Choriocarcinoma
Encyclopedia
Choriocarcinoma is a malignant, trophoblastic and aggressive cancer
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...

, usually of the placenta
Placenta
The placenta is an organ that connects the developing fetus to the uterine wall to allow nutrient uptake, waste elimination, and gas exchange via the mother's blood supply. "True" placentas are a defining characteristic of eutherian or "placental" mammals, but are also found in some snakes and...

. It is characterized by early hematogenous spread to the lungs. It belongs to the far end of the spectrum of gestational trophoblastic disease
Gestational trophoblastic disease
Gestational trophoblastic disease is a term used for a group of pregnancy-related tumours. These tumours are rare, and they appear when cells in the womb start to grow out of control. The cells that form gestational trophoblastic tumours are called trophoblasts and come from tissue that grows to...

 (GTD), a subset of germ cell tumor
Germ cell tumor
A germ cell tumor is a neoplasm derived from germ cells. Germ cell tumors can be cancerous or non-cancerous tumors. Germ cells normally occur inside the gonads...

s.

Pathology

Characteristic feature is the identification of intimately related syncytiotrophoblast
Syncytiotrophoblast
Syncytiotrophoblast is the epithelial covering of the placenta villous tree. It is a unique tissue in that it is a multi-nucleated, terminally differentiated syncytium, extending to 13m^2...

s and cytotrophoblast
Cytotrophoblast
The cytotrophoblast is the inner layer of the trophoblast, interior to the syncytiotrophoblast in an embryo.Cytotrophoblast is considered to be the trophoblastic stem cell; it differentiates into the other forms of trophoblastic tissue .-External links: - "Female Reproductive System: placental...

s without formation of definite placental type villi.

Syncytiotrophoblasts are large cells with bizarre nuclei and an eosinophilic
Eosinophilic
Eosinophilic refers to the staining of certain tissues, cells, or organelles after they have been washed with eosin, a dye.Eosin is an acidic dye; thus, the structure being stained is basic....

 cytoplasm
Cytoplasm
The cytoplasm is a small gel-like substance residing between the cell membrane holding all the cell's internal sub-structures , except for the nucleus. All the contents of the cells of prokaryote organisms are contained within the cytoplasm...

. They often surround the cytotrophoblasts.

Cytotrophoblasts are polyhedral and regular cells. Have a clear/eosinophillic cytoplasm with hyperchromatic nuclei. It's also Chorion associated with the uterus.

Etiology/Epidemiology

Choriocarcinoma of the placenta
Placenta
The placenta is an organ that connects the developing fetus to the uterine wall to allow nutrient uptake, waste elimination, and gas exchange via the mother's blood supply. "True" placentas are a defining characteristic of eutherian or "placental" mammals, but are also found in some snakes and...

 during pregnancy is preceded by:
  • hydatidiform mole
    Hydatidiform mole
    Molar pregnancy is an abnormal form of pregnancy, wherein a non-viable, fertilized egg implants in the uterus, and thereby converts normal pregnancy processes into pathological ones. It is characterized by the presence of a hydatidiform mole...

     (50% of cases)
  • Spontaneous abortion (20% of cases)
  • ectopic pregnancy
    Ectopic pregnancy
    An ectopic pregnancy, or eccysis , is a complication of pregnancy in which the embryo implants outside the uterine cavity. With rare exceptions, ectopic pregnancies are not viable. Furthermore, they are dangerous for the parent, since internal haemorrhage is a life threatening complication...

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


Rarely, choriocarcinoma occurs in primary locations other than the placenta; very rarely, it occurs in testicles. Although trophoblastic components are common components of mixed germ cell tumors, pure choriocarcinoma of the adult testis 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 spread. Because of early spread and inherent resistance to anticancer drugs, patients have poor prognosis. Elements of choriocarcinoma in a mixed testicular tumor have no prognostic importance.

Choriocarcinomas can also occur in the ovaries.

