and metastasis...
types of serous tumors account for about 30% of all ovarian tumors. - 75% are benignBenignA benign tumor is a tumor that lacks the ability to metastasize. Common examples of benign tumors include moles and uterine fibroids.The term "benign" implies a mild and nonprogressive disease. Indeed, many kinds of benign tumors are harmless to human health...
or of borderline malignancy, and 25% are malignantMalignantMalignancy is the tendency of a medical condition, especially tumors, to become progressively worse and to potentially result in death. Malignancy in cancers is characterized by anaplasia, invasiveness, and metastasis... - The malignant form of this tumor, serous cystadenocarcinomaCystadenocarcinomaCystadenocarcinoma is a malignant form of a cystadenoma and is a malignant neoplasm derived from glandular epithelium, in which cystic accumulations of retained secretions are formed. The neoplastic cells manifest varying degrees of anaplasia and invasiveness, and local extension and metastases occur...
, accounts for approximately 40% of all carcinomas of the ovary and are the most common malignant ovarian tumors. - Benign and borderline tumors are most common between the ages of 20 and 50 years.
- Malignant serous tumors occur later in life on average, although somewhat earlier in familial cases.
- 20% of benign, 30% of borderline, and 66% of malignant tumors are bilateral (affect both ovaries).
Components can include:
- cystic areas
- cystic and fibrous areas
- predominantly fibrous areas
The chance of malignancy of the tumor increases with the amount of solid areas present, including both papillary structures and any necrotic tissue present.
Pathology
- lined by tall, columnar, ciliated epithelial cells
- filled with clear serous fluid
- the term serous which originated as a description of the cystCystA cyst is a closed sac, having a distinct membrane and division on the nearby tissue. It may contain air, fluids, or semi-solid material. A collection of pus is called an abscess, not a cyst. Once formed, a cyst could go away on its own or may have to be removed through surgery.- Locations :* Acne...
fluid has come to be describe the particular type of epithelial cellCell (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....
seen in these tumors - may involve the surface of the ovary
- the division between benign, borderline, and malignant is ascertained by assessing:
- cellular atypia (whether or not individual cells look abnormal)
- invasion of surrounding ovarian stroma (whether or not cells are infiltrating surrounding tissue)
- borderline tumors may have cellular atypia but do NOT have evidence of invasion
- the presence of psammoma bodiesPsammoma bodyA psammoma body is a round collection of calcium, seen microscopically. The term is derived from the Greek word psammos meaning "sand."-Etiology:...
are a characteristic microscopic finding of cystadenocarcinomas
Prognosis
The prognosis of a serous tumor, like most neoplasms, depends on- degree of differentiation
- this is how closely the tumor cells resemble benign cells
- a well-differentiated tumor closely resembles benign tumors
- a poorly differentiated tumor may not resemble the cell type of origin at all
- a moderately differentiated tumor usually resembles the cell type of origin, but appears frankly malignant
- extension of tumor to other structures
- in particular with serous malignancies, the presence of malignant spread to the peritoneum is important with regard to prognosis.
The five year survival rate of borderline and malignant tumors confined to the ovaries are 100% and 70% respectively. If the peritoneum is involved, these rates become 90% and 25%.
While the 5-year survival rates of borderline tumors are excellent, this should not be seen as evidence of cure, as recurrences can occur many years later.
Mucinous tumors
Mucinous tumorMucinous tumor
Mucinous tumors are part of the surface epithelial-stromal tumor group of ovarian neoplasms, and account for 12-15% of all ovarian tumors.Approximately 75% are benign, 10% are borderline and 15% are malignant....
s:
- Closely resemble their serous counterparts
- Somewhat less common, accounting for about 25% of all ovarian neoplasms
- Occur principally in middle adult life and are rare before puberty and after menopause
- 80% are benign or borderline and about 15% are malignant
- Mucinous cystadenocarcinomas (the malignant form of this tumor) are relatively uncommon and account for only 10% of all ovarian cancers
- Mucinous tumors are characterized by more cysts of variable size and a rarity of surface involvement as compared to serous tumors
- Also in comparison to serous tumors, mucinous tumors are less frequently bilateral, approximately 5% of primary mucinous tumors are bilateral.
- May form very large cystic masses, with recorded weights exceeding 25 kg
- Appear as multiloculated tumors filled with sticky, gelatinous fluid
Pathology
Benign mucinous tumors are characterized by a lining of tall columnar epithelial cells with apical mucin and the absence of cilia, similar in appearance with benign cervical or intestinal epithelia. Cystadenocarcinomas (malignant tumors) contain a more solid growth pattern with the hallmarks of malignancy: cellular atypia and stratification, loss of the normal architecture of the tissu, and necrosis. The appearance can look similar to colonic cancer. Clear stromal invasion is used to differentiate borderline tumors from malignant tumors.Prognosis
10-year survival rates for borderline tumors contained within the ovary, malignant tumors without invasion, and invasive malignant tumors are greater than 95%, 90%, and 66%, respectively. One rare but noteworthy condition associated with mucinous ovarian neoplasms is pseudomyxoma peritoneiPseudomyxoma 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...
