Vaginal carcinoma after hysterectomy for invasive hydatidiform mole: a diagnostic dilemma between epithelioid trophoblastic tumor and vaginal cancer

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.

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Coexisting Epithelioid Trophoblastic Tumor and Choriocarcinoma of the Uterus Following a Chemoresistant Hydatidiform Mole

Archives of Pathology & Laboratory Medicine ◽

10.5858/2003-127-e291-cettac ◽

2003 ◽

Vol 127 (7) ◽

pp. e291-e293

Author(s):

Dan-Hua Shen ◽

Ui Soon Khoo ◽

Hextan Y. S. Ngan ◽

Tung-Yau Ng ◽

Ming-Tak Chau ◽

...

Keyword(s):

Hydatidiform Mole ◽

Invasive Disease ◽

Chorionic Gonadotrophin ◽

Human Chorionic Gonadotrophin ◽

Immunohistochemical Examination ◽

Trophoblastic Tumor ◽

Epithelioid Trophoblastic Tumor ◽

Trophoblastic Tumors ◽

History Of ◽

Unusual Type

Abstract The epithelioid trophoblastic tumor is an unusual type of trophoblastic tumor. Herein, we describe a patient with coexisting epithelioid trophoblastic tumor and choriocarcinoma in the uterus. The patient had a history of hydatidiform mole with recurrent elevation of human chorionic gonadotrophin level that is resistant to chemotherapy. Histopathologic and immunohistochemical examination showed distinctive differences between the 2 trophoblastic tumors. The development of epithelioid trophoblastic tumor may be related to the persistence of locally invasive disease, which was unresponsive to chemotherapy. The patient responded well to surgery. The presence of an epithelioid trophoblastic tumor should be considered in chemoresistant gestational trophoblast tumor.

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

Journal of Medical Case Reports ◽

10.1186/s13256-020-02485-8 ◽

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.

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Gestational trophoblastic neoplasia: Retrospective analysis of clinical profile, treatment pattern and outcome

10.1055/s-0039-1685377 ◽

2016 ◽

Author(s):

Paramjeet Kaur ◽

Ashok K. Chauhan ◽

Anil Khurana ◽

Yashpal Verma ◽

Nupur Bansal

Keyword(s):

High Risk ◽

Risk Group ◽

Hydatidiform Mole ◽

Single Agent ◽

High Risk Group ◽

Treatment Pattern ◽

Gestational Trophoblastic Neoplasia ◽

Trophoblastic Tumor ◽

Villous Trophoblast ◽

Invasive Mole

Background: Gestational trophoblastic disease is a spectrum of cellular proliferation arising from the placental villous trophoblast. Gestational triphoblastic neoplasia (GTN) is a collective term for GTD that invade locally or metastasize. GTD includes hydatidiform mole (complete and partial) and GTN include invasive mole, choricocarcinoma, placental site trophoblastic tumor and epitheliod trophoblastic tumor. Aim: To evaluate clinicopathological profile, treatment pattern and clinical outcome in patients with gestational trophoblastic neoplasia (GTN). Materials and Methods: Twelve cases of gestational trophoblastic neoplasia treated between 2012 to November 2015 in deptt of Radiotherapy – II, PGIMS, Rohtak were evaluated in this retrospective study. Data was analyzed on the basis of age, histopathology, stage, type of treatment received and treatment related toxicities. Disease free survival was estimated. Results: Out of 12 women 7 (58 %) had hydatidiform mole, 4 (33%) invasive mole and 01 (8%) had choriocarcinoma. All the cases were given chemotherapy. Two patients had low risk disease. Among high risk group seven patients had score of less than 7 and five patients had risk score of 7 or higher. Five patients were given single agent methotrexate, seven patients received multidrug regimens. All patients are on regular follow up. One patient (high risk group) expired as she did not receive treatment. Conclusion: GTN are rare and proliferative disorders with proper diagnosis and treatment most of the cases are amenable to treatment with favorable outcome.

