Choriocarcinoma is the most frequent gestational trophoblastic neoplasia. Unlike usual markers of gestational trophoblastic neoplasia p63, human chorionic gonadotrophin and human placental lactogen, sall4 was expressed in. Sonographic appearance of gestational trophoblastic. Generally the behavior of epithelioid trophoblastic tumor is similar to that of placentalsite trophoblastic tumor, and both of them usually behave in a benign fashion, but metastasis and death occur in about 25% and 10% of cases, respectively3. Epithelioid trophoblastic tumor radiology reference article. Large epithelioid tumours are common and represent a large differential diagnosis when viewed generally. Ett can be a sequela of any gestational event and can present in both intrauterine and extrauterine sites. Glypican3 gpc3 is a membranebound heparan sulfate proteoglycan that functions in embryonic cell growth and differentiation and is highly expressed in the placenta. Epithelioid trophoblastic tumor ett is a rare gestational trophoblastic tumor. Original article extrauterine epithelioid trophoblastic.
A an irregular, plaquelike, soft, brown tumor centered at the previous cesarean section site arrow. Small epithelioid lesions are dealt with in the small round cell tumours article. Because both types of tumors are slow growing, the. Histologically, ett arises from the chorionictype intermediate. The last three entities cca, pstt, and ett are called gestational trophoblastic neoplasia gtn and are considered malignant because of. Research open access expression of glypican 3 in placental. Epithelioid trophoblastic tumor radiology reference. Typically, epithelioid sarcoma starts as a small firm growth or lump thats painless. Epithelioid trophoblastic tumor of the lung urabe 2007. Epithelioid trophoblastic tumor ett is a rare gestational trophoblastic tumor and often poses a diagnostic and therapeutic challenge to the involved clinicians. Sep 23, 2005 epithelioid trophoblastic tumor is a rare neoplasm and represents the most recent addition to the gestational trophoblastic tumor category.
Seven cases of epithelioid trophoblastic tumor treated in the womens hospital of zhejiang university from 2004 september to 2008 december were retrospectively analyzed. Epithelioid trophoblastic tumor placental site trophoblastic tumor unclassified missing histotype all % missing i ii iii iv completehydatidiformmole 552 61. This term covers hydatidiform mole including complete and partial moles, invasive mole, gestational choriocarcinoma, and placental site trophoblastic tumour pstt. A relatively recent addition to the group of gestational trophoblastic disease. Epithelioid trophoblastic tumor ett is an extremely rare subtype of gestational. Gestational trophoblastic neoplasia comprises choriocarcinoma, placental site trophoblastic tumor pstt and epithelioid trophoblastic tumor ett. Here we report on the clinicopathologic and immunohistochemical features of three cases of epithelioid trophoblastic tumor. Researched pathways related to epithelioid trophoblastic tumor include pathogenesis, localization, cell proliferation, cell differentiation, cell adhesion. Epithelioid trophoblastic tumor ett is a rare gestational trophoblastic neoplasm composed of intermediate trophoblasts. Epithelioid trophoblastic tumor is a rare neoplasm and represents the most recent addition to the gestational trophoblastic tumor category.
Gestational trophoblastic disease gynecology and obstetrics. Epithelioid trophoblastic tumor ett is a rare variety of gestational trophoblastic disease gtd that is composed of intermediate trophoblast cells, which was. Dec 02, 2012 epithelioid trophoblastic tumor ett is a rare gestational trophoblastic tumor. Epithelioid trophoblastic tumor in a postmenopausal woman. Gestational trophoblastic disease gtd includes complete and partial hydatiform mole, invasive mole, choriocarcinoma, placental site trophoblastic tumour and. Epithelioid trophoblastic tumor ett is an extremely rare subtype of gestational trophoblastic neoplasia gtn, accounting for only 1. Nearly half arise in the cervix or lower segment of the uterus and some in the fundus and broad ligament. This rare tumor is composed of epithelioid cells with large atypical nuclei and moderate to abundant clear to eosinophilic cytoplasm, resulting in a carcinomalike appearance. Update on the pathology of gestational trophoblastic disease.
Update on the diagnosis and management of gestational. Gestational trophoblastic disease gtd is a term used for a group of pregnancyrelated tumours. Ultrasonography showed inhomogeneous echo measuring 2. The objective of this study is to evaluate the clinicopathological features and immunohistochemical characteristics of epithelioid trophoblastic tumor ett. A rare postgestational trophoblastic tumour composed of epithelioid cells epidemiology. Epithelioid trophoblastic tumour ett is an unusual type of trophoblastic tumour, which can cause difficulties in diagnosis and as a consequence in treatment. Case series of five patients with pure or mixed gestational. It was first proposed by mazur and kurman in 1994 as an unusual type of trophoblastic tumor that is distinct from placental site trophoblastic tumor and choriocarcinoma and has features resembling carcinoma. Cases of ett present as abnormal vaginal bleeding in women of reproductive age, with low human chorionic gonadotropin hcg levels. The literature suggests that surgery should be the treatment of choice for ett as it is not responsive to chemotherapeutic agents, used in the treatment of other types of gestational trophoblastic diseases. Epithelioid trophoblastic tumor is the most recently described type of gestational trophoblastic neoplasia. It usually occurs in the reproductive age range and presents with vaginal bleeding. A case of epithelioid trophoblastic tumour ett, occurring in a fallopian tube of a 39yearold woman, is reported.
