Benifla, Jean-Louis

Bricou, Alexandre

Cortez, Annie

Demaria, Fabien

Marzouk, Paul

Sakr, Rita

English

PubMed

Rita Sakr

Paul Marzouk

Alexandre Bricou

Fabien Demaria

Annie Cortez

Jean-Louis Benifla

Crossref

Uterine adenosarcoma associated to lymphovascular emboli: a case report

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Case report
Open Access
Uterine adenosarcoma associated to lymphovascular emboli:
a case report
Rita Sakr1*, Paul Marzouk1, Alexandre Bricou1, Fabien Demaria1,
Annie Cortez2 and Jean-Louis Benifla1
Address: 1Department of Gynecology, Hôpital Armand-Trousseau, 75012 Paris, France and 2Department of Pathology, Hôpital Tenon, 75020 Paris,
France
Email: RS* - rita.sakr@trs.aphp.fr; ritasakr@yahoo.com; PM - paul.marzouk@trs.aphp.fr; AB - alexandre.bricou@trs.aphp.fr;
FD - fabien.demaria@trs.aphp.fr; AC - annie.cortez@tnn.aphp.fr; JLB - jl.benifla@trs.aphp.fr
*Corresponding author
Published: 18 May 2009 Received: 19 February 2008
Accepted: 29 April 2009
Cases Journal 2009, 2:7515 doi: 10.1186/1757-1626-2-7515
This article is available from: http://casesjournal.com/casesjournal/article/view/7515
© 2009 Sakr et al; licensee Cases Network Ltd.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0),
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Introduction: Uterine adenosarcoma is a rarely observed polypoid tumor with a mixed benign
epithelial element and malignant stromal component. The treatment is total hysterectomy with
bilateral salpingo-oophorectomy. It could be difficult to diagnose and associated to lymphovascular
invasion.
Case presentation: A 45-year-old caucasian uniparous woman presented with uterine bleeding.
She had several surgical procedures and pathology of removed recurrent polyps showed no
malignancy. Finally, a total abdominal hysterectomy was performed because of atypical cells and
suspected uterine adenosarcoma. The hysterectomy specimen confirmed the presence of uterine
adenosarcoma associated with lymphatic and vascular tumor emboli. Surgery was completed with a
second bilateral salpingo-oophorectomy and pelvic lymphadenectomy.
Conclusion: In our report, we present a case of uterine adenosarcoma which was diagnosed after
multiple surgical procedures and associated to lymphovascular emboli known to have a significant
impact on overall survival and distant metastasis-free survival.
Introduction
Uterine adenosarcoma is a mixed epithelial and mesench-
ymal tumor of the uterus with a benign epithelial element
and a malignant stromal component. The lesion is
polypoid and sometimes forms multiple polyps.
Eventhough it is rarely seen in endometrial biopsy
specimens, uterine adenosarcoma is a common neoplasm.
The management comprising hysterectomy is curative in
most cases but additional bilateral salpingo-oophorect-
omy is indicated because of the risk of recurrence. The aim
of our experience is to show the difficulty of the diagnosis
with endometrial biopsies as well as to keep in mind the
possible lymphovascular invasion and the additional
pelvic lymphadenectomy.
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Case presentation
A 45-year-old Caucasian uniparous woman was referred to
our department of gynecology following a suspected
diagnosis of adenosarcoma that had been taken from a
hysteroscopic resection of an endometrial polyp. She had
presented with irregular uterine bleeding of approximately
10 months in duration without dysmenorrhea nor any
other symptoms. The gynaecologic examination revealed
that the uterus and ovaries were of normal size and that
the parametrial tissues were free. The ultrasonographic
evaluation showed a small non specific endometrial
hyperechogenic 1 cm mass. The pathologic diagnosis of
the endometrial tumor removed by surgical hysteroscopy
was a probable fibroid polyp. A diagnostic hysteroscopy
was performed after 3 months because of uncertain
diagnosis and showed a recurrent uterine polyp. Patholo-
gic examination of the endometrium was normal.
Magnetic resonance imaging of the pelvis showed a
small non-specific endometrial irregularity. The polyp
was removed by surgical hysteroscopy and pathologic
examination was benign despite few probable atypical
stromal cells. According to our surveillance protocol, the
performed diagnostic hysteroscopy was normal and the
endometrial biopsy showed no malignancy. A new
surgical hysteroscopy was performed 2 months later
because of irregular uterine bleeding and showed a
recurrent uterine polyp. The presence of atypical stromal
cells was confirmed by standard examination with
Hematoxylin & Eosin Safran of the removed uterine
polyp showing enlarged pleiomorphic nuclei. Finally, a
total abdominal hysterectomy was performed because of
atypical cells and suspected uterine adenosarcoma. The
hysterectomy specimen confirmed the presence of uterine
adenosarcoma associated to myometrial invasion to the
inner half of the myometrium as well as lymphatic and
multiple vascular tumor emboli. The histochemical stain-
ing for estrogen receptor was negative. Pathologic results
of second bilateral salpingo-oophorectomy and pelvic
lymphadenectomy were normal. The patient recovered
well with no evidence of recurrent disease 12 months after
surgery.
Discussion
Adenosarcoma is a rarely observed mullerian tumor of the
uterus and represents 8% of uterine sarcomatous tumors
