Epithelioid trophoblastic tumor ett is a rare variety of gestational trophoblastic tumors distinct from placental site trophoblastic tumor and choriocarcinoma. Epithelioid glioblastoma is a variant of glioblastoma along with gliosarcoma and giant cell glioblastoma only recently added to the who classification of cns tumors as part of the 2016 update 1. Jun 15, 2007 epithelioid trophoblastic tumor ett is a rare type of gestational trophoblastic disease and only 25 cases have been reported so far. Examination of a biopsy specimen resulted in a diagnosis of epithelioid mpnst. These tumors arise from various subtypes of placental trophoblast, and each has a distinct pathobiology attributable to the proliferative ability of its constituent trophoblast.
Epithelioid trophoblastic tumor ett complete and partial moles are abnormal pregnancies and are considered benign. Gestational trophoblastic disease gtd is a term used for a group of pregnancyrelated tumours. 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 tumor. 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. Pathology outlines epithelioid trophoblastic tumor. Gestational trophoblastic disease gtd includes complete and partial hydatiform mole, invasive mole, choriocarcinoma, placental site trophoblastic tumour and. It may also be found in the legs, trunk, head or neck regions.
A case report sakiko kageyama1, masafumi kanoto1, yukio s ugai1, takeshi s uto2, satoru nagase2, mitsumasa osakabe3, and takaaki hosoya1 1department of diagnostic radiology, yamagata university school of medicine, 222 iidanishi, yamagata 9909585, japan. Historical, morphological and clinical overview of placental site trophoblastic tumors. Age and a previous molar pregnancy affect the risk of gtd. To describe a case of epithelioid trophoblastic tumor ett in a postmenopausal woman, which had several peculiar features that differentiate it from previously reported etts. Immunohistochemical staining is a helpful aid to distinguish epithelioid trophoblastic tumor from other gestational trophoblastic neoplasms. Small epithelioid lesions are dealt with in the small round cell tumours article. Sep 10, 2018 epithelioid hemangioendothelioma of lung is a malignant tumor with metastasizing potential. Gestational trophoblastic disease gtd is a broad term encompassing both benign and malignant growths arising from products of conception in the uterus. Typically, epithelioid sarcoma starts as a small firm growth or lump thats painless. Gestational trophoblastic neoplasia gtn gestational neoplasms include. Use the menu below to choose the introduction section to get started. June 2017 includes ptnm requirements from the 8th edition, ajcc staging manual and 2015 figo cancer report.
Misdiagnosis delays effective treatment and affects the survival. Huge epithelioid malignant peripheral nerve sheath tumor in. Sonographic appearance of gestational trophoblastic disease. These tumors develop at the place where the placenta attaches to the wall of the womans uterus. 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. Think of that menu as a roadmap for this complete guide.
We present a case of primary cornual epithelioid trophoblastic tumor ett because of its rarity and diagnostic and therapeutic challenge. Combining with the patients clinical presentation a final diagnosis of mixed gtn located in the cesarean scar was made ett 90% and cc 10%. Aug 12, 2015 this report describes a patient with a rare huge epithelioid malignant peripheral nerve sheath tumor mpnst in the left axilla. Serum human chorionic gonadotropin levels were elevated in eight of nine patients in whom this information was available. 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 is rare and there is little information available in the literature regarding its appearance on ultrasound imaging. 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.
Epithelioid sarcoma is a rare, slowgrowing type of soft tissue cancer. Primary epithelioid trophoblastic tumor of the lung the. Epithelioid trophoblastic tumour ett is an unusual type of trophoblastic tumour, which can cause difficulties in diagnosis and as a consequence in treatment. Cases of ett present as abnormal vaginal bleeding in women of reproductive age, with low human chorionic gonadotropin hcg levels. Epithelioid trophoblastic tumor ett is a gestational trophoblastic disease with about 110 case reports in the literature. The tumor cells are proposed to be of chorionic type intermediate trophoblastic cell lineage due to their. A novel phenotype combining primary ovarian insufficiency growth retardation and pilomatricomas with mcm8 mutation. Apr 26, 2020 gestational trophoblastic disease gtd is a rare group of disorders, both benign and malignant, that affect women of childbearing age. Gestational trophoblastic disease is a tumor originating from the trophoblast, which surrounds the blastocyst and develops into the chorion and amnion see page placenta and embryo at about 11 4. Gestational trophoblastic disease memorial sloan kettering. Webpathology is a free educational resource with 10308 high quality pathology images of benign and malignant neoplasms and related entities. Objectives epithelioid trophoblastic tumor ett is very rare.
