Ovarian germ cell tumors (OGCTs) are heterogeneous tumors that are derived from the primitive germ cells of the embryonic

gonad A gonad, sex gland, or reproductive gland is a mixed gland that produces the gametes and sex hormones of an organism. Female reproductive cells are egg cells, and male reproductive cells are sperm. The male gonad, the testicle, produces sper ...

, which accounts for about 2.6% of all ovarian malignancies. There are four main types of OGCTs, namely

dysgerminoma A dysgerminoma is a type of germ cell tumor; it usually is malignant and usually occurs in the ovary. A tumor of the identical histology but not occurring in the ovary may be described by an alternate name: seminoma in the testis or germinoma ...

yolk sac tumor Endodermal sinus tumor (EST) is a member of the germ cell tumor group of cancers. It is the most common testicular tumor in children under three, and is also known as infantile embryonal carcinoma. This age group has a very good prognosis. In contr ...

teratoma A teratoma is a tumor made up of several different types of tissue, such as hair, muscle, teeth, or bone. Teratomata typically form in the ovary, testicle, or coccyx. Symptoms Symptoms may be minimal if the tumor is small. A testicular terato ...

, and

choriocarcinoma Choriocarcinoma is a malignant, trophoblastic cancer, usually of the placenta. It is characterized by early hematogenous spread to the lungs. It belongs to the malignant end of the spectrum in gestational trophoblastic disease (GTD). It is also cl ...

. Dygerminomas are Malignant germ cell tumor of ovary and particularly prominent in patients diagnosed with

gonadal dysgenesis Gonadal dysgenesis is classified as any congenital developmental disorder of the reproductive system in the male or female. It is the atypical development of the gonads in an embryo, with reproductive tissue replaced with functionless, fibrous ti ...

. OGCTs are relatively difficult to detect and diagnose at an early stage because of the nonspecific histological characteristics. Common symptoms of OGCT are

bloating Abdominal bloating (or simply bloating) is a short-term disease that affects the gastrointestinal tract. Bloating is generally characterized by an excess buildup of gas, air or fluids in the stomach. A person may have feelings of tightness, press ...

abdominal distention Abdominal distension occurs when substances, such as air (gas) or fluid, accumulate in the abdomen causing its expansion. It is typically a symptom of an underlying disease or dysfunction in the body, rather than an illness in its own right. Pe ...

ascites Ascites is the abnormal build-up of fluid in the abdomen. Technically, it is more than 25 ml of fluid in the peritoneal cavity, although volumes greater than one liter may occur. Symptoms may include increased abdominal size, increased weight, ab ...

, and

dyspareunia Dyspareunia ( ) is painful sexual intercourse due to medical or psychological causes. The term ''dyspareunia'' covers both female dyspareunia and male dyspareunia, but many discussions that use the term without further specification concern the f ...

. OGCT is caused mainly due to the formation of malignant cancer cells in the primordial germ cells of the ovary. The exact pathogenesis of OGCTs is still unknown however, various genetic mutations and environmental factors have been identified. OGCTs are commonly found during pregnancy when an adnexal mass is found during a pelvic examination, ultrasound scans show a solid mass in ovary or blood serum test shows elevated

alpha-fetoprotein Alpha-fetoprotein (AFP, α-fetoprotein; also sometimes called alpha-1-fetoprotein, alpha-fetoglobulin, or alpha fetal protein) is a protein that in humans is encoded by the ''AFP'' gene. The ''AFP'' gene is located on the ''q'' arm of chromosome 4 ...

levels. They are unlikely to have

metastasized 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, ...

and therefore the standard tumor management is

surgical resection Segmental resection (or segmentectomy) is a surgical procedure to remove part of an organ or gland, as a sub-type of a resection, which might involve removing the whole body part. It may also be used to remove a tumor and normal tissue around i ...

, coupled with

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. Chemotherap ...

. The occurrence rate is less than 3% worldwide.

Classification

OGCTs can be classified into dysgerminoma, teratomas, yolk sac tumors, and choriocarcinomas, listed in the order of prevalence.

