Malignant tumors that arise from within ectopic endometrium
are thought to be rare. One large series reported 1323 cases of endometriosis which they found malignancy
to arise in %0.9 of them[1
]. Thirty-six percent of those
endometriosis specimens were from the ovary, while only
2% were from uterosacral ligament. In the same study,
three extragonadal sarcomas arose in endometriosis were
Müllerian adenosarcoma arising from the extraovarian
sites have been described[2,3,5-9]. To our knowledge,
this is the first case of adenosarcoma arising from
endometriosis of the uterosacral ligament in the literature.
The histogenesis of the extrauteine müllerian adenosarcoma
is unclear. It may arise from a focus of endometriosis
as in this case. Müllerian adenosarcomas arising in
endometriosis can be incompletely diagnosed as endometriosis,
especially during an intraoperative consultation.
Clement and Scully have recommended that, adenosarcoma
can be diagnosed when one or more of the following criteria are present: 1) stromal mitotic count of
two or more per 10 HPF, 2) marked stromal cellularity, 3)
more than a mild degree of stromal atypia.
Extrauterine müllerian adenosarcomas occur in
younger women and are more aggressive than uterine
The main reason that patients with ovarian adenosarcomas
have poorer prognosis than those with uterine sarcomas
is probably related to the location of the tumors with respect to the abdominal cavity and the lack of an
anatomic barrier to spread.
Surgery is the mainstay of treatment. Müllerian adenosarcomas
in general can be expected to recur or metastasize
locally. In our case, external radiotherapy was
decided but she refused adjuvant therapy. The patient is
free of tumor one year postoperatively.
In conclusion, although it's rare, malignant degeneration
to adenosarcoma in endometriosis cases should be
kept in mind.