[1]. It was first described in 1974 [2]. It has been mostly
reported in post-menopausal women but few cases were
observed in women between the ages of 19 and 40 years
[3-5]. Clinical symptoms vary from vaginal bleeding to
pelvic pain and to protrusion of the tumor from the cervix
or vagina [2,6]. The standard management is total
hysterectomy and bilateral salpingo-oophorectomy. More-
over, adjuvant chemotherapy and/or radiotherapy were
studied in advanced stages because of described cases of
metastatic and recurrent uterine adenosarcoma [7]. In
most cases, adenosarcoma presents as a yellow to grey
endometrial polyp [2]. Morphologically, it is composed of
papillary projections. The epithelial element lining the
papillary projections is benign, simple cuboidal or
columnar, mucinous, ciliated or squamous and the
stromal component is malignant [8]. Adenosarcoma may
present as recurrent endometrial polyp but the correct
diagnosis is only made after pathologic examination
identifying the benign epithelial component and the
malignant stromal one. Histologic findings were easily
confirmed in only 25% of cases [2]. In other cases, it was
difficult to differentiate from papillary adenofibromas,
mulleroblastomas and carcinosarcomas [2]. Difficulty of
diagnosis could be dependent on what areas have been
sampled on biopsy and on the fact that it is a rare observed
pathology. Increased cellularity of stroma around the
epithelial elements forming a cambium layer could be a
useful clue suggesting in diagnosis of adenosarcoma [8].
The diagnosis could be sometimes only made or
confirmed after multiple pathologic examinations of
recurrent polyps. In some cases, many endometrial
samples are needed in order to find uterine adenosarcoma
[8]. In fact, in our case, the diagnosis of uterine
adenosarcoma was difficult and only suspected after
multiple histologic analysis of recurrent uterine polyp.
Because of suspected lesion, hysterectomy was performed
and permitted to confirm histologically the diagnosis of
uterine adenosarcoma. In accordance with the routine
management of uterine adenosarcoma, bilateral salpingo-
oophorectomy was indicated to complete the surgical
treatment [2]. Cases of local recurrences were frequent in
the literature (50%) and related to bad prognosis most of
the times, eventhough the lowmalignancy component [9].
In our case, lymphovascular emboli were found in the
endometrial and myometrial space around the polyp.
Bilateral salpingo-oophorectomy and pelvic lymphade-
nectomy showed no additional lesion in our case. But,
concerning vascular and lymphatic space invasion,
Rovirosa et al found in a study that it had a significant
impact on overall survival and distant disease free survival
in early stages [10]. Our study confirms once again that
lymphovasular invasion could be associated to uterine
adenosarcoma and additional pelvic lymphadenectomy is
to be considered in order to complete the surgical
treatment.
Conclusion
Eventhough rare, uterine adenosarcoma is associated to a
great risk of local recurrences. Pathologic diagnosis could
be difficult and it should be highly suspected in case of
recurrent uterine polyps. The management strategy
requires confirmed diagnosis and surgery with hysterect-
omy and bilateral salpingo-oophorectomy in order to
avoid any treatment delay and recurrence risk. When
associated lymphovascular emboli are described, it seems
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Cases Journal 2009, 2:7515 http://casesjournal.com/casesjournal/article/view/7515
reasonable to consider pelvic lymphadenectomy since
lymphovascular invasion could have impact on overall
survival and distant metastasis-free survival.
Consent
Written informed consent was obtained from the patient
for publication of this case report and accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.
Competing interests
“The authors declare that they have no competing
interests”.
Authors’ contribution
RS analyzed, interpreted the patient’s data and wrote the
manuscript, PM got all the bibliography, AB and FD
performed most of the exams and helped writing the
manuscript, AC performed the histological exam, JLB
performed the surgery and contributed in the manuscript.
All authors read and approved the final manuscript.
References
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adenosarcoma of the uteru: case report and review of
literature. Eur J Gynaecol Oncol 2000, 21:387-390.
2. Clement PB, Scully RE: Mullerian adenosarcoma of the uterus: a
clinicopathologic analysis of 100 cases with a review of the
literature. Hum Pathol 1990, 21:363-381.
3. Fait T, Zivny J, Freitag P, Kuzel D: Adenosarcoma of the uterine
body in a 19-year-old woman - three year survival: case
report. Eur J Gynaecol Oncol 2001, 22:61-63.
4. Abu J, Ireland D, Brown L: Adenosarcoma of an endometrial
polyp in a 27-year-old nulligravida: a case report. J Reprod Med
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9. Taskin S, Bozaci EA, Sönmezer M, Ekinci C, Ortaç F: Late
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10. Rovirosa A, Ascaso C, Ordi J, Abellana R, Arenas M, Lejarcegui JA
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Uterine adenosarcoma associated to lymphovascular emboli: a case report

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