Dysgerminoma

Dysgerminomas are comparable to testicular seminomas and account for approximately 32- 37% of all OGCTs. They are particularly prominent in individuals with dysgenic gonads of 46, XY pure gonadal dysgenesis patients. Based on gross examinations, dysgerminomas are characterized by having a ‘solid,

lobulated A lobulation is an appearance resembling lobules. For instance, the thyroid gland may become large and lobulated in Hashimoto's thyroiditis. Microscopically, the cellular structure is distinguished by a round-ovoid shape containing ample

eosinophilic Eosinophilic (Greek suffix -phil-, meaning ''loves eosin'') is the staining of tissues, cells, or organelles after they have been washed with eosin, a dye. Eosin is an acidic dye for staining cell cytoplasm, collagen, and muscle fibers. ''Eosi ...

cytoplasm and an irregularly shaped nuclei. The uniformly positioned cells are separated through the fibrous strands and lymphocytic infiltration is commonly observed.

Teratomas

Teratoma are most common germ cell tumor of ovary. Teratomas can be divided into two types: mature teratoma (benign) and immature teratoma (malignant). Immature teratomas contain immature or

embryonic Embryonic may refer to: *Of or relating to an embryo * ''Embryonic'' (album), a 2009 studio album by the Flaming Lips *''Embryonics ''Embryonics'' is a double-CD compilation of tracks by the Australian progressive metal band, Alchemist. It w ...

tissue which significantly differentiates them from mature teratomas as they carry

dermoid cyst A dermoid cyst is a teratoma of a cystic nature that contains an array of developmentally mature, solid tissues. It frequently consists of skin, hair follicles, and sweat glands, while other commonly found components include clumps of long hair ...

s. It is commonly observed in 15 to 19-year-old women and rarely in women after menopause. Immature teratomas are characterized with a diameter of 14–25 cm, encapsulated mass, cystic areas, and occasional appearance of

hemorrhagic Bleeding, hemorrhage, haemorrhage or blood loss, is blood escaping from the circulatory system from damaged blood vessels. Bleeding can occur internally, or externally either through a natural opening such as the mouth, nose, ear, urethra, vagi ...

areas. The stage of immature teratomas is determined depending on the amount of immature

neuroepithelium Neuroepithelial cells, or neuroectodermal cells, form the wall of the closed neural tube in early embryonic development. The neuroepithelial cells span the thickness of the tube's wall, connecting with the pial surface and with the ventricular or ...

tissue detected.

Yolk sac tumor

The ovarian

s, also known as

endodermal sinus tumor Endodermal sinus tumor (EST) is a member of the germ cell tumor group of cancers. It is the most common testicular tumor in children under three, and is also known as infantile embryonal carcinoma. This age group has a very good prognosis. In contr ...

s, are accountable for approximately 15.5% of all OGCTs. They have been observed in women particularly in their early ages, and rarely after 40 years of age. The critical pathologic features are a smooth external surface and capsular tears due to their rapid rate of growth. A study consisting of 71 individual cases of ovarian yolk sac tumor provides evidence to the proliferation of the tumor. In one of the cases, the pelvic examination revealed normal activity until a 9 cm and 12 cm sized tumor was discovered 4 weeks later. In another case, a 23 cm tumor was discovered in a pregnant woman who was monitored regularly and had normal findings until

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 to ...

became essential. Histologically, these tumors are characterized by mixed solid and

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

ic components. The mixed solid components are characterized by a soft gray to yellow solid components accompanied with significant

hemorrhage Bleeding, hemorrhage, haemorrhage or blood loss, is blood escaping from the circulatory system from damaged blood vessels. Bleeding can occur internally, or externally either through a natural opening such as the mouth, nose, ear, urethra, vag ...

and

necrosis Necrosis () is a form of cell injury which results in the premature death of cells in living tissue by autolysis. Necrosis is caused by factors external to the cell or tissue, such as infection, or trauma which result in the unregulated dige ...

