Placental site trophoblastic tumor

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Placental site trophoblastic tumor (PSTT) is rare and one of the least common (~ 0.2% ) forms of the gestational trophoblastic disease (GTD).

Epidemiology

PSTT typically occurs in women of reproductive age with the average age around 30. It may occur after a normal pregnancy, molar pregnancy or even after a terminated pregnancy. Time to the presentation can widely vary between 1 week and 14 years.

Clinical presentation

Presenting symptoms can include either abnormal vaginal bleeding and amenorrhea . Up to 30% of patients may present with metastases at the time of diagnosis .

Pathology

PSTT is composed of neoplastic transformation of intermediate trophoblastic cells that form a polypoid mass that may be within the endometrial canal or the myometrium.

It may be divided into two main types depending on vascularity :

  • hypervascular
  • relatively hypovascular
Markers
  • in contrast to other forms of the gestational trophoblastic disease, PSTT produces small amounts of ß-human chorionic gonadotropin; this is thought to be due to relative lack of syncytiotrophoblastic tissue within a tumor
  • human placental lactogen (hPL) may be increased

Radiographic features

There can be some imaging overlap with an invasive mole and choriocarcinoma. One of the key roles of imaging is to attempt to clarify the vascularity of a tumor.

Pelvic ultrasound
  • content needed
Pelvic MRI

Placental site trophoblastic tumor may present as a heterogenous endometrial + myometrial mass although appearances on MRI can be non-specific. The junctional zone is disrupted. In the majority of the cases, there are cystic spaces and vascular structures.

Reported signal characteristics include :

  • T1: typically isointense compared with healthy myometrium
  • T2: isointense to slightly hyperintense compared with myometrium

Treatment and prognosis

A hysterectomy is the primary mode of treatment in the majority of cases . A tumor tends to remain confined to the uterus until late in its course although can eventually metastasize to lung, liver, lymph nodes, and brain. Metastases can occur many years after the initial diagnosis .

Their biological behavior is variable, ranging from benign lesions confined to the uterus, to highly aggressive malignant disease with systemic metastases. The main negative prognostic variables are time to presentation from last known pregnancy and mitotic index.

While there can be a good prognosis with localized disease, the tumors tend to be less sensitive to chemotherapy than other forms of gestational trophoblastic disease . However, chemotherapy may still play a role with lesions that are not amenable for curative surgery .

History and etymology

First described by R J Kurman et.al in 1976 as a "trophoblastic pseudo-tumor". The term PSTT was later coined by R E Scully and R H Young in 1981 .

Differential diagnosis

For MRI appearances consider

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