Choriocarcinoma is a trophoblastic

cancer Cancer is a group of diseases involving Cell growth#Disorders, abnormal cell growth with the potential to Invasion (cancer), invade or Metastasis, spread to other parts of the body. These contrast with benign tumors, which do not spread. Po ...

usually located on the

placenta The placenta (: placentas or placentae) is a temporary embryonic and later fetal organ that begins developing from the blastocyst shortly after implantation. It plays critical roles in facilitating nutrient, gas, and waste exchange between ...

. 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 classified as a

germ cell tumor A germ cell tumor (GCT) is a neoplasm derived from primordial germ cells. Germ-cell tumors can be cancerous or benign. Germ cell tumors typically originate from the gonads (ovary and testis), but can arise in other areas of the body. Extragon ...

and may arise in the

testis A testicle or testis ( testes) is the gonad in all male bilaterians, including humans, and is Homology (biology), homologous to the ovary in females. Its primary functions are the production of sperm and the secretion of Androgen, androgens, p ...

ovary The ovary () is a gonad in the female reproductive system that produces ova; when released, an ovum travels through the fallopian tube/ oviduct into the uterus. There is an ovary on the left and the right side of the body. The ovaries are end ...

Signs and symptoms

* increased quantitative

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 implantat ...

(the "pregnancy hormone") levels * vaginal bleeding *

shortness of breath Shortness of breath (SOB), known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing discomfort that con ...

hemoptysis Hemoptysis or haemoptysis is the discharge of blood or blood-stained sputum, mucus through the mouth coming from the bronchi, larynx, vertebrate trachea, trachea, or lungs. It does not necessarily involve coughing. In other words, it is the airw ...

(coughing up blood) *

chest pain Chest pain is pain or discomfort in the chest, typically the front of the chest. It may be described as sharp, dull, pressure, heaviness or squeezing. Associated symptoms may include pain in the shoulder, arm, upper abdomen, or jaw, along with n ...

chest X-ray A chest radiograph, chest X-ray (CXR), or chest film is a Projectional radiography, projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures. Chest radiographs are the most common fi ...

shows multiple infiltrates of various shapes in both lungs * presents in males as a

testicular cancer Testicular cancer is cancer that develops in the testicles, a part of the male reproductive system. Symptoms may include a lump in the testicle or swelling or pain in the scrotum. Treatment may result in infertility. Risk factors include an c ...

, sometimes with skin hyperpigmentation (from excess chorionic gonadotropin cross-reacting with the alpha MSH receptor),

gynecomastia Gynecomastia (also spelled gynaecomastia) is the non-cancerous enlargement of one or both breasts in men due to the growth of breast tissue as a result of a hormone imbalance between estrogens and androgens. Updated by Brent Wisse (10 Novemb ...

, and weight loss (from excess chorionic gonadotropin cross-reacting with the LH, FSH, and TSH receptor) in males * can present with decreased thyroid-stimulating hormone (TSH) due to

hyperthyroidism Hyperthyroidism is a endocrine disease in which the thyroid gland produces excessive amounts of thyroid hormones. Thyrotoxicosis is a condition that occurs due to elevated levels of thyroid hormones of any cause and therefore includes hyperth ...

Cause

Choriocarcinoma of the

during pregnancy is preceded by: * hydatidiform mole (50% of cases) * spontaneous abortion (20% of cases) *

ectopic pregnancy Ectopic pregnancy is a complication of pregnancy in which the embryo attaches outside the uterus. Signs and symptoms classically include abdominal pain and vaginal bleeding, but fewer than 50 percent of affected women have both of these sympto ...

(2% of cases) * normal-term pregnancy (20–30% of cases) *

hyperemesis gravidarum Hyperemesis gravidarum (HG) is a pregnancy complication that is characterized by severe nausea, vomiting, weight loss, and possibly dehydration. Feeling faint may also occur. It is considered a more severe form of morning sickness. Symptoms ...

Rarely, choriocarcinoma occurs in primary locations other than the placenta; very rarely, it occurs in the testicles. Although trophoblastic components are common components of mixed germ cell tumors, pure choriocarcinoma of the adult

is rare. Pure choriocarcinoma of the testis represents the most aggressive pathologic variant of germ cell tumors in adults, characteristically with early hematogenous and lymphatic

metastatic Metastasis is a pathogenic agent's spreading 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, ...

spread. Because of early spread and inherent resistance to anticancer drugs, patients have a poor prognosis. Elements of choriocarcinoma in a mixed testicular tumor have no prognostic importance. Choriocarcinomas can also occur in the ovaries and other organs.

