Epithelioid trophoblastic tumor ett is an uncommon type of gestational trophoblastic neoplasia originating from the chorionictype intermediate trophoblast. Check this box if you wish to receive a copy of your message. Placental site trophoblastic tumors and epithelioid trophoblastic tumors. Here we present the cytologic findings from a case of infiltrating myoepithelial carcinoma of the breast in a 52yearold female and. Epithelioid trophoblastic tumor is an unusual type of trophoblastic tumor. Pdf extrauterine epithelioid trophoblastic tumor in. Here we present an exceptional case of myoepithelial carcinoma and myoepithelioma association. We identified prognostic factors, optimal treatment, and outcome from the worlds largest case series of patients with ett. Diagnostic challenges in cornual epithelioid trophoblastic.
Myoepitheliosis of breast is a benign tumor of breast affecting the milk ducts. Placental site pstt and epithelioid trophoblastic tumor ett are rare types of gestational trophoblastic neoplasia gtn that arise from intermediate trophoblast. It usually occurs in women in reproductive age group. It, however, has not been reported as a manifestation in the palpebral form of the disease and needs to be kept in the differential diagnosis of a limbal mass lesion in. These tumors start in the cells that would normally develop into the placenta, which connects the fetus to the uterus. Gestational trophoblastic neoplasms gtn are a group of neoplasms from fetal trophoblastic cells including choriocarcinoma cc, epithelioid trophoblastic tumors ett, and placental site trophoblastic tumors pstt 1. The tumor always follows pregnancy, most often molar pregnancy hydatidiform mole. Epithelioid trophoblastic tumor ett is a rare form of gestational trophoblastic neoplasm which is distinct based on its development from intermediate trophoblast cells and nodular growth pattern. It is a trophoblastic tumor of neoplastic chorionic type associated with the intermediate trophoblast. Infiltrating myoepithelial carcinoma of the breast, a case. Unlimited viewing of the articlechapter pdf and any. Infiltrating myoepithelial carcinoma remains a rarely encountered lesion of the breast. Les maladies trophoblastiques gestationnelles emconsulte.
Or, you can choose another section to learn more about a specific question you have. Objectives epithelioid trophoblastic tumor ett is very rare. Gestational trophoblastic tumor genetic and rare diseases. It is considered as a rare subtype of gestational trophoblastic disease 2,4. Synchronous choriocarcinoma and epithelioid trophoblastic.
It is an uncommon manifestation of vernal keratoconjunctivitis and has been reported previously in limbal vkc. Pstt and ett share some clinicalpathological features, such as slow growth rates, early stage at presentation, relatively low. Pathology outlines epithelioid trophoblastic tumor. Gestational trophoblastic disease nord national organization.
Gestational trophoblastic disease memorial sloan kettering. Gestational trophoblastic tumors include 3 welldefined pathologic entities. Use the menu below to choose the introduction section to get started. Jul 28, 2005 adenomyoepithelial tumours and myoepithelial carcinomas of the breast are primarily defined by the presence of neoplastic cells with a myoepithelial immunophenotype. Pdf epithelioid trophoblastic tumor masquerading as.