. As primary ovarian mucinous tumors are usually unilateral (in one ovary), the presentation of bilateral mucinous tumors requires exclusion of a non-ovarian origin.
Endometrioid tumors
Endometrioid tumorEndometrioid tumor
Endometrioid tumors are a class of tumor characterized by a resemblance to endometrium/ endometrial carcinoma, and over a third of cases have focal squamous differentiation.-Ovary:...
s account for approximately 20% of all ovarian cancers and are mostly malignant (endometroid carcinomas). They are made of tubular glands bearing a close resemblance to benign or malignant endometrium. 15-30% of endometrioid carcinomas occur in individuals with carcinoma of the endometrium, and these patients have a better prognosis. They appear similar to other surface epithelial-stromal tumors, with solid and cystic areas. 40% of these tumors are bilateral, when bilateral, metastases is often present.
Pathology
- Glands bearing a strong resemblance to endometrial-type glands
- Benign tumors have mature-appearing glands in a fibrous stroma
- Borderline tumors have a complex branching pattern without stromal invasion
- Carcinomas (malignant tumors) have invasive glands with crowded, atypical cells, frequent mitoses. With poorer differentiation, the tumor becomes more solid.
Prognosis
Prognosis again is dependent on the spread of the tumor, as well as how differentiated the tumor appears. The overall prognosis is somewhat worse than for serous or mucinous tumors, and the 5-year survival rate for patients with tumors confined to the ovary is approximately 75%.Clear cell tumors
Clear cell tumorClear cell adenocarcinoma
Clear cell adenocarcinoma or mesonephroma is a rare type of adenocarcinoma. It usually affects cells in the female genital tract. Vaginal clear cell adenoma is common in women in their late teens and early 20's whose mothers took diethylstilbestrol during pregnancy.Some of these cancers may not...
s are characterized by large epithelial cells with abundant clear cytoplasm and may be seen in association with endometriosis or endometrioid carcinoma of the ovary, bearing a resemblance to clear cell carcinoma of the endometrium. They may be predominantly solid or cystic. If solid, the clear cells tend to be arranged in sheets or tubules. In the cystic variety, the neoplastic cells make up the cyst lining.
Prognosis
These tumors tend to be aggressive, the five year survival rate for tumors confined to the ovaries is approximately 65%. If the tumor has spread beyond the ovary at diagnosis, the prognosis is poorBrenner tumor
Brenner tumors are uncommon surface-epithelial stromal cell tumors in which the epithelial cell (which defines these tumors) is a transitional cell. These are similar in appearance to bladder epithelia. The tumors may be very small to very large, and may be solid or cystic. Histologically, the tumor consists of nests of the aforementioned transitional cells within surrounding tissue that resembles normal ovary. Brenner tumors may be benign or malignant, depending on whether the tumor cells invade the surrounding tissue.Treatment
For more general information, see 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....
.
For advanced cancer of this histology, the US National Cancer Institute
National Cancer Institute
The National Cancer Institute is part of the National Institutes of Health , which is one of 11 agencies that are part of the U.S. Department of Health and Human Services. The NCI coordinates the U.S...
recommends a method of chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
that combines intravenous (IV) and intraperitoneal (IP) administration. Preferred chemotherapeutic agents include a platinum
Platinum
Platinum is a chemical element with the chemical symbol Pt and an atomic number of 78. Its name is derived from the Spanish term platina del Pinto, which is literally translated into "little silver of the Pinto River." It is a dense, malleable, ductile, precious, gray-white transition metal...
drug with a taxane
Taxane
The taxanes are diterpenes produced by the plants of the genus Taxus . As their name suggests, they were first derived from natural sources, but some have been synthesized artificially. Taxanes include paclitaxel and docetaxel . Paclitaxel was originally derived from the Pacific yew tree.Taxanes...
.
Metastases
For surface epithelial-stromal tumors, the most common sites of metastasisMetastasis
Metastasis, or metastatic disease , is the spread of a disease from one organ or part to another non-adjacent organ or part. It was previously thought that only malignant tumor cells and infections have the capacity to metastasize; however, this is being reconsidered due to new research...
are the pleural
cavity (33%), the liver
Liver
The liver is a vital organ present in vertebrates and some other animals. It has a wide range of functions, including detoxification, protein synthesis, and production of biochemicals necessary for digestion...
(26%), and the lung
Lung
The lung is the essential respiration organ in many air-breathing animals, including most tetrapods, a few fish and a few snails. In mammals and the more complex life forms, the two lungs are located near the backbone on either side of the heart...
s (3%).