Pathology

A characteristic feature is the identification of intimately related

syncytiotrophoblast The 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 ...

s and cytotrophoblasts without the formation of definite placental type villi. Since choriocarcinomas include syncytiotrophoblasts (beta-HCG-producing cells), they cause elevated blood levels of beta-human chorionic gonadotropin. Syncytiotrophoblasts are large, multi-nucleated cells with

eosinophilic Eosinophilic (Greek suffix '' -phil'', meaning ''eosin-loving'') describes the staining of tissues, cells, or organelles after they have been washed with eosin, a dye commonly used in histological staining. Eosin is an acidic dye for stainin ...

cytoplasm The cytoplasm describes all the material within a eukaryotic or prokaryotic cell, enclosed by the cell membrane, including the organelles and excluding the nucleus in eukaryotic cells. The material inside the nucleus of a eukaryotic cell a ...

. They often surround the cytotrophoblasts, reminiscent of their normal anatomical relationship in

chorionic villi Chorionic villi are Wiktionary:villus, villi that sprout from the chorion to provide maximal contact area with maternal blood. They are an essential element in pregnancy from a histology, histomorphologic perspective, and are, by definition, a pr ...

. Cytotrophoblasts are polyhedral, mononuclear cells with hyperchromatic nuclei and a clear or pale cytoplasm. Extensive

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, ...

is a common finding. File:Choriocarcinoma - high mag.jpg, High magnification File:Choriocarcinoma - very high mag.jpg, Very high magnification

Treatment

Since gestational choriocarcinoma (which arises from a hydatidiform mole) contains paternal DNA (and thus paternal antigens), it is exquisitely sensitive to

chemotherapy Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...

. The cure rate, even for metastatic gestational choriocarcinoma, is more than 90% when using chemotherapy for invasive mole and choriocarcinoma. As of 2019, treatment with either single-agent methotrexate or actinomycin-D is recommended for low-risk disease, while intense combination regimens including EMACO (

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 ...

methotrexate Methotrexate, formerly known as amethopterin, is a chemotherapy agent and immunosuppressive drug, immune-system suppressant. It is used to treat cancer, autoimmune diseases, and ectopic pregnancy, ectopic pregnancies. Types of cancers it is u ...

, actinomycin D,

cyclophosphamide Cyclophosphamide (CP), also known as cytophosphane among other names, is a medication used as chemotherapy and to suppress the immune system. As chemotherapy it is used to treat lymphoma, multiple myeloma, leukemia, ovarian cancer, breast cancer ...

and

vincristine Vincristine, also known as leurocristine and sold under the brand name Oncovin among others, is a chemotherapy medication used to treat a number of types of cancer. This includes acute lymphocytic leukemia, acute myeloid leukemia, Hodgkin lym ...

(Oncovin) are recommended for intermediate or high-risk disease.

Hysterectomy Hysterectomy is the surgical removal of the uterus and cervix. Supracervical hysterectomy refers to removal of the uterus while the cervix is spared. These procedures may also involve removal of the ovaries (oophorectomy), fallopian tubes ( salpi ...

(surgical removal of the uterus) can also be offered to patients >40 years of age or those for whom sterilisation is not an obstacle. It may be required for those with severe infection and uncontrolled bleeding. Choriocarcinoma arising in the testicle is rare, malignant, and highly resistant to chemotherapy. The same is true of choriocarcinoma arising in the ovary. Testicular choriocarcinoma has the worst prognosis of all germ-cell cancers. File:Diagram showing stage 1 choriocarcinoma CRUK 190.svg, Stage I choriocarcinoma File:Diagram showing stage 2 choriocarcinoma CRUK 207.svg, Stage 2 choriocarcinoma File:Diagram showing stage 3 choriocarcinoma CRUK 220.svg, Stage 3 choriocarcinoma File:Diagram showing stage 4 choriocarcinoma CRUK 229.svg, Stage 4 choriocarcinoma

References

External links

* {{Authority control Gynaecological cancer Male genital neoplasia Germ cell neoplasia