Epithelioid trophoblastic tumour: Difference between revisions

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{{more footnotes|date=September 2017}}
{{Short description|A rare type of gestational trophoblastic disease}}
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'''Epithelioid trophoblastic tumor''' (ETT) is a [[gestational trophoblastic disease]] with about 110 case reports in the literature. It is a trophoblastic tumor of neoplastic chorionic type associated with the intermediate [[trophoblast]].
'''Epithelioid trophoblastic tumour''' (ETT) is a rare form of [[gestational trophoblastic disease]] (GTD), which is a group of rare tumors that involve abnormal growth of cells inside a woman's uterus. ETT is characterized by the proliferation of intermediate trophoblastic cells that resemble carcinoma cells.


==Signs and symptoms==
==Pathophysiology==
* Vaginal bleeding: The most common presenting symptom is [[vaginal bleeding]], which is associated with mild elevation of serum β hCG (< 2,500 IU/l)
ETT arises from the [[intermediate trophoblast]], which is a type of cell that forms part of the [[placenta]] during pregnancy. These cells are responsible for anchoring the placenta to the uterine wall. In ETT, these cells undergo neoplastic transformation, leading to the formation of a tumor. The tumor cells in ETT are typically monomorphic and epithelioid in appearance, which means they resemble epithelial cells.
* [[Amenorrhea]].
<ref>http://www.pathologyoutlines.com/topic/placentaETT.html clinical features</ref>


==Morphology==
==Clinical Presentation==
Patients with ETT may present with irregular [[vaginal bleeding]], which is the most common symptom. Other symptoms can include an enlarged uterus, pelvic pain, or symptoms related to metastasis if the disease has spread. ETT can occur months to years after a [[pregnancy]], including normal pregnancies, [[miscarriages]], or [[molar pregnancies]].


===Gross appearance===
* There is deep infiltration of the surrounding structures by cystic hemorrhagic masses or discrete nodules.
* [[Necrosis]] is present with white to tan-brown cut surface with hemorrhage.
* [[Ulceration]] (common finding)
* [[Fistula]] (common finding)
===Microscopic appearance===
* Nodular, well circumscribed, focal infiltrative at the periphery.
* Uniform, mononucleate tumor cells are arranged in nests and cords.
* Tumor nests are associated with osinophilic, fibrillar, hyaline-like material.
* Extensive necrosis with irregular contours.
* Calcification (common finding)
* Metaplastic endocervical or endometrial surface epithelium into squamous-like epithelium.
===Locations===
Common locations are:
*Uterus
**Lower uterine segment (40%)
**Cervix (31% of cases)
*Lungs (19% of cases)
They may rarely develop in
*Vagina
*Broad ligament
*Fallopian tubes
*Other pelvic organs
<ref name="ETT">Epithelioid trophoblastic tumor [http://www.pathologyoutlines.com/topic/placentaETT.html]. ''Pathology outlines'', August 26, 2017.</ref>
==Diagnosis==
==Diagnosis==
{{Empty section|date=February 2018}}
The diagnosis of ETT is challenging due to its rarity and the overlap of its features with other types of GTD. Diagnosis typically involves a combination of clinical evaluation, imaging studies, and histopathological examination. [[Ultrasound]] and [[MRI]] may be used to assess the extent of the disease. Histologically, ETT is characterized by nests and cords of monomorphic intermediate trophoblastic cells with abundant eosinophilic cytoplasm and distinct cell borders.


==Treatment==
==Treatment==
Epithelioid trophoblastic tumors (ETTs) are resistant to chemotherapy. Stage I disease is treated with hysterectomy, while metastatic disease is treated with surgery and chemotherapy. <ref name="ETT">Epithelioid trophoblastic tumor [http://www.pathologyoutlines.com/topic/placentaETT.html]. ''Pathology outlines'', August 26, 2017.</ref>
The primary treatment for ETT is surgical removal of the tumor, often through a [[hysterectomy]]. Due to the potential for metastasis, additional treatments such as [[chemotherapy]] may be considered, especially in cases where the disease has spread beyond the uterus. The choice of chemotherapy regimen can vary, but it often includes agents used in the treatment of other forms of GTD.


==Prognosis==
==Prognosis==
Metastasis occur in 25% of cases and 10% die of the disease. Mitotic count of > 6/10HPF is an unfavorable prognostic factor. <ref name="ETT">Epithelioid trophoblastic tumor [http://www.pathologyoutlines.com/topic/placentaETT.html]. ''Pathology outlines'', August 26, 2017.</ref>
The prognosis for patients with ETT depends on several factors, including the stage of the disease at diagnosis and the presence of metastasis. Early-stage ETT that is confined to the uterus and treated with surgery generally has a good prognosis. However, metastatic ETT can be more challenging to treat and may have a poorer outcome.


==References==
==Related pages==
{{reflist}}
* [[Gestational trophoblastic disease]]
* [[Choriocarcinoma]]
* [[Placental site trophoblastic tumor]]
* [[Hydatidiform mole]]


[[Category:Germ cell neoplasia]]
[[Category:Gestational trophoblastic disease]]
[[Category:Rare diseases]]
[[Category:Rare cancers]]
{{dictionary-stub1}}
{{No image}}

Revision as of 19:16, 22 March 2025

A rare type of gestational trophoblastic disease


Epithelioid trophoblastic tumour (ETT) is a rare form of gestational trophoblastic disease (GTD), which is a group of rare tumors that involve abnormal growth of cells inside a woman's uterus. ETT is characterized by the proliferation of intermediate trophoblastic cells that resemble carcinoma cells.

Pathophysiology

ETT arises from the intermediate trophoblast, which is a type of cell that forms part of the placenta during pregnancy. These cells are responsible for anchoring the placenta to the uterine wall. In ETT, these cells undergo neoplastic transformation, leading to the formation of a tumor. The tumor cells in ETT are typically monomorphic and epithelioid in appearance, which means they resemble epithelial cells.

Clinical Presentation

Patients with ETT may present with irregular vaginal bleeding, which is the most common symptom. Other symptoms can include an enlarged uterus, pelvic pain, or symptoms related to metastasis if the disease has spread. ETT can occur months to years after a pregnancy, including normal pregnancies, miscarriages, or molar pregnancies.

Diagnosis

The diagnosis of ETT is challenging due to its rarity and the overlap of its features with other types of GTD. Diagnosis typically involves a combination of clinical evaluation, imaging studies, and histopathological examination. Ultrasound and MRI may be used to assess the extent of the disease. Histologically, ETT is characterized by nests and cords of monomorphic intermediate trophoblastic cells with abundant eosinophilic cytoplasm and distinct cell borders.

Treatment

The primary treatment for ETT is surgical removal of the tumor, often through a hysterectomy. Due to the potential for metastasis, additional treatments such as chemotherapy may be considered, especially in cases where the disease has spread beyond the uterus. The choice of chemotherapy regimen can vary, but it often includes agents used in the treatment of other forms of GTD.

Prognosis

The prognosis for patients with ETT depends on several factors, including the stage of the disease at diagnosis and the presence of metastasis. Early-stage ETT that is confined to the uterus and treated with surgery generally has a good prognosis. However, metastatic ETT can be more challenging to treat and may have a poorer outcome.

Related pages