Epithelioid trophoblastic tumor ett is a rare kind of gestational trophoblastic tumor gtn, which was previously termed atypical choriocarcinoma 1,2. Placental site trophoblastic tumor and choriocarcinoma. Herein, we report a rare case of an epithelioid trophoblastic tumor of the uterus with radiologicpathologic correlation in a 56. Epithelioid trophoblastic tumor ett is an unusual variant of gestational trophoblastic tumor that is closely related to choriocarcinoma and placental site trophoblastic tumor but shows different morphologic and immunohistochemical features. Epithelioid trophoblastic tumor masquerading as invasive squamous cell carcinoma article pdf available in gynecologic oncology 1172. Epithelioid trophoblastic tumor ett is a term proposed for an unusual variant of trophoblastic tumor that is closely related to choriocarcinoma but. Gestational trophoblastic disease gtd is a term used for a group of tumors including complete and partial hydatidiform mole, invasive mole, choriocarcinoma cc, placental site trophoblastic tumor pstt, exaggerated placental site eps, placental site nodule psn, and epithelioid trophoblastic tumor ett. Ovarian epithelioid trophoblastic tumor in a cynomolgus monkey. Isolated epithelioid trophoblastic tumor of the vagina. Due to the rarity of disease, epithelioid trophoblastic tumor remains a diagnostic challenge despite intense research e. Placental site trophoblastic tumors and epithelioid. Case report diagnostic challenges in cornual epithelioid trophoblastic tumor fengying fang a, b, chiungru lai c, d, mingjie yang a, b, bengshian huang b, e, chihyao chen a, b, yiutai li f, mingshyen yen a, b,1, penghui peter wang a, b, g,1, a division of gynecology, department of obstetrics and gynecology, taipei veterans general hospital, taipei, taiwan. An epithelioid trophoblastic tumor ett is an extremely rare form of trophoblastic neoplasm.
The term epithelioid trophoblastic tumor ett was coined for these lesions by mazur and kurman in 1994. Ett used to be called atypical choriocarcinoma because the cells look like choriocarcinoma cells under the microscope, but it is now thought to be a separate disease. This analysis aimed to assess the effects of current management on clinical outcomes and to identify potential prognostic indicators in ett. 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. It is mostly observed in women of all ages that include premenopausal and postmenopausal women the cause of myoepitheliosis of breast is unknown, but the risk factors may include gender women are affected more than men, the use of birth control pills, obesity, and.
Gestational trophoblastic neoplasia is divided into 4 tumor subtypes including invasive mole, choriocarcinoma, placentalsite trophoblastic tumor, and epithelioid trophoblastic tumor ett. Biology, natural history, and treatment modalities. Laparoscopic diagnosis and treatment of an isolated epithelioid trophoblastic tumor in recto. In some cases, there is a risk of recurrence though even though a majority of the tumors are benign, adenomyoepithelioma of breast is associated with a very high risk for breast cancer into the future. The first report, a series of 14 cases by shih and kurman in 1998, described this unique type of tumor comprised of chorionic type intermediate trophoblastic cells distinct from placental site.
Webpathology is a free educational resource with 10308 high quality pathology images of. Abstractan epithelioid trophoblastic tumor ett is a rare trophoblastic tumor originating from. Epithelioid trophoblastic tumor of uterus presenting as an. The patient had a history of hydatidiform mole with recurrent elevation of human chorionic gonadotrophin level that is resistant to chemotherapy. These tumors arise from various subtypes of placental trophoblast, and each has a distinct pathobiology attributable to the proliferative ability of its. Wed like to understand how you use our websites in order to improve them. Metastasizing placental site trophoblastic tumor core. Epithelioid trophoblastic tumor is a rare neoplasm and represents the most recent addition to the gestational trophoblastic tumor category. Placentalsite trophoblastic tumor pstt is a rare form of gestational. Given that this cell of origin is different from other forms of gtn, it is not surprising that the clinical presentation, tumor marker profile, and treatment paradigm for pstt and ett are quite different as well.
Gestational trophoblastic neoplasia includes invasive mole, choriocarcinoma, placental site trophoblatic tumor and epithelioid trophoblastic tumor. Knowledge on prognostic factors and optimal management is limited. The most serious form, choriocarcinoma, is usually preceded by a hydatidiform mole hm, but can. Review of a rare neoplasm of the chorionictype intermediate trophoblast. Epithelioid trophoblastic tumor similar in behavior to pstt, ett is derived from chorionic type extravillous trophoblast, and so has a different immunoprofile than pstt table 2. The epithelioid trophoblastic tumor is an unusual type of trophoblastic tumor. The cells that form gestational trophoblastic tumours are called trophoblasts and come from tissue that grows to form the placenta during pregnancy. In this volume dr hui has brought together a comprehensive overview of gestational trophoblastic disease that includes all the currently recognized entities. Imaging of metastatic epithelioid trophoblastic tumor with. Imaging and clinical data of placental site trophoblastic tumor.
