Gestational trophoblastic neoplasia overview: Difference between revisions
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==Pathophysiology== | ==Pathophysiology== | ||
Gestational trophoblastic neoplasia | [[Pregnancy]] occurs when an [[Ovum|egg]], which is released from the [[ovary]] during [[ovulation]], is fertilized by a [[sperm]]. Human [[pregnancy]] takes approximately 40 weeks. Gestational trophoblastic neoplasia arises from the [[Trophoblast|trophoblastic]] tissue, which provides nutrients to the [[embryo]] and develops into a large part of the [[placenta]]. Invasive mole is basically a [[benign]] [[tumor]] which arises from the invasion of the [[myometrium]] of a [[hydatidiform mole]]. Choriocarcinoma is a [[malignant]] [[tumor]] of the [[Trophoblast|trophoblastic]] [[epithelium]]. Placental-site trophoblastic tumor (PSTT), a rare [[tumor]], arises from the [[implantation]] site of [[placenta]]. Epithelioid trophoblastic tumor (ETT) is basically a rare variant of placental-site trophoblastic tumor (PSTT) which arises from the [[malignant]] transformation of [[Chorion|chorionic]]-type intermediate [[Trophoblast|trophoblastic]] [[Cell (biology)|cells]]. Invasive mole is usually [[Ploidy|diploid]] but can also be [[Aneuploidy|aneuploid]] in [[Karyotype|karyotype.]] Choriocarcinoma has an [[Aneuploidy|aneuploid]][[karyotype]] and majority of the cases have a [[Y chromosome]]. | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
Choriocarcinoma must be differentiated from non neoplastic diseases, [[neoplastic]] diseases, and other causes of bleeding during [[pregnancy]]. | Choriocarcinoma must be differentiated from non neoplastic diseases, [[neoplastic]] diseases, and other causes of bleeding during [[pregnancy]]. | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Monalisa Dmello, M.B,B.S., M.D. [2]
Overview
Gestational trophoblastic disease (GTD) includes several rare tumors that occur in the uterus and start in the cells that form the placenta during pregnancy. Only women develop gestational trophoblastic disease. Most gestational trophoblastic diseases are benign, but some are malignant(gestational trophoblastic neoplasia). It is estimated that the malignant forms of gestational trophoblastic disease account for less than 1% of all women’s reproductive system cancers. Gestational trophoblastic neoplasia may be classified according to histology into four subtypes: invasive mole, choriocarcinoma, placental-site trophoblastictumor, and epithelioid trophoblastic tumor.[1] On gross pathology, dark, shaggy, focally hemorrhagic & friable/necrotic-appearing, and invasive border are characteristic findings of gestational trophoblastic neoplasia.[1][2][3] Symptoms of choriocarcinoma include vaginal bleeding, passing of tissue resembling a “bunch of grapes” from the vagina, and abdominal distention.[4] Common physical examination findings of choriocarcinoma include abdominal distention, pelvic/adnexal mass, and blood in vaginal discharge.[4] Choriocarcinoma must be differentiated from non neoplastic diseases, neoplastic diseases, and other causes of bleeding during pregnancy. Elevated serum human chorionic gonadotropin is diagnostic of choriocarcinoma.[1][2] CT scan, MRI, and chest radiography may be performed to detect metastasis of choriocarcinoma to lungs, brain, and liver.[5] The mainstay of therapy for choriocarcinoma is chemotherapy and surgery.[1][4][6]
Historical Perspective
In 6th century, Aetius of Amida, a physician at Justinian's court came up with the term 'hydatid'. Next mention of 'mole' is from 1276 when Margaret, Countess of Henneberg, delivered approximately 300 babies on Good Friday (the Friday before Easter Sunday). In 1827, Marie Anne Victoire Boivin, a Parisian midwife, proposed her findings of this condition in 'Nouvelles Recherches de la Mole Visiculaire' (News Searches of the Vesicular Mole). In 1840, William Wilton reported a case of invasive mole that was complicated by uterine perforation and fatal internal hemorrhage. In 1867, Richard von Volkmann, a German surgeon, also described a lesion resembling an invasive mole. In 1877, Hans Chiari, an Austrian pathologist, reported three cases of choriocarcinoma. He recognized the tumors as epithelial. In 1888, Max Sanger, a German obstetrician, proposed his theory that these tumors were actually sarcomas('deciduoma malignum'). In 1890, Pfeiffer, a student of Hans Chiari, re-examined Chiari's cases and added a fourth case. He named them all 'deciduoma malignum'. In 1891, Pestalozza from Italy, reported three cases of a malignantuterine tumor associated with pregnancy. He described these cases as 'sarcoma hemorrhagicum sen infectiosum'.