Three cases of epithelioid trophoblastic tumor were selected from the yale pathology files. Although diagnosis was usually associated with a gestational event, the latter was sometimes remote. It consists of a trimorphic proliferation of intermediate trophoblastic cells, syncytiotrophoblast and cytotrophoblast. Choriocarcinoma, placental site trophoblastic tumor, and epithelioid trophoblastic tumor are the main types of gestational trophoblastic neoplasms. Epithelioid trophoblastic tumor found on hysteroscopy zhang. The patient presented with a positive pregnancy test, but continued to have periods. Epithelioid trophoblastic tumor ett is a gestational trophoblastic disease with about 110 case. Epithelioid trophoblastic tumor, like placentalsite trophoblastic tumor, may be. Epithelioid trophoblastic tumors etts are the rarest type of gestational. Epithelioid sarcoma is a rare, slowgrowing type of soft tissue cancer. The tumor cells are proposed to be of chorionic type intermediate trophoblastic cell lineage due to their. Cases of ett present as abnormal vaginal bleeding in women of reproductive age, with low human chorionic.
Serum human chorionic gonadotropin levels were elevated in eight of nine patients in whom this information was available. Most cases of ett are reported in women of reproductive age following a prior gestation within 2 weeks to 30 years. Cases of ett present as abnormal vaginal bleeding in women of reproductive age. Two of the 14 patients presented with extrauterine ett without evidence of prior gestational trophoblastic disease in the uterus. Manifestations may include excessive uterine enlargement, vomiting, vaginal bleeding, and preeclampsia, particularly during early pregnancy. Malignant epithelioid lesions are often carcinomas. Two of the most common risk factors for women include 1.
This study reveals the molecular features of malignant gtn, including choriocarcinoma cc, epithelioid trophoblastic tumor ett, and placental site trophoblastic tumor pstt, by whole transcriptome sequencing analysis. Macroscopic features of a coexisting epithelioid trophoblastic tumor ett and placental site trophoblastic tumor pstt at the cesarean section site. Epithelioid trophoblastic tumor is a rare and special type of intermediate trophoblastic tumor with a high degree of malignancy and poor prognosis. Gestational trophoblastic tumors gtt are malignant forms of gestational trophoblastic disease. We report a case of epithelioid trophoblastic tumor in a young woman which involved the uterus, parametrium and the right ovary. Epithelioid trophoblastic tumor ett is a rare trophoblastic tumor originating from chorionictype inter mediate trophoblasts its. The number of mitotic figures varies, but at least a low level of mitotic activity is present. Gtn may be nonmetastatic or metastatic 2, with the cure rate approaching 100% and fertility preservation usually possible.
Epithelioid trophoblastic tumor ett is a rare type of gestational trophoblastic disease and only 25 cases have been reported so far. All of the following are features of epithelioid trophoblastic tumor except. May 20, 20 macroscopic features of a coexisting epithelioid trophoblastic tumor ett and placental site trophoblastic tumor pstt at the cesarean section site. Although gtn is generally seen in molar pregnancies, it can be seen in any pregnancy. Epithelioid trophoblastic tumor ett complete and partial moles are abnormal pregnancies and are considered benign. Epithelioid trophoblastic tumor found on hysteroscopy a 36yearold woman presented with a history of prolonged menstrual period and increased menstrual volume of 4 months. These tumors start in the cells that would normally develop into. We report a rare case of choriocarcinoma admixed with placental site trophoblastic tumor pstt. The last three entities cca, pstt, and ett are called gestational trophoblastic neoplasia gtn and are considered malignant because of their potential for local invasion and metastases. Case report epithelioid trophoblastic tumor after induced abortion. Epithelioid trophoblastic tumour, ett immunohistochemistry. An epithelioid trophoblastic tumor is an extremely rare gestational trophoblastic tumor gtt.
Etts exhibit slow growth and remain confined to the uterus for extended periods of time. Calcification diffusely positive for hpl presence of geographic necrosis tumor is well circumscribed and grows in nests, cords or solid masses. An additional two cases were included in the dna genotyping study. Original article extrauterine epithelioid trophoblastic tumor of the vagina. Large epithelioid tumours are common and represent a large differential diagnosis when viewed generally malignant epithelioid lesions are often carcinomas. A more general discussion of epithelioid lesions is in the epithelioid cell lesions article. Apr 09, 2020 gestational trophoblastic disease gtd is a rare group of disorders, both benign and malignant, that affect women of childbearing age. Gestational trophoblastic tumor genetic and rare diseases. Patients often have symptoms resembling those in placental site trophoblastic tumor pstt. All three patients were of reproductive age and presented with vaginal bleeding and mild elevation of human chorionic gonadotropin hcg. Three cases of epithelioid trophoblastic tumor was identified in yale pathology files, one case was obtained from memorial sloankettering. Jan 20, 2010 the objective of this study is to evaluate the clinicopathological features and immunohistochemical characteristics of epithelioid trophoblastic tumor ett. Gestational trophoblastic disease gtd is a group of rare tumors that begin during a pregnancy.