Epithelioid trophoblastic tumor was first described recently by shih et al 1 in 1998 as a separate entity, and since then, only 70 cases have been reported. Epithelioid trophoblastic tumor found on hysteroscopy. In this study, we describe a 4yearold cynomolgus monkey macaca fascicularis showing, at necropsy, a lobulated mass replacing the left ovary and several nodular lesions within the lungs. Jul 22, 2015 circulating tumor cells ctcs play a crucial role in tumor dissemination and are an independent survival predictor in breast cancer bc patients. Mar 21, 2018 the prognosis of adenomyoepithelioma of breast is generally excellent on a surgical excision and removal of the tumor. Prior gestational trophoblastic tumor present in 20% of cases, prior normal pregnancy in 63% of cases and prior spontaneous abortion in 17% of cases acta cytol 2014. In the absence of tissue for a definitive histopathologic diagnosis, disease diagnosed as a result of persistent elevation of human chorionic gonadotropin hcg.
Epithelioid trophoblastic tumor ett is an extremely rare form of gestational trophoblastic neoplasia gtn representing less than 2% of all gestational trophoblastic diseases. Pstt and ett are rare types of gtn both arise from intermediate. Variants differ in propensity for spontaneous resolution, local invasion, and metastasis 1. We report an ett discovered in paracervix, parametrium, and periadnexal soft tissue of a 44yearold woman. Gestational trophoblastic disease represents a wide spectrum of proliferative disorders characterized by proliferation of trophoblasts, including premalignant and malignant lesions.
Epidemiology it typically presents in women of reproductive age peak age. The cells that form gestational trophoblastic tumours are called trophoblasts and come from tissue that grows to form the placenta during pregnancy there are several different types of gtd. Ett can be a sequela of any gestational event and can present in both intrauterine and extrauterine sites. Most breast adenomyoepitheliomas are considered to be benign or to have a lowgrade malignant potential, characterized by propensity for local recurrence. All three patients were of reproductive age and presented with vaginal bleeding and mild elevation of human chorionic gonadotropin hcg. Two of the 14 patients presented with extrauterine ett without evidence of prior gestational trophoblastic disease in the uterus. Cases of ett present as abnormal vaginal bleeding in women of reproductive age, with low human chorionic gonadotropin hcg levels. After appropriate diagnosis and treatment, the tumor responded to therapy. These tumours are rare, and they appear when cells in the womb start to proliferate uncontrollably. Malignant changes arising in this lesion are extremely rare and may involve one or both cellular components. Epithelioid trophoblastic tumor ett is an extremely rare form of gestational trophoblastic neoplasia gtn. Synchronous choriocarcinoma and epithelioid trophoblastic tumor ett concurring at the cesarean scar. Gestational trophoblastic neoplasia gtn gestational neoplasms include. Gestational trophoblastic tumors gtt are malignant forms of gestational trophoblastic disease.
Intermediate trophoblastic tumors, which include placental site trophoblastic tumor pstt and epithelioid trophoblastic tumor ett, are rare in the ovary, with most assumed to be gestational. Oct 30, 20 breast adenomyoepithelioma is an unusual tumor characterized by a biphasic proliferation of epithelial and myoepithelial cells. It was first described by shih and kurman am j surg pathol 1998. Less than 300 cases of pstt have been reported in literature, with an incidence of. Nov 18, 2016 myoepithelial carcinoma is a rare malignant cancerous tumor that usually occurs in the salivary glands in the mouth, but can also occur in skin and soft tissues. The tumor is composed exclusively of myoepithelial cells that show spindle, plasmacytoid, and clear cell morphology. Histologically, it is composed by spindle cells and an infiltrate of inflammatory cells.