Classification
Gestational trophoblastic neoplasia may be classified according to histology into four subtypes: invasive mole, choriocarcinoma, placental-site trophoblastictumor, and epithelioid trophoblastic tumor.[1]
Pathophysiology
Pregnancy occurs when an egg, which is released from the ovary during ovulation, is fertilized by a sperm. Human pregnancy takes approximately 40 weeks. Gestational trophoblastic neoplasia arises from the trophoblastic tissue, which provides nutrients to the embryo and develops into a large part of the placenta. Invasive mole is basically a benign tumor which arises from the invasion of the myometrium of a hydatidiform mole. Choriocarcinoma is a malignant tumor of the trophoblastic epithelium. Placental-site trophoblastic tumor (PSTT), a rare tumor, arises from the implantation site of placenta. Epithelioid trophoblastic tumor (ETT) is basically a rare variant of placental-site trophoblastic tumor (PSTT) which arises from the malignant transformation of chorionic-type intermediate trophoblastic cells. Invasive mole is usually diploid but can also be aneuploid in karyotype. Choriocarcinoma has an aneuploidkaryotype and majority of the cases have a Y chromosome.
Differential Diagnosis
Choriocarcinoma must be differentiated from non neoplastic diseases, neoplastic diseases, and other causes of bleeding during pregnancy.
Epidemiology and Demographics
The incidence of choriocarcinoma is approximately 110-120 per 100,000 pregnancies.[1]
Risk Factors
Common risk factors in the development of choriocarcinoma are child-bearing age, previous hydatidiform mole, and family history of gestational trophoblastic disease.[1]
Natural History, Complications and Prognosis
Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good.[1]
Diagnosis
Staging
According to the Féderation Internationale de Gynécologie et d’Obstétrique (FIGO) cancer staging system, there are 4 stages of choriocarcinoma.[6]
History and Symptoms
Symptoms of choriocarcinoma include vaginal bleeding, passing of tissue resembling a “bunch of grapes” from the vagina, and abdominal distention.[4]
Physical Examination
Common physical examination findings of choriocarcinoma include abdominal distention, pelvic/adnexal mass, and blood in vaginal discharge.[4]
Laboratory Findings
Elevated serum human chorionic gonadotropin is diagnostic of choriocarcinoma.[1][2]
Chest Xray
Chest radiography (CXR) may be helpful in the diagnosis of pulmonary metastasis of choriocarcinoma. The characteristic findings of pulmonary metastasis are peripheral, rounded nodules of variable size scattered throughout both lungs.
CT
CT scan may be performed to detect metastasis of choriocarcinoma to lung, brain, and liver.[5]
MRI
MRI may be performed to detect metastasis of choriocarcinoma to brain and spinal cord.[7]
Ultrasound
Ultrasound may be performed to detect metastasis of choriocarcinoma to pelvis and abdomen.[8]
Treatment
Medical therapy
The mainstay of therapy for choriocarcinoma is chemotherapy.[1][4]
Surgery
Surgery is the mainstay of treatment for choriocarcinoma.[6]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 1.9 Cellular Classification of Gestational Trophoblastic Disease. National Cancer Institute. http://www.cancer.gov/types/gestational-trophoblastic/hp/gtd-treatment-pdq/#section/_5 Accessed on October 8, 2015
- ↑ 2.0 2.1 2.2 Woo J, Hsu C, Fung L, Ma H (1983). "Partial hydatidiform mole: ultrasonographic features". Aust N Z J Obstet Gynaecol. 23 (2): 103–7. PMID 6578773.
- ↑ Choriocarcinoma. librepathology.org. http://librepathology.org/wiki/index.php/Choriocarcinoma Accessed on October 8, 2015
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 Signs and symptoms of gestational trophoblastic disease. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/gestational-trophoblastic-disease/signs-and-symptoms/?region=ns Accessed on October 10, 2015
- ↑ 5.0 5.1 Choriocarcinoma. Radiopaedia.org. http://radiopaedia.org/articles/choriocarcinoma Accessed on October 11, 2015
- ↑ 6.0 6.1 6.2 Treatment of gestational trophoblastic disease. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/gestational-trophoblastic-disease/treatment/?region=ns#type Accessed on October 10, 2015
- ↑ . Diagnosing gestational trophoblastic disease Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/gestational-trophoblastic-disease/diagnosis/?region=ns Accessed on october 13, 2015
- ↑ Diagnosing gestational trophoblastic disease. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/gestational-trophoblastic-disease/diagnosis/?region=ns Accessed on October 12, 2015