Gestational trophoblastic neoplasia gtn gestational neoplasms include. Epithelioid trophoblastic tumor ett is a term proposed for an unusual variant of trophoblastic tumor that is closely related to choriocarcinoma but shows monomorphic growth of highly atypical. Original article extrauterine epithelioid trophoblastic tumor. These tumours are rare, and they appear when cells in the womb start to proliferate uncontrollably. Gestational trophoblastic disease gtd is a group of disorders derived from a pregnancy. The 36yearold patient with persistent vaginal bleeding and elevated human chorionic gonadotropin hcg received three curretages and laparoscopic hysterectomy followed by five cycles of combination chemotherapy with emaco. Case report a rare case of choriocarcinoma admixed with. Epidemiology it typically presents in women of reproductive age peak age.
In the absence of tissue for a definitive histopathologic diagnosis, disease diagnosed as a result of persistent elevation of human chorionic gonadotropin hcg. Diagnosis and management of mixed gestational trophoblastic. The cells that form gestational trophoblastic tumours are called trophoblasts and come from tissue that grows to form the placenta during pregnancy. Most cases begin in the soft tissue under the skin of a finger, hand, forearm, lower leg or foot, though it can start in other areas of the body. The tumor always follows pregnancy, most often molar pregnancy hydatidiform mole. It is a trophoblastic tumor of neoplastic chorionic type associated with the intermediate trophoblast. Genomic characteristics of gestational trophoblastic neoplasm gtn are mostly unknown. Gestational trophoblastic disease is proliferation of trophoblastic tissue in pregnant or recently pregnant women. Pathology outlines epithelioid trophoblastic tumor. Sall4 expression in gestational trophoblastic tumors. Metastatic epithelioid trophoblastic tumor of the lung. Misdiagnosis delays effective treatment and affects the survival. It is considered as a rare subtype of gestational trophoblastic disease 2,4. The study of epithelioid trophoblastic tumor has been mentioned in research publications which can be found using our bioinformatics tool below.
Whole transcriptome analysis of gestational trophoblastic. Gestational trophoblastic disease memorial sloan kettering. These tumors start in the cells that would normally develop into the placenta, which connects the fetus to the uterus. Gestational trophoblastic disease gtd is a rare group of disorders, both benign and malignant, that affect women of childbearing age. Epithelioid trophoblastic tumor is an unusual type of trophoblastic tumor. Epithelioid trophoblastic tumor ett is a gestational trophoblastic disease with about 110 case reports in the literature. Epithelioid trophoblastic tumor of uterus presenting as an. This article examines the subtypes of gtd, and their treatment. Pdf epithelioid trophoblastic tumor alexandros rouvas. An epithelioid trophoblastic tumor ett is an extremely rare form of trophoblastic neoplasm. Research open access expression of glypican 3 in placental site trophoblastic tumor robin j ouyang1, pei hui2, ximing j yang1, debra l zynger3 abstract background. Placental site trophoblastic tumor and epithelioid trophoblastic tumor placental site trophoblastic tumor pstt is a rare malignancy occurring in 1 of 100,000 pregnancies. Specifically, itt comprise placental site trophoblastic tumor pstt and epithelioid trophoblastic tumor ett 1.
Mar 01, 2011 the tumor always follows pregnancy, most often molar pregnancy hydatidiform mole. The tumor is composed of intermediate trophoblastic cells arranged in nests and sheets embedded with a. This rare tumor is composed of epithelioid cells with large atypical nuclei and moderate to. Transformation of a postcesarean section placental site. Adenomyosis with thickened myometrium was noted in the myometrium arrowhead. An unusual malignancy of ovary humera mahmood1, mohammad faheem 1, mohammad tahir2, sarosh arif, sana mahmood and javaid irfan1 abstract epithelioid trophoblastic tumor ett is a distinct but rare variety of gestational trophoblastic tumors. Epithelioid trophoblastic tumor found on hysteroscopy.
Initially described as atypical choriocarcinoma, shih and kurman first reported the clinicopathological features of ett in 1998 3, and the world health organization 4 classified ett as a form of gtn in 2003. This tumour occurs in women of reproductive age some years after a gestation, which may be a term delivery, spontaneous abortion or molar gestation. What links here related changes upload file special pages permanent link page. Sonographic appearance of gestational trophoblastic disease.