Epithelioid Trophoblastic Tumor: Review of a Rare Neoplasm of the Chorionic-Type Intermediate Trophoblast

2006 ◽  
Vol 130 (12) ◽  
pp. 1875-1877
Author(s):  
Kimberly H. Allison ◽  
Jason E. Love ◽  
Rochelle L. Garcia
Keyword(s):  
Gestational Trophoblastic Disease ◽  
Reproductive Age ◽  
Placental Lactogen ◽  
Variable Expression ◽  
Trophoblastic Tumor ◽  
Trophoblastic Cells ◽  
Epithelioid Trophoblastic Tumor ◽  
Positive Immunostaining ◽  
Nodular Growth ◽  
Type Intermediate

Abstract We present a brief review of epithelioid trophoblastic tumor, a rare trophoblastic neoplasm derived from chorionic-type intermediate trophoblastic cells that typically presents in reproductive-age women between 1 and 18 years following a previous gestation. Histologic features include a nodular growth pattern of monomorphic, epithelioid cells within a hyaline matrix. Areas of necrosis and mitotic activity (0–9 mitoses per 10 high-power fields) are additional features of this neoplasm. Positive immunostaining for p63 and cytokeratin, frequent location in the lower uterine segment and endocervix, as well as the epithelioid appearance can lead to confusion with squamous cell carcinoma. Inhibin-α is typically expressed, as well as focal, more variable expression of other trophoblastic markers including β-human chorionic gonadotropin, human placental lactogen, placental alkaline phosphate, and Mel-CAM (CD148). The clinical behavior of this rare form of gestational trophoblastic disease is difficult to predict. Although most cases follow a benign course following resection, there is a potential for metastatic disease.

scholarly journals Uterine Epithelioid Trophoblastic Tumor in a 44-Year-Old Woman: A Diagnostic Dilemma

2021 ◽  
Vol 11 (3) ◽  
pp. 631-639
Author(s):  
Maria-Gabriela Aniţei ◽  
Diana-Elena Lazăr ◽  
Raluca Alina Pleşca ◽  
Cristina Terinte ◽  
Radu Iulian ◽  
...  
Keyword(s):  
Reproductive Age ◽  
Diagnostic Challenge ◽  
Diagnostic Dilemma ◽  
Natural Childbirth ◽  
Trophoblastic Tumor ◽  
Epithelioid Trophoblastic Tumor ◽  
Uterine Curettage ◽  
Normal Human ◽  
Poorly Differentiated ◽  
Β Hcg

Background: Epithelioid trophoblastic tumor (ETT) is a rare and newly defined disease, which most commonly occurs in women of reproductive age and can be a sequela of any gestational event. ETT can be present in both intrauterine and extrauterine sites. Case report: A woman of reproductive age, without specific comorbidities and with a single pregnancy and natural childbirth eight years ago, was diagnosed initially with poorly differentiated pleomorphic leiomyosarcoma on the hemostatic uterine curettage. Conclusion: Our case highlights that ETT presents a diagnostic challenge due to its rarity and histologic resemblance to other pathologies. Misdiagnosis delays effective treatment and affects survival. To date, only 8 cases of ETT of the uterus without previous gestational event and normal human chorionic gonadotropin (β-HCG) levels in a 60-year literature survey have been reported.

scholarly journals A Rare Gestational Trophoblastic Disease: Placental Site Trophoblastic Tumor

2016 ◽  
Author(s):  
Senem Yaman Tunç ◽  
Elif Ağaçayak ◽  
Mehmet Sait İçen ◽  
Serdar Başaranoğlu ◽  
Mehmet Sıddık Evsen ◽  
...  
Keyword(s):  
Uterine Cavity ◽  
Total Abdominal Hysterectomy ◽  
Abdominal Hysterectomy ◽  
Gestational Trophoblastic Disease ◽  
Placental Site Trophoblastic Tumor ◽  
Trophoblastic Tumor ◽  
Trophoblastic Cells ◽  
Placental Site ◽  
Gestational Trophoblastic Diseases ◽  
Abnormal Vaginal Bleeding

<p>Placental site trophoblastic tumor (PSTT) is a highly rare form of gestational trophoblastic diseases that arise from intermediate trophoblastic cells. By presenting this case, we aimed to review the treatment and diagnosis, approach to PSTT.<br />A 31-year-old (G2P1A1L1) patient had abnormal vaginal bleeding. Serum ß-HCG was 5.82 mIU/ml and the transvaginal USG detected a polypoid mass in uterine cavity. Probe curettage was performed. Histopathologic specimens were confirmed as PSTT. No metastasis was detected. A total abdominal hysterectomy was performed.<br />PSTT is a rare tumor. In contrast to other trophoblastic tumors, PSTT produces a small amount of ß-HCG and it is relatively insensitive to chemotherapy. Adjuvant chemotherapy is suggested to follow surgical treatment in the cases with metastasis.</p>