Epithelioid trophoblastic tumor masquerading as invasive squamous cell carcinoma article pdf available in gynecologic oncology 1172. Incidence and mortality the reported incidence of gtd varies widely worldwide, from a low of 23 per 100,000 pregnancies paraguay to a high of 1,299 per 100,000 pregnancies indonesia. Extrauterine ett is extremely rare, and there is currently no consistent strategy for its treatment and management. Placental site trophoblastic tumors and epithelioid trophoblastic tumors. Epithelioid trophoblastic tumor ett derived from intermediate trophoblasts is one type of gestational trophoblastic neoplasia gtn, and it accounts for less than 2% of all gestational trophoblastic diseases gtd. Manifestations may include excessive uterine enlargement, vomiting, vaginal bleeding, and preeclampsia, particularly during early pregnancy. All three patients were of reproductive age and presented with vaginal bleeding and mild elevation of human chorionic gonadotropin hcg. Synchronous choriocarcinoma and epithelioid trophoblastic tumor.
It is considered a rare gestational trophoblastic disease and is frequently misdiagnosed as carcinoma. The patients with this neoplasm were in the reproductive age group and presented with abnormal vaginal bleeding. Pdf epithelioid trophoblastic tumor masquerading as. Treatments, outcomes, and potential therapeutic targets junjun yang 1, liju zong, jing wang2, xirun wan1, fengzhi feng 1, yang xiang 1. Nuclear positivity for p63 allowed differentiation from a placental site trophoblastic tumor. Sep 16, 2011 epithelioid sarcoma is a rare cancer that most often occurs in the soft tissue of the fingers, hands and forearms of young adults.
Epithelioid sarcoma genetic and rare diseases information. Mr imaging of uterine epithelioid trophoblastic tumor. Pdf primary epithelioid trophoblastic tumor with a. Gestational trophoblastic disease is proliferation of trophoblastic tissue in pregnant or recently pregnant women. Placentalsite trophoblastic tumorepithelioid trophoblastic tumor. Diagnostic challenges in cornual epithelioid trophoblastic tumor. Ett patients with advancedstage disease or an interval of. Researched pathways related to epithelioid trophoblastic tumor include pathogenesis, localization, cell proliferation, cell differentiation, cell adhesion. In both cases, the hysterectomy specimen confirmed the presence of two large epithelioid trophoblastic tumors arising in the endocervix and lower uterine segment with no extrauterine disease. Transformation of a postcesarean section placental site nodule into.
Epithelioid trophoblastic tumor of the lung urabe 2007. This analysis aimed to assess the effects of current management on clinical outcomes and to identify potential prognostic indicators in ett. Placental site trophoblastic tumor and epithelioid. Epithelioid trophoblastic tumour, ett immunohistochemistry. A surgical approach is recommended for ett patients with earlystage disease. Extrauterine epithelioid trophoblastic tumor arising in. It is a trophoblastic tumor of neoplastic chorionic type associated with the intermediate trophoblast. Primary epithelioid trophoblastic tumor with a synchronous breast carcinoma detected only with fdgpetct scan. A single institution case series at the new england trophoblastic disease center. Protocol for the examination of specimens from patients with primary gestational trophoblastic malignancy. There is a wide range in patient age at presentation, but it mainly occurs in the reproductive years, with a mean age of 30 years.
Gestational trophoblastic disease gtd is a rare group of disorders, both benign and malignant, that affect women of childbearing age. An intermediate trophoblast is a distinctive trophoblastic cell population from which four trophoblastic lesions are thought to arise. Epithelioid sarcoma is a rare soft tissue sarcoma in young adults 2039 year olds involving the upper extremities 60% of the time. Pdf historical, morphological and clinical overview of placental. Extrauterine gestational trophoblastic neoplasms include metastasis of an unknown uterine choriocarcinoma or an epithelioid trophoblastic tumor ett, which may. Ett can be a sequela of any gestational event and can present in both intrauterine and extrauterine sites. In the present report a case of uterine ett that was diagnosed in a. You will find basic information about this group of diseases and the parts of the body they may affect. The pathology of intermediate trophoblastic tumors and tumorlike lesions. Gestational trophoblastic disease gynecology and obstetrics.
