Surface epithelial-stromal tumors are a class of

ovarian The ovary is an organ in the female reproductive system that produces an ovum. When released, this travels down the fallopian tube into the uterus, where it may become fertilized by a sperm. There is an ovary () found on each side of the body ...

neoplasm A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

s that may be

benign Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

malignant Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

Neoplasm A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

s in this group are thought to be derived from the ovarian surface epithelium (modified

peritoneum The peritoneum is the serous membrane forming the lining of the abdominal cavity or coelom in amniotes and some invertebrates, such as annelids. It covers most of the intra-abdominal (or coelomic) organs, and is composed of a layer of meso ...

) or from ectopic

endometrial The endometrium is the inner epithelial layer, along with its mucous membrane, of the mammalian uterus. It has a basal layer and a functional layer: the basal layer contains stem cells which regenerate the functional layer. The functional layer ...

or Fallopian tube (tubal) tissue. Tumors of this type are also called ovarian adenocarcinoma. This group of tumors accounts for 90% to 95% of all cases of

ovarian cancer Ovarian cancer is a cancerous tumor of an ovary. It may originate from the ovary itself or more commonly from communicating nearby structures such as fallopian tubes or the inner lining of the abdomen. The ovary is made up of three different c ...

; however is mainly only found in postmenopausal women with the exception of the United States where 7% of cases occur in women under the age of 40. Serum

CA-125 Mucin-16 (MUC-16) also known as Ovarian cancer-related tumor marker CA125 is a protein that in humans is encoded by the ''MUC16'' gene. MUC-16 is a member of the mucin family glycoproteins. MUC-16 has found application as a tumor marker or biom ...

is often elevated but is only 50% accurate so it is not a useful tumor marker to assess the progress of treatment. 75% of women with epithelial ovarian cancer are found within the advanced-stages; however younger patients are more likely to have better prognoses than older patients.

Classification

Ovarian tumors by incidence and cancer risk

Epithelial-stromal tumors are classified on the basis of the epithelial cell type, the relative amounts of epithelium and stroma, the presence of

papillary Papilla (Latin, 'nipple') or papillae may refer to: In animals * Papilla (fish anatomy), in the mouth of fish * Basilar papilla, a sensory organ of lizards, amphibians and fish * Dental papilla, in a developing tooth * Dermal papillae, part of ...

processes, and the location of the epithelial elements.

Microscopic The microscopic scale () is the scale of objects and events smaller than those that can easily be seen by the naked eye, requiring a lens or microscope to see them clearly. In physics, the microscopic scale is sometimes regarded as the scale be ...

pathological Pathology is the study of the causes and effects of disease or injury. The word ''pathology'' also refers to the study of disease in general, incorporating a wide range of biology research fields and medical practices. However, when used in th ...

features determine whether a surface epithelial-stromal tumor is

, a borderline tumor, or

(evidence of malignancy and stromal invasion). Borderline tumors are of uncertain malignant potential. This group consists of

serous In physiology, serous fluid or serosal fluid (originating from the Medieval Latin word ''serosus'', from Latin ''serum'') is any of various body fluids resembling serum, that are typically pale yellow or transparent and of a benign nature. The flu ...

mucinous Mucus ( ) is a slippery aqueous secretion produced by, and covering, mucous membranes. It is typically produced from cells found in mucous glands, although it may also originate from mixed glands, which contain both serous and mucous cells. It i ...

, endometrioid,

clear cell In histology, a clear cell is a cell that shows a clear cytoplasm when stained with hematoxylin and eosin (H&E). Normal histology In the skin, some secretory cells in the epithelium appear as clear cells, and are one of the components of eccrine ...

, and brenner (transitional cell) tumors, though there are a few mixed, undifferentiated and unclassified types.

Serous tumors

Histopathology of lining of a benign serous tumor of the ovary

*These tumors vary in size from small and nearly imperceptible to large, filling the

abdominal The abdomen (colloquially called the belly, tummy, midriff, tucky or stomach) is the part of the body between the thorax (chest) and pelvis, in humans and in other vertebrates. The abdomen is the front part of the abdominal segment of the torso ...

cavity. *

Benign Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

, borderline, and

types of serous tumors account for about 30% of all ovarian tumors. *75% are benign or of borderline malignancy, and 25% are malignant *The malignant form of this tumor, serous cystadenocarcinoma, 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 Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

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 The cilium, plural cilia (), is a membrane-bound organelle found on most types of eukaryotic cell, and certain microorganisms known as ciliates. Cilia are absent in bacteria and archaea. The cilium has the shape of a slender threadlike project ...

epithelial Epithelium or epithelial tissue is one of the four basic types of animal tissue, along with connective tissue, muscle tissue and nervous tissue. It is a thin, continuous, protective layer of compactly packed cells with a little intercell ...

cells *filled with clear

fluid *the term

which originated as a description of the

cyst A cyst is a closed sac, having a distinct envelope and division compared with the nearby tissue. Hence, it is a cluster of cells that have grouped together to form a sac (like the manner in which water molecules group together to form a bubble) ...

fluid has come to be describe the particular type of

cell Cell most often refers to: * Cell (biology), the functional basic unit of life Cell may also refer to: Locations * Monastic cell, a small room, hut, or cave in which a religious recluse lives, alternatively the small precursor of a monastery ...