. The cysts are approximately 2 cm in diameter and populated throughout the tissue which results in giving the

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 ...

a ‘honeycombed appearance’.

Choriocarcinoma

Choriocarcinomas are exceptionally rare which account for 2.1%-3.4% of all OGCTs. Under gross examination, the

syncytiotrophoblast Syncytiotrophoblast (from the Greek 'syn'- "together"; 'cytio'- "of cells"; 'tropho'- "nutrition"; 'blast'- "bud") is the epithelial covering of the highly vascular embryonic placental villi, which invades the wall of the uterus to establish nut ...

cells are aligned in a plexiform arrangement with the mononucleated

cytotrophoblast "Cytotrophoblast" is the name given to both the inner layer of the trophoblast (also called layer of Langhans) or the cells that live there. It is interior to the syncytiotrophoblast and external to the wall of the blastocyst in a developing embryo ...

cells surrounding the foci of the hemorrhage. Choriocarcinomas can be divided into gestational choriocarcinomas and non-gestational choriocarcinomas which have

immunohistochemical Immunohistochemistry (IHC) is the most common application of immunostaining. It involves the process of selectively identifying antigens (proteins) in cells of a tissue section by exploiting the principle of antibodies binding specifically to ant ...

differences.

Signs and Symptoms

OGCTs are relatively difficult to detect and diagnose at an early stage mainly because the symptoms are normally subtle and nonspecific. They become detectable when as they become large, tangible masses. Symptoms include bloating, abdominal distention, ascites, and dyspareunia. In rare cases where the tumor ruptures, acute

abdominal pain Abdominal pain, also known as a stomach ache, is a symptom Signs and symptoms are the observed or detectable signs, and experienced symptoms of an illness, injury, or condition. A sign for example may be a higher or lower temperature than ...

can be experienced. The critical indicator of malignancy is usually the appearance of the Sister Mary Joseph Nodule. OGCTs can further give rise to

ovarian torsion Ovarian torsion (OT) or adnexal torsion is an abnormal condition where an ovary twists on its attachment to other structures, such that blood flow is decreased. Symptoms typically include pelvic pain on one side. While classically the pain is su ...

, hemorrhage, and even isosexual precocious puberty in young children.

Causes

The exact cause of OGCT is yet to be determined. However, a few factors have been identified which may contribute to increased risk of OGCTs including

endometriosis Endometriosis is a disease of the female reproductive system in which cells similar to those in the endometrium, the layer of tissue that normally covers the inside of the uterus, grow outside the uterus. Most often this is on the ovaries, f ...

polycystic ovarian syndrome Polycystic ovary syndrome, or PCOS, is the most common endocrine disorder in women of reproductive age. The syndrome is named after the characteristic cysts which may form on the ovaries, though it is important to note that this is a sign and no ...

, and genetic risk factors. Individuals who are more prone to developing OGCTs usually contain the

autosomal dominant In genetics, dominance is the phenomenon of one variant (allele) of a gene on a chromosome masking or overriding the effect of a different variant of the same gene on the other copy of the chromosome. The first variant is termed dominant and t ...

, BRCA-1/ BRCA-2, mutations. Complications with other cancers such as

hereditary nonpolyposis colorectal cancer Hereditary nonpolyposis colorectal cancer (HNPCC) or Lynch syndrome is an autosomal dominant genetic condition that is associated with a high risk of colon cancer as well as other cancers including endometrial cancer (second most common), ovary, ...

, also known as the

Lynch syndrome Hereditary nonpolyposis colorectal cancer (HNPCC) or Lynch syndrome is an autosomal dominant genetic condition that is associated with a high risk of colon cancer as well as other cancers including endometrial cancer (second most common), ovary, ...

, increases the risk of developing

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 ...