Epithelioid trophoblastic tumor radiology reference article. Shen x, xiang y, guo l, ren t, feng f, wan x, et al. Contact your institutions library about getting access. Epithelioid trophoblastic tumors etts are resistant to chemotherapy mitotic count of 610 hpf is an unfavorable prognostic factor am j obstet gynecol. An intermediate trophoblast is a distinctive trophoblastic cell population from which four trophoblastic lesions are thought to arise. Placental site trophoblastic tumor and epithelioid.
This article examines the subtypes of gtd, and their treatment. Malignant myoepithelioma of the breast is an extremely rare tumor composed entirely or almost entirely of malignant spindle cells with myoepithelial differentiation. Laparoscopic diagnosis and treatment of an isolated. Pseudoepitheliomatous hyperplasia at the limbus can mimic an ocular surface squamous neoplasia. Chorioepithelioma article about chorioepithelioma by the. Similar tumors were subsequently described in patients with molar gestations in the uterus. Combined myoepithelial carcinoma and myoepithelioma in soft.
A series of 27 adenomyoepithelial tumours of the breast was analysed immunohistochemically. Placental site trophoblastic tumor and epithelioid trophoblastic tumor. The pathology of intermediate trophoblastic tumors and tumorlike lesions article in international journal of gynecological pathology 201. Placental site trophoblastic tumor pstt is a very rare variant of gestational. Apr 26, 2020 gestational trophoblastic disease gtd is a rare group of disorders, both benign and malignant, that affect women of childbearing age. The pathology of intermediate trophoblastic tumors and tumor. Epithelioid trophoblastic tumor ett is an extremely rare subtype of gestational trophoblastic neoplasia gtn, accounting for only 1. We retrospectively analyzed 49 cases of fep diagnosed and excised in a single academic institution from 1995 to 2011. The number of mitotic figures varies, but at least a low level of mitotic activity is present. Limbal pseudoepitheliomatous hyperplasia mimicking ocular.
Pstt is a slowgrowing malignant monomorphic tumor that arises from placental intermediate trophoblasts, which are responsible for embryo implantation, and have features of both. We present here a case of a 33yearold woman with an epithelioid trophoblastic tumor with metastatic disease to the lungs that was initially thought to represent cervical carcinoma. Mar 01, 2011 the tumor always follows pregnancy, most often molar pregnancy hydatidiform mole. Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by dail and liebow as an aggressive bronchoalveolar cell carcinoma. Placental site trophoblastic tumor pstt and epithelioid trophoblastic tumor ett are the rarest gestational trophoblastic neoplasias, developing from intermediate trophoblast of the implantation site and chorion leave, respectively. The remarkable curability of gtn might be attributable partly to a host immunologic response to paternal antigens expressed on trophoblastic cells. Epidemiology 49 3 epidemiology chan lee, harriet o smith, seung j kim gestational trophoblastic disease gtd encompasses an intriguing group of interrelated diseases derived from placental trophoblasts.
They were incorporated into the world health organization classification of soft tissue tumors in 2002. This tumour occurs in women of reproductive age some years after a gestation, which may be a term delivery, spontaneous abortion or molar gestation. An important characteristic is the presence of geographic necrosis fig. Gestational trophoblastic disease gtd is a term used for a group of pregnancyrelated tumours. Although diagnosis was usually associated with a gestational event, the latter was sometimes remote. Approximately 66% of these tumors occur in a part of the salivary gland, known as the parotid gland.
Sep 24, 2014 soft tissue myoepithelial carcinoma and myoepithelioma are rare entities, part of myoepithelial tumors. Epithelial to mesenchymal transition emt is involved in cancer invasion and metastasis. Chorioepitheliomas arise from trophoblastic elements in young women 25 to 35 years of age, usually after the formation of a hydatiform mole, after abortion, or, less commonly, after childbirth several years may elapse between the end of the last pregnancy and onset of the disease. Epithelioid trophoblastic tumor ett confined to the. Initially described as atypical choriocarcinoma, shih and kurman first reported the clinicopathological features of ett in 1998 3, and the world health organization 4. To the best of our knowledge, such an association has never been reported in the literature.
