Epithelioid trophoblastic tumour

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
SpecialtyOncology

Signs and symptoms
  • Vaginal bleeding: The most common presenting symptom is vaginal bleeding, which is associated with mild elevation of serum β hCG (< 2,500 IU/L)
  • Amenorrhea.

[1]

Morphology

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 eosinophilic, 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

[2]

Treatment

Epithelioid trophoblastic tumors (ETTs) are resistant to chemotherapy, but an immunotherapy drug called dostarlimab treats gestational trophoblastic neoplasia. ETT is a form of GTN. Stage I disease is treated with hysterectomy, while metastatic disease is treated with surgery and chemotherapy. [2]

Prognosis

Metastasis occur in 25% of cases and 10% die of the disease. Mitotic count of > 6/10HPF is an unfavorable prognostic factor. [2]

References
  1. http://www.pathologyoutlines.com/topic/placentaETT.html clinical features
  2. Epithelioid trophoblastic tumor . Pathology outlines, August 26, 2017.
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