. Pregnancy, breastfeeding, and oral

contraceptives Birth control, also known as contraception, anticonception, and fertility control, is the use of methods or devices to prevent unwanted pregnancy. Birth control has been used since ancient times, but effective and safe methods of birth contr ...

are known to have reduced risk for OGCTs. The

etiology Etiology (pronounced ; alternatively: aetiology or ætiology) is the study of causation or origination. The word is derived from the Greek (''aitiología'') "giving a reason for" (, ''aitía'', "cause"); and ('' -logía''). More completely, e ...

of OGCT is still under study, however, genetic alterations may contribute to development of OGCTs, such as the classical

tumor suppressor genes A tumor suppressor gene (TSG), or anti-oncogene, is a gene that regulates a cell during cell division and replication. If the cell grows uncontrollably, it will result in cancer. When a tumor suppressor gene is mutated, it results in a loss or red ...

and

oncogene An oncogene is a gene that has the potential to cause cancer. In tumor cells, these genes are often mutated, or expressed at high levels.

s. Along with genetic modifications, certain environmental factors such as

endocrine disruptors Endocrine disruptors, sometimes also referred to as hormonally active agents, endocrine disrupting chemicals, or endocrine disrupting compounds are chemicals that can interfere with endocrine (or hormonal) systems. These disruptions can cause ca ...

, presence of a daily routine that affects the individual's biochemistry, and exposure to maternal hormones could also contribute to the proliferation of OGCT. A recent study on rats showed the

transgenerational epigenetic inheritance Transgenerational epigenetic inheritance is the transmission of epigenetic markers from one organism to the next (i.e., from parent to child) that affects the traits of offspring without altering the primary structure of DNA (i.e. the sequence of ...

supporting the influence of hazardous environmental substances, including plastics, pesticides, and

dioxins Dioxin may refer to: * 1,2-Dioxin or 1,4-Dioxin, two unsaturated heterocyclic 6-membered rings where two carbon atoms have been replaced by oxygen atoms, giving the molecular formula C4H4O2 *Dibenzo-1,4-dioxin, the parent compound also known as ...

, on the pathogenesis of OGCT.

Pathogenesis

Nonetheless, a few speculations for the causes have been made. During

ovulation Ovulation is the release of eggs from the ovaries. In women, this event occurs when the ovarian follicles rupture and release the secondary oocyte ovarian cells. After ovulation, during the luteal phase, the egg will be available to be fertilized ...

, the follicle ruptures resulting in

epithelial cell 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 intercellula ...

damage. In order to heal the tissue and replace the damage, the cells undergoes

cell division Cell division is the process by which a parent cell (biology), cell divides into two daughter cells. Cell division usually occurs as part of a larger cell cycle in which the cell grows and replicates its chromosome(s) before dividing. In eukar ...

. Each time the cell divides, there is a possibility for mutations to occur and the chance of tumor formation increases. The tumor is caused when the germ cells in the ovaries begin divide uncontrollably and become malignant which are characterized with their less organized nuclei and unclearly defined border. Another potential etiology is the dysfunctioning of the

tumor suppressor gene A tumor suppressor gene (TSG), or anti-oncogene, is a gene that regulates a cell during cell division and replication. If the cell grows uncontrollably, it will result in cancer. When a tumor suppressor gene is mutated, it results in a loss or red ...

, TRC8/RNF139, or even

karyotypic A karyotype is the general appearance of the complete set of metaphase chromosomes in the cells of a species or in an individual organism, mainly including their sizes, numbers, and shapes. Karyotyping is the process by which a karyotype is disce ...

abnormalities after close molecular examination. OGCT has its roots in embryonic development where the

primordial germ cells Primordial may refer to: * Primordial era, an era after the Big Bang. See Chronology of the universe * Primordial sea (a.k.a. primordial ocean, ooze or soup). See Abiogenesis * Primordial nuclide, nuclides, a few radioactive, that formed before ...

(PGCs) are isolated in early stages and have the ability to alter the genome as well as the

transcriptome The transcriptome is the set of all RNA transcripts, including coding and non-coding, in an individual or a population of cells. The term can also sometimes be used to refer to all RNAs, or just mRNA, depending on the particular experiment. The t ...