Seven cases of epithelioid trophoblastic tumor treated in the womens hospital of zhejiang university from 2004 september to 2008 december were retrospectively analyzed. Its presentation in postmenopausal women is extremely rare. Extrauterine epithelioid trophoblastic tumor has been reported in multiple anatomical sites. Gestational trophoblastic disease gtd is a heterogeneous group of interrelated lesions and is biologically unique for several reasons. Epithelioid trophoblastic tumor of uterus presenting as an.
Although diagnosis was usually associated with a gestational event, the latter was sometimes remote. Pdf epithelioid trophoblastic tumor of the endocervix. Accurate diagnosis is important for clinical management of the patient. This tumour occurs in women of reproductive age some years after a gestation, which may be a term delivery, spontaneous abortion or molar gestation. Pdf placental site nodules psns and epithelioid trophoblastic tumors etts respectively represent. The reported gestational trophoblastic tumors mostly were hydatidiform mole. Epithelioid glioblastoma radiology reference article.
For accreditation purposes, this protocol should be used for the following procedures and tumor types. This disease can occur during or after an intrauterine or ectopic pregnancy. Epithelioid trophoblastic tumor is a rare neoplasm and represents the most recent addition to the gestational trophoblastic tumor category. Epithelioid trophoblastic tumor ett is an extremely rare form of gestational trophoblastic neoplasia gtn representing less than 2% of all gestational trophoblastic diseases 1. An epithelioid trophoblastic tumor ett is a rare trophoblastic tumor originating from chorionictype intermediate trophoblasts. Jan 20, 2010 the objective of this study is to evaluate the clinicopathological features and immunohistochemical characteristics of epithelioid trophoblastic tumor ett. Methods we applied a literature search using pubmed to analyze the clinical data of 78. Epithelioid trophoblastic tumor is an unusual type of trophoblastic tumor. Herein, we report a rare case of an epithelioid trophoblastic tumor of the uterus with radiologicpathologic correlation in a. The study of epithelioid trophoblastic tumor has been mentioned in research publications which can be found using our bioinformatics tool below. Etts exhibit slow growth and remain confined to the uterus for extended periods of time. The prognosis of the tumor is dependent upon several factors and is generally guarded the prognosis of similar tumors depend on a combination of factors, such as initial detection of the tumor, size and location, whether it has metastasized, its response. It elaborates a reliable and specific tumor marker, human chorionic gonadotropin hcg.
The cells that form gestational trophoblastic tumours are called trophoblasts and come from tissue that grows to form the placenta during pregnancy. Epithelioid trophoblastic tumor ett is an extremely rare potentially highrisk gestational trophoblastic neoplasia. Gestational trophoblastic disease poliklinika harni. Who classification of tumours of female reproductive organs, 4th edition, 2014. This report describes the clinicopathologic and immunohistochemical features of 14 cases of epithelioid trophoblastic tumor ett, a distinctive but rare gestational trophoblastic tumor. In the absence of tissue for a definitive histopathologic diagnosis, disease diagnosed as a result of persistent elevation of human chorionic gonadotropin hcg. 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. A rare postgestational trophoblastic tumour composed of epithelioid cells epidemiology. Case report epithelioid trophoblastic tumor after induced abortion. Ab this report describes the clinicopathologic and immunohistochemical features of 14 cases of epithelioid trophoblastic tumor ett, a distinctive but rare gestational trophoblastic tumor. Gestational trophoblastic disease treatment pdq health. Epidemiology it typically presents in women of reproductive age peak age. Epithelioid trophoblastic tumor is a neoplasm of the chorionictype intermediate trophoblasts.
Webpathology is a free educational resource with 10214 high quality pathology images of benign and malignant neoplasms and related entities. An epithelioid trophoblastic tumor ett is an extremely rare form of trophoblastic neoplasm. The number of mitotic figures varies, but at least a low level of mitotic activity is present. Hydatidiform mole hm is the most common type of gtd. Unlimited viewing of the articlechapter pdf and any associated supplements and figures. Large epithelioid tumours are common and represent a large differential diagnosis when viewed generally malignant epithelioid lesions are often carcinomas. Two weeks after the biopsy was performed, the tumor. Epithelioid trophoblastic tumors etts are resistant to chemotherapy mitotic count of 610 hpf is an unfavorable prognostic factor am j obstet gynecol. Synchronous choriocarcinoma and epithelioid trophoblastic. Epithelioid trophoblastic tumor ett is a rare gestational trophoblastic neoplasm of chorionictype intermediate trophoblasts, and it is most frequently located in the lower uterine segment and endocervix. Gestational trophoblastic tumor genetic and rare diseases. Biology, natural history, and treatment modalities.