Epithelioid Trophoblastic Tumor: An Outcome-Based Literature Review of 78 Reported Cases

2013 ◽  
Vol 23 (7) ◽  
pp. 1334-1338 ◽  
Author(s):  
Xiaofei Zhang ◽  
Weiguo Lü ◽  
Bingjian Lü
Keyword(s):  
Squamous Cell Carcinoma ◽  
Cell Carcinoma ◽  
Squamous Cell ◽  
Figo Stage ◽  
Lower Uterine Segment ◽  
Reproductive Age ◽  
Small Data ◽  
Data Set ◽  
Trophoblastic Tumor ◽  
Epithelioid Trophoblastic Tumor

ObjectivesEpithelioid trophoblastic tumor (ETT) is very rare; and therefore, a substantially increased data set is unlikely to be obtained in the near future. This analysis aimed to assess the effects of current management on clinical outcomes and to identify potential prognostic indicators in ETT.MethodsWe applied a literature search using PubMed to analyze the clinical data of 78 published cases of ETT.ResultsWomen with ETT present at reproductive age (mean ± SD, 37.1 ± 8.7 years) and have a slightly to moderately elevated serum β-human chorionic gonadotropin (median, 665 IU/L). Epithelioid trophoblastic tumor is frequently present in the lower uterine segment/cervix (26/58 cases) and can be misdiagnosed as squamous cell carcinoma (6/26). Lung is the most common extrauterine site of ETT (5/11 with uterine ETT and 10/20 without uterine ETT). Kaplan-Meier analysis indicates that chemotherapy (surgery with postoperative chemotherapy vs surgery alone) is associated with increased ETT relapse (P= 0.005), even after stratification by International Federation of Gynecology and Obstetrics (FIGO) stage (P= 0.008); but FIGO stage remains the only significant prognostic indicator for ETT (P= 0.015).ConclusionsThis analysis confirms the hypothetical chemotherapy resistance and prognostic value of FIGO staging in ETT. These findings remain tentative given the small data set available for analysis and the reporting bias from these published cases; however, they may confer a risk-adapted therapy. Finally, both gynecologists and pathologists should be alert to the potential misdiagnosis of squamous cell carcinoma when ETT is present in the lower uterine segment/cervix.

scholarly journals Epithelioid Trophoblastic Tumor: A Case Report and Review of the Literature

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Eirwen M. Scott ◽  
Ashlee L. Smith ◽  
Mohamed Mokhtar Desouki ◽  
Alexander B. Olawaiye
Keyword(s):  
Reproductive Age ◽  
Women Of Reproductive Age ◽  
Diagnostic Challenge ◽  
Postoperative Evaluation ◽  
Trophoblastic Tumor ◽  
Laparoscopic Assisted Vaginal Hysterectomy ◽  
Epithelioid Trophoblastic Tumor ◽  
Abnormal Vaginal Bleeding ◽  
Laparoscopic Assisted ◽  
Disease Free

Epithelioid trophoblastic tumor (ETT) is a rare gestational trophoblastic tumor. Cases of ETT present as abnormal vaginal bleeding in women of reproductive age, with low human chorionic gonadotropin (hCG) levels. ETT can be a sequela of any gestational event and can present in both intrauterine and extrauterine sites. Metastasis and death have been reported. We present a case of a 44-year-old female incidentally diagnosed with ETT following laparoscopic-assisted vaginal hysterectomy. Postoperative evaluation for metastatic disease was negative. The patient has been closely followed and remains disease free 8 months postoperatively. ETT presents a diagnostic challenge due to its rarity and histologic resemblance to other pathologies. ETT is relatively chemoresistant and managed surgically. Misdiagnosis delays effective treatment and affects survival.

scholarly journals Fertility and pregnancy outcome in gestational trophoblastic disease

2021 ◽  
Vol 31 (3) ◽  
pp. 399-411
Author(s):  
Ulrika Joneborg ◽  
Leonoor Coopmans ◽  
Nienke van Trommel ◽  
Michael Seckl ◽  
Christianne A R Lok
Keyword(s):  
Pregnancy Outcome ◽  
Single Agent ◽  
Gestational Trophoblastic Disease ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Gestational Trophoblastic Neoplasia ◽  
Trophoblastic Tumor ◽  
Epithelioid Trophoblastic Tumor ◽  
Placental Site ◽  
Multi Agent