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Epithelioid trophoblastic tumor of uterus presenting as an ovarian mass: A diagnostic and therapeutic dilemma

Indian Journal of Pathology and Microbiology ◽

10.4103/0377-4929.41669 ◽

2008 ◽

Vol 51 (2) ◽

pp. 242 ◽

Cited By ~ 11

Author(s):

Tanuja Shet ◽

Mahendra Parage ◽

Amita Maheshwari ◽

Reena Nair ◽

Sudeep Gupta ◽

...

Keyword(s):

Ovarian Mass ◽

Trophoblastic Tumor ◽

Epithelioid Trophoblastic Tumor

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Laparoscopic diagnosis and treatment of an isolated epithelioid trophoblastic tumor in recto-uterine pouch

Journal of Obstetrics and Gynaecology Research ◽

10.1111/jog.13593 ◽

2018 ◽

Vol 44 (5) ◽

pp. 960-965 ◽

Cited By ~ 4

Author(s):

Fang Jiang ◽

Yang Xiang ◽

Li-na Guo

Keyword(s):

Diagnosis And Treatment ◽

Trophoblastic Tumor ◽

Epithelioid Trophoblastic Tumor

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

Archives of Pathology & Laboratory Medicine ◽

10.5858/2006-130-1875-ettroa ◽

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.

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Extrauterine Epithelioid Trophoblastic Tumor (ETT) Arising from an Antecedent Undiagnosed Molar Pregnancy Presenting as an Adnexal mass: A Case Report

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqab191.164 ◽

2021 ◽

Vol 156 (Supplement_1) ◽

pp. S78-S78

Author(s):

J Gallardo ◽

K Hummel ◽

K McCluskey ◽

R Masand ◽

J Sunde

Keyword(s):

Case Report ◽

Tumor Cells ◽

Dna Fingerprinting ◽

Adnexal Mass ◽

Molar Pregnancy ◽

Fingerprinting Analysis ◽

Trophoblastic Tumor ◽

Eosinophilic Cytoplasm ◽

Epithelioid Trophoblastic Tumor ◽

Β Hcg

Abstract Introduction/Objective Epithelioid trophoblastic tumor (ETT) is an extremely rare neoplasm derived from chorionic type intermediate trophoblast. ETT usually follows an antecedent term pregnancy but can also follow spontaneous abortions or molar pregnancy. ETT most often arises from the endometrium, followed by the cervix. Extrauterine ETT are extremely rare, with few cases reported in literature. Methods/Case Report A 41-year-old woman with three term pregnancies presented with abdominal pain, ten years after her last pregnancy. Imaging findings of a 3.5 cm adnexal mass coupled with an elevated serum β-hCG (~ 900 mIU/ml), led to the suspicion of an ectopic pregnancy. Hysterectomy with salpingectomy revealed a 4.7 cm, tan- yellow, necrotic mass in the adnexal region abutting but distinct from the uterine serosa. Histologic evaluation showed a well- circumscribed tumor with pushing borders. The tumor cells were epithelioid with well-defined eosinophilic cytoplasm, monomorphic nuclei, frequent mitosis, and abundant geographic necrosis. The tumor cells were positive for β-hCG, GATA-3, PLAP and inhibin, with focal weak staining squamous markers p63 and p40. DNA fingerprinting analysis, performed to confirm the diagnosis of ETT, revealed a homozygous tumor with two copies of non-maternal genes indicating that the antecedent index gestation giving rise to the tumor was an undiagnosed hydatidiform mole. Following surgery, serum β-hCG levels were normal and the patient is currently on surveillance. Results (if a Case Study enter NA) NA Conclusion We present an extremely rare case of extrauterine ETT arising from a previously undetected molar pregnancy. The diagnosis should be suspected when a mass is observed at extrauterine sites with elevated β-hCG levels in patients with or without vaginal bleeding. Histologic differential of squamous cell carcinoma needs to be ruled out with immunostains. Due to its rarity and highly variable presentation, this entity remains a diagnostic challenge. DNA fingerprinting analysis demonstrating non-maternal genes can help confirm the diagnosis of ETT.

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