. OGCTs can be attributed to the internal mechanism of PGCs and their transforming characteristics.

Staging

After OGCT has been diagnosed, various tests will be conducted to determine whether the cancer has spread to other areas in the body. The spread of OGCT is identified through different stages: stage I, stage II, stage III, and stage IV namely. Stage I: Tumor cells are localized in the ovaries or the fallopian tubes without extensive spread to other body regions. Stage II: The cancer is in one or both ovaries or fallopian tubes and has spread into the pelvis. Stage III: The cancer has spread beyond the pelvis into the abdomen and to the retroperitoneal lymph nodes (located at the back of the

abdomen 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. ...

). The substages are characterized by the relative size of the tumor. Note: Stage II ovarian cancer will also be declared if the cancerous cells have spread to the

liver The liver is a major Organ (anatomy), organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the Protein biosynthesis, synthesis of proteins and biochemicals necessary for ...

. Stage IV: The cancer has spread outside of the abdomen and

pelvis The pelvis (plural pelves or pelvises) is the lower part of the trunk, between the abdomen and the thighs (sometimes also called pelvic region), together with its embedded skeleton (sometimes also called bony pelvis, or pelvic skeleton). The ...

to more distant organs, such as the

lung The lungs are the primary organs of the respiratory system in humans and most other animals, including some snails and a small number of fish. In mammals and most other vertebrates, two lungs are located near the backbone on either side of t ...

Diagnosis

The preliminary diagnosis begins with a

pelvic examination A pelvic examination is the physical examination of the external and internal female pelvic organs. It is frequently used in gynecology for the evaluation of symptoms affecting the female reproductive and urinary tract, such as pain, bleeding, dis ...

, serum tumor marker test and imaging. Physicians may feel a large palpable mass or lump in lower abdomen upon insertion of the gloved fingers into the vagina. To further identify the histologic subtypes of OGMTs, blood samples of patients are collected to analyse the serum level of biomarkers released by the tumor cells. A surge in the plasma levels of

human chorionic gonadotropin Human chorionic gonadotropin (hCG) is a hormone for the maternal recognition of pregnancy produced by trophoblast cells that are surrounding a growing embryo (syncytiotrophoblast initially), which eventually forms the placenta after implantation ...

and alpha-fetoprotein is indicative of OGMTs.

Lactate dehydrogenase Lactate dehydrogenase (LDH or LD) is an enzyme found in nearly all living cells. LDH catalyzes the conversion of lactate to pyruvate and back, as it converts NAD+ to NADH and back. A dehydrogenase is an enzyme that transfers a hydride from on ...

alkaline phosphatase The enzyme alkaline phosphatase (EC 3.1.3.1, alkaline phosphomonoesterase; phosphomonoesterase; glycerophosphatase; alkaline phosphohydrolase; alkaline phenyl phosphatase; orthophosphoric-monoester phosphohydrolase (alkaline optimum), systematic ...

and cancer antigen 125 might potentially increase as well. To visualize the location and morphology of the tumor,

transvaginal ultrasonography Vaginal ultrasonography is a medical ultrasonography that applies an ultrasound transducer (or "probe") in the vagina to visualize organs within the pelvic cavity. It is also called transvaginal ultrasonography because the ultrasound waves go '' ...

is usually employed. The most characteristic appearance is a parenchymal-like heteroechoic mass with sharp borders and high vascularization. Computed tomography would produce stacked image inside the peritoneal region of the body to visualise the lobular pattern of the tumour. Usually for dysgerminoma, solid mass being compartmentalized into lobules with enhancing septa may be evident for

haemorrhage Bleeding, hemorrhage, haemorrhage or blood loss, is blood escaping from the circulatory system from damaged blood vessels. Bleeding can occur internally, or externally either through a natural opening such as the mouth, nose, ear, urethra, vagi ...

or necrosis.