The aim of this review is to provide an overview of existing literature and current knowledge on fertility rates and reproductive outcomes after gestational trophoblastic disease. A systematic literature search was performed to retrieve all available studies on fertility rates and reproductive outcomes after hydatidiform mole pregnancy, low-risk gestational trophoblastic neoplasia, high- and ultra-high-risk gestational trophoblastic neoplasia, and the rare placental site trophoblastic tumor and epithelioid trophoblastic tumor forms of gestational trophoblastic neoplasia. The effects of single-agent chemotherapy, multi-agent including high-dose chemotherapy, and immunotherapy on fertility, pregnancy wish, and pregnancy outcomes were evaluated and summarized. After treatment for gestational trophoblastic neoplasia, most, but not all, women want to achieve another pregnancy. Age and extent of therapy determine if there is a risk of loss of fertility. Single-agent treatment does not affect fertility and subsequent pregnancy outcome. Miscarriage occurs more often in women who conceive within 6 months of follow-up after chemotherapy. Multi-agent chemotherapy hastens the natural menopause by three years and commonly induces a temporary amenorrhea, but in young women rarely causes permanent ovarian failure or infertility. Subsequent pregnancies have a high chance of ending with live healthy babies. In contrast, high-dose chemotherapy typically induces permanent amenorrhea, and no pregnancies have been reported after high-dose chemotherapy for gestational trophoblastic neoplasia. Immunotherapy is promising and may give better outcomes than multiple schedules of chemotherapy or even high-dose chemotherapy. The first pregnancy after immunotherapy has recently been described. Data on fertility-sparing treatment in placental site trophoblastic tumor and epithelioid trophoblastic tumor are still scarce, and this option should be offered with caution. In general, patients with gestational trophoblastic neoplasia may be reassured about their future fertility and pregnancy outcome. Detailed registration of high-risk gestational trophoblastic neoplasia is still indispensable to obtain more complete data to better inform patients in the future.

Epithelioid trophoblastic tumor: a case report

2006 ◽  
Vol 16 (3) ◽  
pp. 1473-1476 ◽  
Author(s):  
C. Lo ◽  
I. Low ◽  
A. L. Tan ◽  
J. Baranyai
Keyword(s):  
Gestational Trophoblastic Disease ◽  
Human Chorionic Gonadotrophin ◽  
Trophoblastic Disease ◽  
Trophoblastic Tumor ◽  
Uncomplicated Pregnancy ◽  
Epithelioid Trophoblastic Tumor ◽  
Trophoblastic Tumors ◽  
Clinicopathologic Findings ◽  
Tumor Like Lesions ◽  
Distinguishing Features

Epithelioid trophoblastic tumor is a rare and distinctive pathologic entity within the complex family of gestational trophoblastic disease. We describe a case of epithelioid trophoblastic tumor occurring in a 34-year-old woman, who presented with a large uterine tumor 3 years following an uncomplicated pregnancy. The clinicopathologic findings in this case are typical of this unusual entity and consistent with current literature, with the exception of negative beta-human chorionic gonadotrophin levels. The distinguishing features from other intermediate trophoblastic tumors and tumor-like lesions are discussed.

Spectrum of Pathological Lesions in Uterine Specimens with Focus on Gestational Trophoblastic Disease in Karachi

2020 ◽  
Keyword(s):  
Hydatidiform Mole ◽  
Endometrial Stromal Sarcoma ◽  
Gestational Trophoblastic Disease ◽  
Cross Sectional Study ◽  
Reproductive Age ◽  
P Value ◽  
Cross Sectional ◽  
Endometrial Polyps ◽  
Trophoblastic Disease ◽  
Proliferative Endometrium

Background: The most common benign pathological lesion in women of reproductive age is uterine leiomyoma. Gestational trophoblastic disease includes tumors and tumor like lesions originating from trophoblastic tissue. The aim of this study was to find the spectrum of molar pregnancy and uterine pathologies focusing on gestational trophoblastic disease as no study has been done in the past few years. Methods: Endometrial and uterine specimens of patients (n=436) between the ages of 15-65 years were collected from a private hospital in Karachi from December 2018 to December 2019. This cross-sectional study was carried out by pathological diagnosis of patients’ samples under light microscopy using hematoxylin and eosin staining. Stratification was done about age and nature of specimen to control the effect modifiers. The post stratification Chi square test was applied and p value <0.05 was considered significant. Results: Mean age of the patients was 36.1 years ±7.8. Total 436 uterine biopsies included 260(59.6%) hysterectomies, 56(12.8%) endometrial curetting’s, 117(26.8%) evacuation specimens and 3(0.7%) polypectomies. Common pathologies included 124(28.4%) leiomyomas, 61(14%) proliferative endometrium, 52(11.9%) adenomyosis and 32(7.3%) endometrial polyps. Gestational trophoblastic disease was seen in 9(2.06%). Seven (87.5%) were partial hydatidiform moles, one (12.5%) exaggerated placental site reaction and one choriocarcinoma. Mole was common between 26-30 years with mean age of 27.2 years and prevalence was 6/100 abortions. Conclusion: Leiomyoma was the commonest (28.4%) uterine pathology followed by proliferative endometrium (14.5%). However, endometrial stromal sarcoma and endometriosis were found 0.2% each. High prevalence of mole was seen in this study. Partial mole was most common and choriocarcinoma was least common. Keywords: Hydatidiform Mole; Pathology; Prevalence.