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 bodies 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 The five-year survival rate is a type of survival rate for estimating the prognosis of a particular disease, normally calculated from the point of diagnosis. Lead time bias from earlier diagnosis can affect interpretation of the five-year surviva ...

of borderline tumors 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 tumors: *Closely resemble their serous counterparts but unlikely to be bilateral *Somewhat less common, accounting for about 25% of all ovarian neoplasms * In some cases 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

Pathology

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. The appearance can look similar to colonic or ovarian cancer, but typically originates from the appendix (see

mucinous adenocarcinoma A mucinous neoplasm (also called colloid neoplasm) is an abnormal and excessive growth of tissue (neoplasia) with associated mucin (a fluid that sometimes resembles thyroid colloid). It arises from epithelial cells that line certain internal organs ...

with clinical condition

Pseudomyxoma peritonei Pseudomyxoma peritonei (PMP) is a clinical condition caused by cancerous cells (mucinous adenocarcinoma) that produce abundant mucin or gelatinous ascites. The tumors cause fibrosis of tissues and impede digestion or organ function, and if left unt ...

). 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 peritonei Pseudomyxoma peritonei (PMP) is a clinical condition caused by cancerous cells (mucinous adenocarcinoma) that produce abundant mucin or gelatinous ascites. The tumors cause fibrosis of tissues and impede digestion or organ function, and if left unt ...

. As primary ovarian mucinous tumors are usually unilateral (in one ovary), the presentation of bilateral mucinous tumors requires exclusion of a non-ovarian origin, usually the appendix.

Endometrioid tumors

Endometrioid tumors 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

Ovarian clear cell carcinoma -a- very high mag - cropped

Clear cell tumors 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 poor

Brenner tumor

Brenner tumor Brenner tumors are an uncommon subtype of the surface epithelial-stromal tumor group of ovarian neoplasms. The majority are benign, but some can be malignant. They are most frequently found incidentally on pelvic examination or at laparotomy. Bre ...

s 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.

Small cell tumors

Small cell ovarian cancer (SCCO)
are generally classified into epithelial tumors associated with distinctive endocrine features. The World Health Organisation (WHO) recognises SCCO as two distinct entities
Small Cell Ovarian Cancer of Hypercalcemic Type (SCCOHT)
and Small Cell Ovarian Cancer of Pulmonary Type (SCCOPT). Small cell tumours are rare and aggressive, they contribute to less than 2% of all gynaecologic malignancies. The average age of diagnosis is 24 years old, and the majority of patients also present with hypercalcemia (62%). It typically present with a unilateral large tumor. Most women die within a year of diagnosis.

Treatment

For more general information, see

. Research suggests that in the first line treatment of Endometrial Ovarian Cancer (EOC), Pegylated Liposomal Doxorubicin paired with Carboplatin is a satisfactory alternative to Paclitaxel with Carboplatin. In people with platinum-sensitive relapsed EOC, research has found that Pegylated Liposomal Doxorubicin with Carboplatin is a better treatment than Paclitaxel with Carboplatin. For advanced cancer of this histology, the US

National Cancer Institute The National Cancer Institute (NCI) coordinates the United States National Cancer Program and is part of the National Institutes of Health (NIH), which is one of eleven agencies that are part of the U.S. Department of Health and Human Services. ...

recommends a method of

chemotherapy Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs ( chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemothe ...

that combines intravenous (IV) and

intraperitoneal The peritoneum is the serous membrane forming the lining of the abdominal cavity or coelom in amniotes and some invertebrates, such as annelids. It covers most of the intra-abdominal (or coelomic) organs, and is composed of a layer of mesothel ...

(IP) administration. Preferred

chemotherapeutic agent This is a list of chemotherapeutic agents, also known as cytotoxic agents or cytostatic drugs, that are known to be of use in chemotherapy for cancer. This list is organized by type of agent, although the subsections are not necessarily definitive ...

s include a

platinum Platinum is a chemical element with the symbol Pt and atomic number 78. It is a dense, malleable, ductile, highly unreactive, precious, silverish-white transition metal. Its name originates from Spanish , a diminutive of "silver". Pla ...

drug with a

taxane Taxanes are a class of diterpenes. They were originally identified from plants of the genus ''Taxus'' (yews), and feature a taxadiene core. Paclitaxel (Taxol) and docetaxel (Taxotere) are widely used as chemotherapy agents. Cabazitaxel was FDA ap ...

Metastases

For surface epithelial-stromal tumors, the most common sites of

metastasis Metastasis is a pathogenic agent's spread from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, then ...

are the

pleural cavity The pleural cavity, pleural space, or interpleural space is the potential space between the pleurae of the pleural sac that surrounds each lung. A small amount of serous pleural fluid is maintained in the pleural cavity to enable lubrication b ...

(33%), the

liver The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it ...

(26%), and the lungs (3%).

Effect on fertility

Fertility Fertility is the capability to produce offspring through reproduction following the onset of sexual maturity. The fertility rate is the average number of children born by a female during her lifetime and is quantified demographically. Fertili ...

subsequent to treatment of surface epithelial-stromal tumors depends mainly on histology and initial staging to separate it into early borderline (or more benign) versus advanced stages of borderline (or more malignant). Conservative management (without bilateral

oophorectomy Oophorectomy (; from Greek , , 'egg-bearing' and , , 'a cutting out of'), historically also called ''ovariotomy'' is the surgical removal of an ovary or ovaries. The surgery is also called ovariectomy, but this term is mostly used in reference ...

) of early stage borderline tumors have been estimated to result in chance of over 50% of spontaneous pregnancy with a low risk of lethal recurrence of the tumor (0.5%). On the other hand, in cases of conservative treatment in advanced stage borderline tumors, spontaneous pregnancy rates have been estimated to be 35% and the risk of lethal recurrence 2%.

References

Sources

External links

Johns Hopkins: "Surface Epithelial Tumors"

Small Cell Ovarian Cancer Research Collaboration & Patients Registry
{{DEFAULTSORT:Surface Epithelial-Stromal Tumor Gynaecological cancer