Ovarian cancer is a heterogeneous (diverse) group of diseases. The most common type of ovarian cancer, epithelial ovarian cancer, tends to occur in women over the age of 50. This form of ovarian cancer the most publicized with the diagnosis. It is this form of cancer that most people
think of when they hear the term "ovarian cancer". However, there are two
other forms of ovarian cancer: germ cell tumors and stromal tumors
Ovarian cancers are divided into type 1 cancers and a more aggressive
type 2 variant. Type 1 tumors are characterized by low-grade histology and
more indolent behavior. These tumors include low- malignant potential
tumors, low-grade endometrial and mucinous histologies, and clear cell
tumors. Genetic alterations commonly include mutations in KRAS, BRAF, PTEN
and PIK3CA. In contrast, studies have implicated serial genetic changes in
the fallopian tubes as the actual site of origin for most type 2 serous
epithelial ovarian tumors. These aggressive tumors are more common and are
related to losses in TP53 and DNA repair capacity
Carcinoma in situ has been identified in tubal epithelium with early
losses in the TP53 and BRCA1 / BRCA2 genes that characterize early tubal
intraepithelial tumors. After these first two genetic events, further
mutations in these transformed cells lead to tumor cell shedding,
metastasis and invasion of cancer cells. These poorly differentiated type
2 ovarian cancer cells can then spread to the ovaries and peritoneal
cavity, with the help of the ovarian cancer cells' affinity for the cells
of the mesothelial lining
Epithelial Tumors
Serous cystadenomas Serous and mucinous cystadenomas are the most common
benign epithelial ovarian neoplasms, originating from the reproductive
cells of the ovaries. Most epithelial ovarian tumors are benign. However,
cancerous epithelial tumors (also known as ovarian epithelial
carcinomas)
The classification of common epithelial tumors was developed by the World
Health Organization and the International Federation of Gynecology and
Obstetrics. The nomenclature takes into account cell type, tumor location
and degree of malignancy, ranging from benign tumors to low-grade tumors and
invasive carcinomas. Low-grade epithelial tumors ("borderline malignancy")
have a much better prognosis than invasive carcinomas and are characterized
by epithelial papillae with atypical cell clusters, cellular stratification,
nuclear atypia and increased mitotic activity. Malignant tumors are
characterized by an infiltrating destructive growth pattern with malignant
cells that grow disorganized and dissect into the stromal planes. Invasive
epithelial carcinomas are characterized by histologic type and grade (degree
of cell differentiation)
Serous carcinoma
Serous carcinoma is the most common type of epithelial ovarian cancer,
accounting for over 50% of cases. The maximum age range is 45 to 65 years.
Typical serous carcinomas show complex solid and papillary solid patterns
and qualify as high-grade carcinomas
Endometrioid
Endometrioid carcinomas are seen in women between the ages of 40 and 50
and comprise approximately 10% -15% of ovarian cancers, of which
approximately 6% are surface epithelial neoplasms. Endometrioid tumors are
generally diagnosed as stage I disease and have a better prognosis than
tumors with serous histology
Mucinous carcinomas
Mucinous carcinomas occur in women between the ages of 40 and 70 and
account for approximately 12% of all ovarian cancers. The overall
prognosis for mucinous carcinoma is better than for serous carcinoma
because most patients have stage 1 disease
Clear cell carcinoma
Clear cell carcinoma comprises approximately 3% of epithelial ovarian
cancers and approximately 8-10% of ovarian cancers in women, with a mean
age of 57 years. Although clear cell carcinoma is the least common
ovarian neoplasm, it is most often associated with
paraneoplastic-related hypercalcemia
Germ Cell Tumors
Germ cell tumors, like their counterparts in the testis, are cancers of
germ cells. These totipotent cells contain programming for the
differentiation of nearly all types of tissue, and hence the germ cell
tumors include a histologic menagerie of bizarre tumors, including benign
teratomas (dermoid cysts) and a variety of malignant tumors, such as
dysgerminoma, immature teratomas, yolk sac malignancies, and
choriocarcinomas. Patients generally complain of abdominal pain and have an
associated pelvic or abdominal mass. Others may have acute abdominal pain as
a result of ovarian rupture, hemorrhage, or torsion
Teratoma
These germ cell tumors are either benign (mature) or cancerous
(immature), and their cells can contain different types of tissue, such as
hair, muscle, and bone. Immature teratomas are rare
• Mature Cystic Teratoma
Mature cystic teratomas, also known as dermoid cysts, are the most
common benign ovarian neoplasms, with a peak incidence between the ages
of 20 and 40. However, they can also be observed in infancy, as well as
in menopausal women. Mature cystic teratomas originate from primordial