Preoperative procedures

In accordance with FIGO staging guidelines, comprehensive surgical staging will be conducted to examine the extent of tumor spread via

peritoneal 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 mesothe ...

regions or lymph drainages. 28% of stage II patients will be found with the development of secondary malignant growths at lymph nodes with a distance from a primary site of cancer, called lymph node

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, ...

. There are three major lymphatic drainage pathways: # drainage to the

paraaortic lymph nodes The periaortic lymph nodes (also known as lumbar) are a group of lymph nodes that lie in front of the lumbar vertebrae near the aorta. These lymph nodes receive drainage from the gastrointestinal tract and the abdominal organs. The periaortic lymp ...

via

ovarian vein The ovarian vein, the female gonadal vein, carries deoxygenated blood from its corresponding ovary to inferior vena cava or one of its tributaries. It is the female equivalent of the testicular vein, and is the venous counterpart of the ovarian ...

s # drainage from

broad ligament The broad ligament of the uterus is the wide fold of peritoneum that connects the sides of the uterus to the walls and floor of the pelvis. Structure Subdivisions Contents The contents of the broad ligament include the following: * Reproduc ...

to the iliac lymph nodes # drainage from round ligament to the

inguinal lymph nodes Inguinal lymph nodes are lymph nodes in the human groin. Located in the femoral triangle of the inguinal region, they are grouped into superficial and deep lymph nodes. The superficial have three divisions: the superomedial, superolateral, and inf ...

Palpation or biopsies of unilateral pelvic and para-aortic lymph nodes will be conducted as a preoperative step to deduce the

prognosis Prognosis (Greek: πρόγνωσις "fore-knowing, foreseeing") is a medical term for predicting the likely or expected development of a disease, including whether the signs and symptoms will improve or worsen (and how quickly) or remain stabl ...

of the tumour and lymphatic spread Peritoneal biopsies and

omentectomy The greater omentum (also the great omentum, omentum majus, gastrocolic omentum, epiploon, or, especially in animals, caul) is a large apron-like fold of Peritoneum#Layers, visceral peritoneum that hangs down from the stomach. It extends from the ...

will also be employed to evaluate the extent of tumour content spillage or implantation in peritoneal cavity. Tumor cells may shed off from the original site into the peritoneal cavity and implant onto the liver capsule surface or diaphragm. They may clog up inside the lymphatic vessel around the diaphragm and prevent resorption of peritoneal fluid. In the end, pericardiophrenic

lymphadenopathy Lymphadenopathy or adenopathy is a disease of the lymph nodes, in which they are abnormal in size or consistency. Lymphadenopathy of an inflammatory type (the most common type) is lymphadenitis, producing swollen or enlarged lymph nodes. In cli ...

and ascites may result from this frank invasion.

Treatment

Surgery

Malignant OGCTs are predominantly unilateral and chemosensitive, which means they are localized in only one side of the ovary. Fertility-preserving surgery is primarily standardized to keep the contralateral ovary and fallopian tube intact, also known as unilateral salpingo-oophorectomy. For Stage II patients with observable metastasis,

cytoreductive surgery Cytoreductive surgery (CRS) is a surgical procedure that aims to reduce the amount of cancer cells in the abdominal cavity for patients with tumors that have spread intraabdominally (peritoneal carcinomatosis). It is often used to treat ovarian can ...

may be performed to debulk the volume of the tumor, such as

hysterectomy Hysterectomy is the surgical removal of the uterus. It may also involve removal of the cervix, ovaries (oophorectomy), Fallopian tubes (salpingectomy), and other surrounding structures. Usually performed by a gynecologist, a hysterectomy may b ...

(removal of all or part of the uterus) and bilateral salpingo-oophorectomy. A surgical incision at the abdominal cavity after the completion of adjuvant chemotherapy, called second look

laparotomy A laparotomy is a surgical procedure involving a surgical incision through the abdominal wall to gain access into the abdominal cavity. It is also known as a celiotomy. Origins and history The first successful laparotomy was performed without ane ...

, is best applicable for patients reported with teratomatous elements after previous cytoreductive surgery.