scholarly journals Epithelioid trophoblastic tumor coexisting with choriocarcinoma around an abdominal wall cesarean scar: a case report and review of the literature

2020 ◽  
Vol 14 (1) ◽  
Author(s):  
Chunfeng Yang ◽  
Jianqi Li ◽  
Yuanyuan Zhang ◽  
Hanzhen Xiong ◽  
Xiujie Sheng
Keyword(s):  
Abdominal Wall ◽  
Chorionic Gonadotropin ◽  
Human Chorionic Gonadotropin ◽  
Unknown Origin ◽  
Exploratory Laparotomy ◽  
Cesarean Scar ◽  
Trophoblastic Tumor ◽  
Epithelioid Trophoblastic Tumor ◽  
Trophoblastic Tumors ◽  
Wall Lesion

Abstract Background Mixed gestational trophoblastic neoplasms are extremely rare and comprise a group of fetal trophoblastic tumors including choriocarcinomas, epithelioid trophoblastic tumors, and placental site trophoblastic tumors. We present a case of a patient with extrauterine mixed gestational trophoblastic neoplasm adjacent to the abdominal wall cesarean scar. On the basis of a literature review, this type of case has never been reported before due to the unique lesion location and low incidence. Case presentation Our patient was a 39-year-old Chinese woman who had a history of two cesarean sections and one miscarriage. She had a recurrent anterior abdominal wall mass around her cesarean scar, and the mass was initially suspected of being choriocarcinoma of unknown origin. The patient had concomitant negative or mildly increased serum β-human chorionic gonadotropin at follow-up and no abnormal vaginal bleeding or abdominal pain. However, she underwent local excision twice and had two courses of chemotherapy with an etoposide and cisplatin regimen. She finally opted for exploratory laparotomy with abdominal wall lesion removal, subtotal hysterectomy, bilateral salpingectomy, and left ovarian cyst resection, which showed the abdominal wall lesion, whose components were revealed by microscopy and immunohistochemical staining to be approximately 90% epithelioid trophoblastic tumors and 10% choriocarcinomas from a solely extrauterine mixed gestational trophoblastic neoplasm around an abdominal wall cesarean scar. Conclusions It is worth noting whether epithelioid trophoblastic tumor exists in the setting of persistent positive low-level β-human chorionic gonadotropin. More studies are required to provide mechanistic insights into these mixed gestational trophoblastic neoplasms.

scholarly journals “It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case

Lupus ◽  
2021 ◽  
pp. 096120332098176
Author(s):  
Sarah J van der Lely ◽  
Jeffrey Boorsma ◽  
Marc Hilhorst ◽  
Jesper Kers ◽  
Joris Roelofs ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Gestational Trophoblastic Disease ◽  
Grand Round ◽  
Placental Site Trophoblastic Tumor ◽  
Trophoblastic Tumor ◽  
Anchoring Bias ◽  
Placental Site ◽  
History Of

Introduction: Placental site trophoblastic tumor (PSTT) is a rare subtype of gestational trophoblastic disease. Association of PSTT and nephrotic syndrome is exceedingly rare and has been described in 8 cases thus far. In all cases hysterectomy was performed within months after onset of symptoms, leading to immediate remission of nephrotic syndrome, except for one patient who died of complications of PSTT. Case: We describe the history of a woman in which PSTT was discovered years after onset of nephrotic syndrome. Kidney biopsy revealed lupus-like mesangiocapillary nephritis and over time the patient developed additional symptoms mimicking systemic lupus erythematosus (SLE). Discussion: We provide an overview of the literature on this clinical entity and elaborate on its pathophysiology. In addition, we reflect on the phenomenon of anchoring bias, that led physicians to assume the patient had SLE without questioning this diagnosis in the light of the unexplained finding of increased tumor markers.

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