OBJECTIVE: To analyze the genetic background of tumors in patients wit
h placental site trophoblastic tumors (PSTTs) and clinical outcome in
patients treated for this rare variant of trophoblastic disease at a s
ingle center. STUDY DESIGN: Analysis of all patients with PSTTs treate
d at the Charing Cross Hospital between 1975 and 1995. RESULTS: We stu
died the molecular genetics of a group of PSTTs using polymerase chain
reaction allelotyping and GeneScan software. We were able to show, in
the seven cases in which detailed molecular analysis was successful,
that four of these PSTTs were from diploid, biparental pregnancies and
three were androgenetic tumors following monospermic complete hydatid
iform moles. For patients with PSTT localized to the uterus, the treat
ment of choice is hysterectomy. The sensitivity of PSTT to current cyt
otoxic chemotherapy is variable. Several patients have been cured usin
g the etoposide, methotrexate, actinomycin D/cyclophosphamide, vincris
tine schedule, but clinical impressions suggest that cisplatinum proba
bly should be introduced into the chemotherapy schedule from the outse
t in a schedule such as etoposide, cisplatin/etoposide, methotrexate,
actinomycin D. CONCLUSION: PSTT is a very rate variant of gestational
trophoblastic tumor, and its biologic behavior is clearly heterogeneou
s. The treatment of choice for patients whose disease is limited to th
e uterus is hysterectomy; for patients with more extensive or metastat
ic disease, chemotherapy is indicated, but the clinical outcome is var
iable. A long interval from the antecedent pregnancy to clinical prese
ntation is a major adverse prognostic variable, and the outcome in pat
ients whose last known pregnancy was > 2 years prior to presentation w
ith PSTT is poor.