Adjuvant Chemotherapy

With a recurrence up to 15-25% for early-stage patients,

adjuvant chemotherapy Adjuvant therapy, also known as adjunct therapy, adjuvant care, or augmentation therapy, is a therapy that is given in addition to the primary or initial therapy to maximize its effectiveness. The surgeries and complex treatment regimens used in ...

needs to couple with surgical resection of tumor to ensure full salvage. For systemic chemotherapy (issued orally or intravenously), the regimen is standardised in every FIGO staging to comprise

bleomycin -13- (1''H''-imidazol-5-yl)methyl9-hydroxy-5- 1''R'')-1-hydroxyethyl8,10-dimethyl-4,7,12,15-tetraoxo-3,6,11,14-tetraazapentadec-1-yl}-2,4'-bi-1,3-thiazol-4-yl)carbonyl]amino}propyl)(dimethyl)sulfonium , chemical_formula = , C=55 , H=84 , N=1 ...

etoposide Etoposide, sold under the brand name Vepesid among others, is a chemotherapy medication used for the treatments of a number of types of cancer including testicular cancer, lung cancer, lymphoma, leukemia, neuroblastoma, and ovarian cancer. It is ...

, and

cisplatin Cisplatin is a chemotherapy medication used to treat a number of cancers. These include testicular cancer, ovarian cancer, cervical cancer, breast cancer, bladder cancer, head and neck cancer, esophageal cancer, lung cancer, mesothelioma, br ...

, also known as the BEP treatment. Patients should be issued with 3-4 cycles of BEP to ensure full salvage. Depending on the personalised conditions, some patients who are non-responders to BEP therapy will be prescribed with salvage therapy, which consists of cisplatin,

ifosfamide Ifosfamide (IFO), sold under the brand name Ifex among others, is a chemotherapy medication used to treat a number of types of cancer. This includes testicular cancer, soft tissue sarcoma, osteosarcoma, bladder cancer, small cell lung cancer, ...

and

paclitaxel Paclitaxel (PTX), sold under the brand name Taxol among others, is a chemotherapy medication used to treat a number of types of cancer. This includes ovarian cancer, esophageal cancer, breast cancer, lung cancer, Kaposi's sarcoma, cervical cancer ...

. Yet, it is likely that OGCT survivors after BEP therapy will have

premature menopause Primary ovarian insufficiency (POI) (also called premature ovarian insufficiency, premature menopause, and premature ovarian failure) is the partial or total loss of reproductive and hormonal function of the ovaries before age 40 because of fol ...

at a rough age of 36. Alternatively, some hospitals opted for platinum-based chemotherapy since the platinum complexes present in the drug intervenes

DNA transcription Transcription is the process of copying a segment of DNA into RNA. The segments of DNA transcribed into RNA molecules that can encode proteins are said to produce messenger RNA (mRNA). Other segments of DNA are copied into RNA molecules called ...

by forming chemical cross-links within the DNA strands, which prevents the reproduction of cancerous cells. The major elements are cisplatin,

carboplatin Carboplatin, sold under the trade name Paraplatin among others, is a chemotherapy medication used to treat a number of forms of cancer. This includes ovarian cancer, lung cancer, head and neck cancer, brain cancer, and neuroblastoma. It is used b ...

, and

oxaliplatin Oxaliplatin, sold under the brand name Eloxatin among others, is a cancer medication (platinum-based antineoplastic class) used to treat colorectal cancer. It is given by injection into a vein. Common side effects include numbness, feeling ti ...