Gestational trophoblastic disease gtd is a group of rare diseases in which abnormal trophoblast cells grow inside the uterus after conception. Vaginal involvement in ett in the form of isolated lesions has not. Pdf epithelioid trophoblastic tumor alexandros rouvas. Epithelioid trophoblastic tumor radiology reference article. Mar 01, 2011 the tumor always follows pregnancy, most often molar pregnancy hydatidiform mole. Gestational trophoblastic tumors include 3 distinct entities. Here we report on the clinicopathologic and immunohistochemical features of three cases of epithelioid trophoblastic tumor. A male in his 70s was admitted to our hospital for evaluation of a growing tumor in his left axilla. Epithelioid trophoblastic tumor radiology reference. 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 trophoblastic cells instead of the typical dimorphic pattern of choriocarcinoma. It usually occurs in women in reproductive age group. Placental site trophoblastic tumor and epithelioid trophoblastic tumor are rare gestational trophoblastic neoplasias.
Epithelioid trophoblastic tumor ett is a rare gestational trophoblastic tumor and often poses a diagnostic and therapeutic challenge to the involved clinicians. A 40yearold woman presented to our institution with abnormal uterine bleeding, elevated. These tumours are rare, and they appear when cells in the womb start to proliferate uncontrollably. 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. Pstt components, both type of tumor cells merged imperceptibly. The name was given by enzinger in 1970 to a group of soft tissue sarcomas that were confused with a variety of malignant and benign conditions, especially granulomatous process, synovial sarcoma and ulcerating squamous cell carcinoma. Clinical characteristics and outcomes of extrauterine. Epithelioid trophoblastic tumor ett is a new entity of trophoblastic tumor and 14 such cases were reported by shih and kurman in 1998. She underwent a thoracoscopic lobectomy after further staging and workup revealed no other abnormalities. Pathologic examination of the lung mass revealed an epithelioid trophoblastic tumor. Pdf technical insights into highly sensitive isolation. Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by dail and liebow as an aggressive bronchoalveolar cell carcinoma. Epithelioid trophoblastic tumor ett is an extremely rare subtype of gestational trophoblastic neoplasia gtn, accounting for only 1. It is rare in young children and adults, and it occurs more frequently in men.
The patients with this neoplasm were in the reproductive age. Epithelioid trophoblastic tumor of the uterus in a postmenopasual woman. Two of the 14 patients presented with extrauterine ett without evidence of prior gestational trophoblastic disease in the uterus. Epithelioid trophoblastic tumor is a rare gestational trophoblastic neoplasm usually presenting in women of reproductive age, with a history of a prior gestational event. Signs of gtd include abnormal vaginal bleeding and a uterus that is larger than normal. It derives from intermediate trophoblastic cells of chorion laeve, while placentalsite trophoblastic tumor. Protocol for the examination of specimens from patients.
Epithelioid trophoblastic tumor ett is a rare type of gestational trophoblastic disease and only 25 cases have been reported so far. Placentalsite trophoblastic tumors pstt and epithelioid trophoblastic tumors ett are the rarest forms of gtd. Epithelioid trophoblastic tumor ett is a relatively recently described entity, distinct from placental site trophoblastic tumor pstt and choriocarcinoma cc, with a. Dec 02, 2012 epithelioid trophoblastic tumor ett is a rare gestational trophoblastic tumor. It is considered as a rare subtype of gestational trophoblastic disease 2,4. This article examines the subtypes of gtd, and their treatment. Epithelioid trophoblastic tumor, the most recent addition to gestational trophoblastic diseases, is a rare but distinctive proliferative lesion of the putative chorionictype intermediate. Initially described as atypical choriocarcinoma, shih and kurman first reported the clinicopathological features of ett in 1998 3, and the world health organization 4. Or, you can choose another section to learn more about a specific question you have. Pdf transformation of a postcesarean section placental site. Department of obstetrics and gynecology, peking union medical college hospital, chinese academy of medical sciences, beijing 100730, pr china. Patients diagnosed at early stage should be treated surgically with adjuvant chemotherapy reserved for high risk cases. Placental site nodules psns and epithelioid trophoblastic tumors etts. Gestational choriocarcinoma is the most common gestational trophoblastic tumor.