germ cells and consist of
well differentiated derivatives of any combination of the three germ
layers: ectoderm, mesoderm, endoderm. Ectodermal elements usually
predominate. Although these mature tissues are benign in the vast
majority of cases, on rare occasions they can undergo a malignant
transformation, with squamous cell carcinoma being the most common
malignant histology
•Immature Cystic Teratoma
Immature cystic teratoma is composed of tissues derived from all three
germ layers, except that they also contain embryonic tissue, like a mature
cystic teratoma. This group of cancers is the third most common germ cell
malignancy, accounting for approximately 25% of all these cancers in
patients under the age of 20. Unlike mature cystic teratoma, which occurs
in all ages but more frequently during the reproductive years, immature
cystic teratoma is found mainly during the first two decades of life. It
usually grows rapidly through its capsule, forming adhesions to
surrounding structures and implants in the peritoneal cavity
Dysgerminoma
Dysgerminoma is the most common germ cell malignancy of the ovary,
accounting for 2% of all ovarian cancers. About 50% of patients with this
cancer are under the age of 20 and 80% are under the age of 30. Children
with dysgerminoma may have early puberty or primary amenorrhea. The serum
level of lactate dehydrogenase is often elevated and can act as a tumor
marker during treatment and follow-up
Endodermal sinus tumor
Endodermal sinus tumor, or yolk sac cancer, is the second most common
germ cell cancer and accounts for 1% of all ovarian cancers. It can be
pure or part of a mixed germ cell malignancy. The reported age
distribution ranges from 16 months to 46 years, but the majority of
patients are under the age of 30. The serum a-fetoprotein (AFP) level is
frequently elevated in these patients, making it a useful diagnostic test
in the initial assessment, assessment of response to treatment and in the
follow-up of relapses. Symptoms are typical of those seen with other germ
cell cancers. There have been several cases during pregnancy. No endocrine
manifestations were observed with the pure Endodermal sinus tumor form.
Over 70% of patients with endodermal sinus tumors are diagnosed with stage
I, although they are biologically virulent
Choriocarcinoma
Choriocarcinoma is a rare, pure or mixed germ cell tumor. Pure
choriocarcinoma is generally found in prepubertal children. Isosexual
precocious puberty is a common clinical finding in premenarchical
patients. In postmenarcheal patients, the presence of other germ cell
components is useful in distinguishing ovarian germ cell tumors from a
gestation choriocarcinoma
Ovarian Stromal Tumors
Ovarian stromal tumors or sex cord tumors are most common in women in their
fifties or sixties, but the tumors can present at any age. About 7% of
ovarian neoplasms are stromal tumors and tend to be diagnosed in stage I.
These tumors are associated with hormonal effects, such as precocious
puberty, amenorrhea and postmenopausal bleeding
These tumors arise from the mesenchymal components of the ovary, including
steroid-producing cells and fibroblasts. Most of these tumors are indolent
tumors with limited metastatic potential and present as unilateral solid
masses. These tumors are mainly discovered by detecting an abdominal mass
sometimes with abdominal pain due to ovarian torsion, intratumoral
hemorrhage, or rupture. Rarely, stromal tumors can produce estrogen and
present with breast tenderness, as well as precocious puberty in
children, menstrual disturbances in reproductively active women, or
postmenopausal bleeding. In some women, estrogen-associated secondary
malignancies, such as endometrial or breast cancer, may present as
synchronous malignancies
Thecoma
Thecoma is a benign tumor that affects all ages, mainly in the
postmenopausal group, it is rare in patients under the age of 35. It
accounts for 2% of all ovarian cancers. Many women with thecoma have
abnormal or postmenopausal uterine bleeding, some have endometrial
adenocarcinoma due to the uncontrolled estrogen produced by the tumor.
Thecoma is composed of lipid-laden stromal cells that resemble theca
cells. Rather than arising as a de novo neoplasm, it may represent changes
occurring in underlying cortical stromal hyperplasia
Fibroma
Like thecoma, fibroma is a benign tumor or that affects all ages,
although most occur in women between the ages of 40 and 60, less than 10%
of patients are 30 or younger. Fibroma is not associated with
hormone production. In some cases, hydrothorax and ascites are associated
with a pelvic mass, a constellation of findings known as Meigs syndrome.
In other cases, fibroma is seen in patients with an inherited basal cell
nevus syndrome, characterized by early-appearing basal cell carcinomas,
keratocysts of the jaw, calcification of the dura, and mesenteric
cysts
Important topics that you may read it
Internal Medicine,
Obstetrics and Gynecology,
Ovarian Cancer
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