Symptoms/Signs/Labs

  • increased quantitative β-hCG
    Human chorionic gonadotropin
    Human chorionic gonadotropin or human chorionic gonadotrophin is a glycoprotein hormone produced during pregnancy that is made by the developing embryo after conception and later by the syncytiotrophoblast .. Some tumors make this hormone; measured elevated levels when the patient is not...

     levels
  • vaginal bleeding
    Vaginal bleeding
    Vaginal bleeding refers to bleeding in females that is either a physiologic response during the non-conceptional menstrual cycle or caused by hormonal or organic problems of the reproductive system. Vaginal bleeding may occur at any age, but always needs investigation when encountered in female...

  • shortness of breath
  • hemoptysis
    Hemoptysis
    Hemoptysis or haemoptysis is the expectoration of blood or of blood-stained sputum from the bronchi, larynx, trachea, or lungs Hemoptysis or haemoptysis is the expectoration (coughing up) of blood or of blood-stained sputum from the bronchi, larynx, trachea, or lungs Hemoptysis or haemoptysis ...

     (coughing up blood)
  • chest pain
    Chest pain
    Chest pain may be a symptom of a number of serious conditions and is generally considered a medical emergency. Even though it may be determined that the pain is non-cardiac in origin, this is often a diagnosis of exclusion made after ruling out more serious causes of the pain.-Differential...

  • chest X-ray
    Chest X-ray
    In medicine, a chest radiograph, commonly called a chest X-ray , is a projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures...

     shows multiple infiltrates of various shapes in both lungs
  • presents in males as a testicular neoplasm
    Testicular cancer
    Testicular cancer is cancer that develops in the testicles, a part of the male reproductive system.In the United States, between 7,500 and 8,000 diagnoses of testicular cancer are made each year. In the UK, approximately 2,000 men are diagnosed each year. Over his lifetime, a man's risk of...

    , sometimes with skin hyperpigmentation (from excess beta hCG cross reacting with the alpha MSH receptor), gynecomastia, and weight loss (from excess beta hCG cross reacting with the TSH receptor) in males
  • can present with increased TSH
    Thyroid-stimulating hormone
    Thyrotrophin-stimulating hormone is a peptide hormone synthesized and secreted by thyrotrope cells in the anterior pituitary gland, which regulates the endocrine function of the thyroid gland.- Physiology :...


Treatment

Choriocarcinoma is one of the tumors that is most sensitive to chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....

. The cure rate, even for metastatic choriocarcinoma, is around 90-95%. Virtually everyone without metastases can be cured; however, metastatic disease to the liver and/or brain is usually fatal. At present, treatment with single-agent methotrexate
Methotrexate
Methotrexate , abbreviated MTX and formerly known as amethopterin, is an antimetabolite and antifolate drug. It is used in treatment of cancer, autoimmune diseases, ectopic pregnancy, and for the induction of medical abortions. It acts by inhibiting the metabolism of folic acid. Methotrexate...

 or actinomycin D is recommended for low-risk disease, while intense combination regimens including EMACO (etoposide
Etoposide
Etoposide phosphate is an anti-cancer agent. It is known in the laboratory as a topoisomerase poison. Etoposide is often incorrectly referred to as a topoisomerase inhibitor in order to avoid using the term "poison" in a clinical setting...

, methotrexate, actinomycin D, cyclosphosphamide and vincristine
Vincristine
Vincristine , formally known as leurocristine, sometimes abbreviated "VCR", is a vinca alkaloid from the Catharanthus roseus , formerly Vinca rosea and hence its name. It is a mitotic inhibitor, and is used in cancer chemotherapy.-Mechanism:Tubulin is a structural protein that polymerizes to...

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

Hysterectomy
Hysterectomy
A hysterectomy is the surgical removal of the uterus, usually performed by a gynecologist. Hysterectomy may be total or partial...

(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.

External links

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