Surface epithelial-stromal tumor

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Surface epithelial-stromal tumor
Brenner tumour high mag cropped.jpg
High magnification micrograph of a Brenner tumor, a type of surface epithelial-stromal tumor. H&E stain.
Specialty Oncology

Surface epithelial-stromal tumors are a class of ovarian neoplasms 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. Tumors of this type are also called ovarian adenocarcinoma. [1] This group of tumors accounts for 90% to 95% of all cases of ovarian cancer; 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. [2] [3] [4] [5] [6] [7] 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. 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. [8] [9] [10] [11] [12]

Contents

Classification

Ovarian tumors by incidence and risk of ovarian cancer, with surface epithelial-stromal tumors at top. Ovarian tumors by incidence and cancer risk.png
Ovarian tumors by incidence and risk of ovarian cancer, with surface epithelial-stromal tumors at top.
Ovarian cancers in women aged 20+, with area representing relative incidence and color representing 5-year relative survival rate. Surface epithelial-stromal tumors are labeled in center of the main diagram, and represent all types except the ones separated at top. Incidence of ovarian cancers by histopathology.png
Ovarian cancers in women aged 20+, with area representing relative incidence and color representing 5-year relative survival rate. Surface epithelial-stromal tumors are labeled in center of the main diagram, and represent all types except the ones separated at top.

Epithelial-stromal tumors are classified on the basis of the epithelial cell type, the relative amounts of epithelium and stroma, the presence of papillary processes, and the location of the epithelial elements. Microscopic pathological features determine whether a surface epithelial-stromal tumor is benign, a borderline tumor, 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.

Serous tumors

Histopathology of lining of a benign serous tumor of the ovary. Benign serous ovarian tumors are thin walled unilocular cysts that are lined by ciliated pseudostratified cuboidal or columnar epithelium. Histopathology of lining of a benign serous tumor of the ovary.jpg
Histopathology of lining of a benign serous tumor of the ovary. Benign serous ovarian tumors are thin walled unilocular cysts that are lined by ciliated pseudostratified cuboidal or columnar epithelium.

Components can include:

  1. cystic areas
  2. cystic and fibrous areas
  3. 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 cyst fluid has come to be describe the particular type of epithelial cell 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 [15]

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 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

Histopathology of lining of a benign mucinous tumor of the ovary. Benign mucinous ovarian tumors consist of simple, nonstratified columnar epithelium with basally-located hyperchromatic nuclei and resemble gastric foveolar epithelium. Histopathology of lining of a benign mucinous tumor of the ovary.jpg
Histopathology of lining of a benign mucinous tumor of the ovary. Benign mucinous ovarian tumors consist of simple, nonstratified columnar epithelium with basally-located hyperchromatic nuclei and resemble gastric foveolar epithelium.

Mucinous tumors:

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 with clinical condition Pseudomyxoma peritonei). 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. 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 (endometrioid 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

Micrograph of an ovarian clear cell carcinoma. H&E stain. Ovarian clear cell carcinoma -a- very high mag - cropped.jpg
Micrograph of an ovarian clear cell carcinoma. H&E stain.

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 tumour. H&E stain. Brenner tumour4.jpg
Brenner tumour. H&E stain.

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.

Small cell tumors

Small cell ovarian cancer (SCCO) are generally classified into epithelial tumors [16] associated with distinctive endocrine features. [17]

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). [17]

Small cell tumours are rare and aggressive, they contribute to less than 2% of all gynaecologic malignancies. [17] The average age of diagnosis is 24 years old, and the majority of patients also present with hypercalcemia (62%). [18] It typically present with a unilateral large tumor. [18] Most women die within a year of diagnosis. [18]

Treatment

For more general information, see ovarian cancer.

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. [19] In people with platinum-sensitive relapsed EOC, research has found that Pegylated Liposomal Doxorubicin with Carboplatin is a better treatment than Paclitaxel with Carboplatin. [20] There is also evidence to suggest that Pegylated Liposomal Doxorubicin with Carboplatin is tolerated better by people with platinum-sensitive relapsed EOC. [20]

For advanced cancer of this histology, the US National Cancer Institute recommends a method of chemotherapy that combines intravenous (IV) and intraperitoneal (IP) administration. [21] Preferred chemotherapeutic agents include a platinum drug with a taxane.

Metastases

For surface epithelial-stromal tumors, the most common sites of metastasis are the pleural cavity (33%), the liver (26%), and the lungs (3%). [22]

Effect on fertility

Fertility 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). [23] Conservative management (without bilateral oophorectomy) 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%). [23] 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%. [23]

Related Research Articles

<span class="mw-page-title-main">Ovarian cyst</span> Fluid-filled sac in the ovary

An ovarian cyst is a fluid-filled sac within the ovary. They usually cause no symptoms, but occasionally they may produce bloating, lower abdominal pain, or lower back pain. The majority of cysts are harmless. If the cyst either breaks open or causes twisting of the ovary, it may cause severe pain. This may result in vomiting or feeling faint, and even cause headaches.

<span class="mw-page-title-main">Endometrial cancer</span> Uterine cancer that is located in tissues lining the uterus

Endometrial cancer is a cancer that arises from the endometrium. It is the result of the abnormal growth of cells that have the ability to invade or spread to other parts of the body. The first sign is most often vaginal bleeding not associated with a menstrual period. Other symptoms include pain with urination, pain during sexual intercourse, or pelvic pain. Endometrial cancer occurs most commonly after menopause.