Gestational trophoblastic disease gtd is a group of rare tumors that begin during a pregnancy. The pathology of intermediate trophoblastic tumors and tumor like lesions. Epithelioid trophoblastic tumor ett is a gestational trophoblastic disease with about 110 case reports in the literature. Differentiating squamous cell carcinoma of the cervix and. Gestational trophoblastic neoplasia gtn refers more specifically to malignant disease. Herein, we describe a patient with coexisting epithelioid trophoblastic tumor and choriocarcinoma in the uterus. The tumor cells are proposed to be of chorionic type intermediate trophoblastic cell lineage due to their. Serum human chorionic gonadotropin levels were elevated in eight of nine patients in whom this information was available. 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 gestational trophoblastic neoplasm of intermediate trophoblasts. Given that this cell of origin is different from other forms of gtn, it is not surprising that the clinical presentation, tumor marker profile, and treatment paradigm for pstt and.
Analysis of clinicopathologic prognostic factors in 9 patients with epithelioid trophoblastic tumor. Who classification of tumours of female reproductive organs, 4th edition, 2014. Epithelioid trophoblastic tumor ett is an extremely rare type of gestational trophoblastic disease that can be hard to diagnose. This article is from oncotargets and therapy, volume 6. A rare postgestational trophoblastic tumour composed of epithelioid cells epidemiology. Placental site trophoblastic tumor pstt is a very rare malignant tumor, belonging to a family of pregnancyrelated illnesses, called gestational trophoblastic diseases gtd. Stage i disease is treated with hysterectomy, while metastatic disease is treated with surgery and chemotherapy. Gestational trophoblastic disease gtd includes complete and partial hydatiform mole, invasive mole, choriocarcinoma, placental site trophoblastic tumour and epithelioid trophoblastic tumour ett. Epithelioid trophoblastic tumors etts are resistant to chemotherapy. The aim of this study was to assess correlation between ctcs and expression of emt transcription factors twist1 and slug in breast tumor tissue. Gestational trophoblastic disease williams obstetrics, 25e. Mitotic count of 610hpf is an unfavorable prognostic factor. Clinical and pathological features, prognostic variables and treatment strategy. Epithelioid trophoblastic tumor ett is an unusual type of trophoblastic tumor, with features resembling carcinoma.
Placental site trophoblastic tumor pstt is a very rare malignant tumor. Relationship between circulating tumor cells and epithelial. Epithelioid trophoblastic tumor ett is a rare gestational trophoblastic tumor. The few cases that have surfaced firmly document the histopathology of this tumor, but its cytologic characteristics seemingly have been described in only one other report. Epithelioid trophoblastic tumour, ett immunohistochemistry. Department of obstetrics and gynecology, peking union medical college hospital, peking union medical college, chinese academy of medical sciences, beijing, china. Epithelioid trophoblastic tumors are exceptionally rare malignant forms of gestational trophoblastic neoplasia arising from the placenta. Its biological behavior is different from the general gtn, but similar to placental site trophoblastic tumor pstt. Methods we applied a literature search using pubmed to analyze the clinical data of 78. Here we report on the clinicopathologic and immunohistochemical features of three cases of epithelioid trophoblastic tumor. Epithelioid trophoblastic tumor ett is a rare variety of gestational trophoblastic tumors distinct from placental site trophoblastic tumor and choriocarcinoma. Gestational trophoblastic disease gtd includes complete and partial hydatiform mole, invasive mole, choriocarcinoma, placental site trophoblastic tumour and. Dec 02, 2012 epithelioid trophoblastic tumor ett is a rare gestational trophoblastic tumor. Epithelioid trophoblastic tumor ett is a rare gestational trophoblastic tumor and often poses a diagnostic and therapeutic challenge to the involved clinicians.
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