. It has been reported with full recovery among early-stage patients and only a quarter of advanced-stage patients are not salvaged potentially due to drug resistance. For advanced-stage patients, after cytoreductive surgery, invisible microscopic cancerous cells or nodules may still be present at the site of infection. Therefore, doctors may instill a heated chemotherapy solution (~42-43 °C) into the abdominal cavity through carters tubes for 1.5 hours. Based on the principle that cancer cells normally dies at 40 °C,

somatic cell A somatic cell (from Ancient Greek σῶμα ''sôma'', meaning "body"), or vegetal cell, is any biological cell forming the body of a multicellular organism other than a gamete, germ cell, gametocyte or undifferentiated stem cell. Such cells compo ...

s remains unaffected since they die at 44 °C. This novel method is proven effective with only 10% recurrence rate and no mortality recorded. It is known as the hyperthermic intraperitoneal chemotherapy (HIPEC), containing

docetaxel Docetaxel (DTX or DXL), sold under the brand name Taxotere among others, is a chemotherapy medication used to treat a number of types of cancer. This includes breast cancer, head and neck cancer, stomach cancer, prostate cancer and non-small-ce ...

, and cisplatin. Given the drug is disseminated locally in intraperitoneal regions, it has no systemic side effects on other actively reproducing cells and is preferred over systemic chemotherapy. Typically, uncontrollable drug distribution in systemic chemotherapy results in

myelosuppression Bone marrow suppression also known as myelotoxicity or myelosuppression, is the decrease in production of cells responsible for providing immunity (leukocytes), carrying oxygen (erythrocytes), and/or those responsible for normal blood clotting (t ...

, specifically with observed

febrile neutropenia Febrile neutropenia is the development of fever, often with other signs of infection, in a patient with neutropenia, an abnormally low number of neutrophil granulocytes (a type of white blood cell) in the blood. The term neutropenic sepsis is also ...

neurotoxicity Neurotoxicity is a form of toxicity in which a biological, chemical, or physical agent produces an adverse effect on the structure or function of the central and/or peripheral nervous system. It occurs when exposure to a substance – specificall ...

ototoxicity Ototoxicity is the property of being toxic to the ear (''oto-''), specifically the cochlea or auditory nerve and sometimes the vestibular system, for example, as a side effect of a drug. The effects of ototoxicity can be reversible and temporary, ...

, and

nephrotoxicity Nephrotoxicity is toxicity in the kidneys. It is a poisonous effect of some substances, both toxic chemicals and medications, on kidney function. There are various forms, and some drugs may affect kidney function in more than one way. Nephrotoxin ...

. Remedial treatments to deal with chemotherapy-induced toxicities is through injection of

granulocyte colony-stimulating factor Granulocyte colony-stimulating factor (G-CSF or GCSF), also known as colony-stimulating factor 3 (CSF 3), is a glycoprotein that stimulates the bone marrow to produce granulocytes and stem cells and release them into the bloodstream. Functional ...

or myeloid growth factors or oral intake of prophylactic antibiotics.

Epidemiology

OGCT is a rare tumour under the scope of ovarian cancer, accounting for less than 5% of all ovarian malignancies. It occurs mostly in 15-19-year-old women and shows 75% incidence rate for women aged <30 years. In 2011, the number of new cases occurred worldwide is 5.3 per million. In most countries, the occurrence rate on average is less than 3% of the population. However, Asia has reported the highest proportion of cases up to 4.3% due to the younger age profile of the population. For other regions, the incidence rates reported are 2.5% in Oceania, 2.0% in North America and 1.3% in Europe. The five-year survival rates have reached up to 90-92%, which is much higher than that of

epithelial ovarian cancer 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 Fallopi ...

s. The main reason is the high effectiveness of

platinum-based chemotherapy Platinum-based antineoplastic drugs (informally called platins) are chemotherapeutic agents used to treat cancer. They are coordination complexes of platinum. These drugs are used to treat almost half of people receiving chemotherapy for cancer. I ...

References

External links

{{Medical resources , ICD10 = {{ICD10, C56, {{ICD10, D27 , ICD10CM = , ICD9 = , ICDO = , OMIM = , DiseasesDB = , MedlinePlus = , eMedicineSubj = , eMedicineTopic = , MeshID = , GeneReviewsNBK = , GeneReviewsName = , NORD = , GARDNum = , GARDName = , Orphanet = , AO = , RP = , WO = , OrthoInfo = , NCI = , Scholia = , SNOMED CT = Ovarian cancer