<span class="mw-page-title-main">Ovarian cancer</span> Cancer originating in or on the ovary

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 cell types including epithelial cells, germ cells, and stromal cells. When these cells become abnormal, they have the ability to divide and form tumors. These cells can also invade or spread to other parts of the body. When this process begins, there may be no or only vague symptoms. Symptoms become more noticeable as the cancer progresses. These symptoms may include bloating, vaginal bleeding, pelvic pain, abdominal swelling, constipation, and loss of appetite, among others. Common areas to which the cancer may spread include the lining of the abdomen, lymph nodes, lungs, and liver.

<span class="mw-page-title-main">Brenner tumour</span> Medical condition

Brenner tumours are an uncommon subtype of the surface epithelial-stromal tumour group of ovarian neoplasms. The majority are benign, but some can be malignant.

<span class="mw-page-title-main">Ovarian clear-cell carcinoma</span> One of the subtypes of ovarian carcinoma

Ovarian clear-cell carcinoma, or clear-cell carcinoma of the ovary, also called ovarian clear-cell adenocarcinoma, is one of several subtypes of ovarian carcinoma – a subtype of epithelial ovarian cancer, in contrast to non-epithelial cancers. According to research, most ovarian cancers start at the epithelial layer which is the lining of the ovary. Within this epithelial group ovarian clear-cell carcinoma makes up 5–10%.

<span class="mw-page-title-main">Endometrioid tumor</span> Medical condition

Endometrioid tumors are a class of tumors that arise in the uterus or ovaries that resemble endometrial glands on histology. They account for 80% of endometrial carcinomas and 20% of malignant ovarian tumors.

<span class="mw-page-title-main">Ovarian mucinous tumor</span> Type of tumor

Mucinous tumors are a type of ovarian tumor. They are typically large.

<span class="mw-page-title-main">Serous tumour</span> Medical condition

A serous tumour is a neoplasm that typically has papillary to solid formations of tumor cells with crowded nuclei, and which typically arises on the modified Müllerian-derived serous membranes that surround the ovaries in females. Such ovarian tumors are part of the surface epithelial-stromal tumour group of ovarian tumors. They are common neoplasms with a strong tendency to occur bilaterally, and they account for approximately a quarter of all ovarian tumors.

<span class="mw-page-title-main">Germ cell tumor</span> Medical condition

Germ cell tumor (GCT) is a neoplasm derived from the primordial germ cells. Germ-cell tumors can be cancerous or benign. Germ cells normally occur inside the gonads. GCTs that originate outside the gonads may be birth defects resulting from errors during development of the embryo.

The International Classification of Diseases for Oncology (ICD-O) is a domain-specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. This classification is widely used by cancer registries.

<span class="mw-page-title-main">Ovarian tumor</span> Benign, borderline, or malignant neoplasm involving the ovary

Ovarian tumors, or ovarian neoplasms, are tumors in the ovary. Not all are ovarian cancer. They consist of mainly solid tissue, while ovarian cysts contain fluid.

<span class="mw-page-title-main">Adnexal mass</span> Medical condition

An adnexal mass is a lump in the tissue of the adnexa of the uterus. Adnexal masses can be benign (noncancerous) or malignant (cancerous), and they can be categorized as simple or complex.

<span class="mw-page-title-main">Mucinous neoplasm</span> Medical condition

A mucinous neoplasm is an abnormal and excessive growth of tissue (neoplasia) with associated mucin. It arises from epithelial cells that line certain internal organs and skin, and produce mucin. A malignant mucinous neoplasm is called a mucinous carcinoma. For example, for ovarian mucinous tumors, approximately 75% are benign, 10% are borderline and 15% are malignant.

<span class="mw-page-title-main">Fallopian tube cancer</span> Medical condition

Primary fallopian tube cancer (PFTC), also known as tubal cancer, is a malignant neoplasm that originates from the fallopian tube. Along with primary ovarian and peritoneal carcinomas, it is grouped under epithelial ovarian cancers; cancers of the ovary that originate from a fallopian tube precursor.

<span class="mw-page-title-main">Mucinous cystadenoma</span> Medical condition

Mucinous cystadenoma is a benign cystic tumor lined by a mucinous epithelium. It is a type of cystic adenoma (cystadenoma).

A borderline tumor, sometimes called low malignant potential (LMP) tumor, is a distinct but yet heterogeneous group of tumors defined by their histopathology as atypical epithelial proliferation without stromal invasion. It generally refers to such tumors in the ovary but borderline tumors may rarely occur at other locations as well.

<span class="mw-page-title-main">Ovarian serous cystadenoma</span> Medical condition

Ovarian serous cystadenoma is a non-cancerous type of tumor of the ovary. It is typically larger than 1cm in diameter and presents with signs and symptoms of a growth in the pelvis, or is discovered when investigating something else. A fifth occur in both ovaries at the same time.

Ovarian cystadenoma is a cystic benign tumor of the ovary. Two types are recognized: serous and mucinous. Ovarian cystadenomas are common benign epithelial neoplasms that carry an excellent prognosis.

<span class="mw-page-title-main">Ovarian squamous cell carcinoma</span> Medical condition

Ovarian squamous cell carcinoma (oSCC) or squamous ovarian carcinoma (SOC) is a rare tumor that accounts for 1% of ovarian cancers. Included in the World Health Organization's classification of ovarian cancer, it mainly affects women above 45 years of age. Survival depends on how advanced the disease is and how different or similar the individual cancer cells are.

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