CASE REPORT

Cotyledonoid dissecting leiomyoma of the uterus

with intravascular growth: report of two cases
Ksenya V. Shelekhova & Dmitry V. Kazakov &
Michal Michal
Received: 19 April 2006 / Accepted: 13 October 2006 / Published online: 23 November 2006
# Springer-Verlag 2006
Abstract We present two cases of cotyledonoid dissecting
leiomyoma of the uterus with intravascular involvement,
which occurred in women aged 73 and 48 years. Grossly
and microscopically, both neoplasms had an extrauterine
cotyledonoid part and intrauterine dissecting fascicles of
disorganized, swirled neoplastic smooth muscle with
hydropic degeneration and foci of an intravascular growth
(the latter was identified histologically). To our knowledge,
the intravascular component of such a neoplasm is a very
rare feature that has previously been described only in three
cases in the literature.
Keywords Leiomyoma of the uterus
.
Cotyledonoid
.
Intravascular
.
Dissecting
.
Intravenous leiomyomatosis
Introduction
Cotyledonoid dissecting leiomyoma (Sternberg tumor)
is an unusual type of a benign uterine smooth muscle
tumor that combines two main features: an exophytic
component of smooth muscle grossly resembling placental
tissue and an intrauterine component which irregularly
extends into the myometrium between the fascicles of
normal smooth muscle cells [1, 35, 7, 9]. This is a very
rare variant of smooth muscle tumors with a distinctive
gross appearance. Approximately 20 cases have been
reported in the literature [35, 7, 9], and only three cases
showed the association of a tumorous tissue with vessels
[3]. We describe herein two additional cases of cotyle-
donoid dissecting leiomyoma of the uterus with intravas-
cular involvement, a very uncommon and infrequent feature
to occur in this type of leiomyoma.
Materials and methods
Tissue samples were fixed in 4% formaldehyde and were
paraffin-embedded. Sections (5 m thick) were stained with
hematoxylin and eosin.
A panel of immunohistochemical stains included anti-
bodies against CD34 (QBEnd/10, 1:800, Neo Markers),
CD31 (JC70A, 1:50, DakoCytomation), factor VIII-related
antigen (F8/86, DakoCytomation), -smooth muscle actin
(1A4; 1:1,000, DakoCytomation), muscle-specific actin
(HHF35; 1:3,000, DakoCytomation), and desmin (D33,
1:3,000, DakoCytomation).
For electron microscopic investigation, wet glutaralde-
hyde-fixed tissue was available in one case. It was con-
trasted in 1% osmium tetroxide and embedded into epoxy
resin (Durcupan-Epon). Sections were cut 1 m thick,
stained with toluidine blue, and examined by light micros-
copy. Appropriate areas were selected, and thin sections
were cut and stained with uranyl acetate and lead citrate
and were examined with a Philips (Eindhoven, Holland)
EM 208S electron microscope.
Virchows Arch (2007) 450:119121
DOI 10.1007/s00428-006-0329-8
K. V. Shelekhova
Department of Pathology, Petrovs Institute of Oncology,
Saint-Petersburg, Russia
D. V. Kazakov
:
M. Michal (*)
Sikls Department of Pathology, Charles University,
Medical Faculty Hospital,
Alej Svobody 80,
30460 Pilsen, Czech Republic
e-mail: michal@medima.cz
Case reports
Case 1 A 73-year-old woman underwent a laparotomy for a
uterine mass. Her detailed gynecological history and
follow-up were unavailable. Grossly, the tumor measured
8 cm and involved the uterus in the region of the fundus.
There were numerous exophytic, rubbery reddish nodules
that measured approximately 1.5 cm in diameter, resembled
placental tissue, and extended into the broad ligament
and projected into the pelvic cavity. Numerous ill-defined
yellowish-white subserosal, intramyometrial, and submuco-
sal nodules with a marked hydropic change represented the
intrauterine component.
Case 2 A 48-year-old woman, G3, P3 presented with a
palpable abdominal mass, for which she underwent a total
abdominal hysterectomy and salpingo-oophorectomy. Two
years before presentation, the patient was diagnosed with
myomatosis of the uterus, and 2 months before surgery, the
patient started to experience pain, and the abdominal mass
became palpable. She never used hormonal anticonceptives,
but in the past, she had had an IUD for 21 years removed
2 years before the diagnosis of myomatosis. The patient
was alive and well 2 years after surgery. Macroscopically,
the enlarged uterus, measuring 13105 cm, revealed a
bulbous tumorous mass measuring 945 cm, involving
the fundus and the cornua, with extension into the broad
ligament (Fig. 1). The cut surface of the uterus showed
ill-defined intramural nodules of a tumor with variegated
coloration. The adnexa were normal both grossly and
microscopically.
No intravascular growth was identified on gross exam-
ination in either case.
Microscopic and ultrastructural findings
In both cases, there were variably sized micronodules of
muscle fascicles, which were composed of disorganized
Fig. 1 Case 2. Gross appearance of the uterus with the exophytic
multinodular part of the tumor resembling placental tissue and
extending into the broad ligament. The tumorous nodules were
originally red in color and appear brown here because of long fixation
in formalin
Fig. 2 a Case 1. Intravascular
extension of leiomyoma. b Case
2. Neoplastic leiomyomatous
growth into the lumen of the
small vein. c Case 2. CD31
staining: a tumorous mass in the
lumen of the vein. d Case 2.
Processes of the tumor dissect
the myometrium. Note a nodule
of swirled smooth muscle sur-
rounded by a hydropic fibrous
connective tissue
120 Virchows Arch (2007) 450:119121
hypertrophied smooth muscle cells and had a swirled
appearance. The tumorous nodules were separated by a
connective tissue with marked hydropic change and rich
vascularity. Immunohistochemically, the tumor cells ex-
hibited strong staining for desmin and smooth muscle actin.
Within the leiomyomas, we discovered focal intra-
vascular intrusion of the tumor masses. Foci of neoplastic
leiomyomatous tissue sometimes entirely filled the lumens
of the preexisting veins (Fig. 2a,b). CD31 and factor
VIII-related antigen highlighted the endothelial cells of
the blood vessels surrounding the foci of the intravascular
growth of the leiomyoma (Fig. 2c). In other foci, dissecting
tumor areas within the fascicles of normal-appearing
myometrium were observed (Fig. 2d). There were neither
nuclear atypia nor mitotic figures. No coagulative tumor-
cell necrosis was observed. In both cases, mitotic figures
were absent or rare (01 per 10 high-power fields).
Case 2 was studied ultrastructurally [2]. The tumor was
composed of cells that resembled normal smooth muscle
cells and showed the characteristic folded nucleus. The
cytoplasm contained intermediate filaments and microfila-
ments with focal densities. There were also abundant
collagen fibrils in the matrix.
Discussion
The classification of most benign smooth muscle tumors of
the uterus is based on the combination of gross and
microscopic features such as growth pattern, histologic
appearance, association with vessels, and others [1, 37].
Therefore, numerous variants of uterine smooth muscle
tumors may be manifestations of a common pathological
process.
Roth et al. [7], in 1996, noticed the associations and
similarities between intravenous leiomyomatosis, infil-
trating leiomyoma, multinodular hydropic leiomyoma, and
cotyledonoid dissecting leiomyoma, and in view of these
relationships, the authors believed that a cotyledonoid
variant of intravenous leiomyomatosis can be anticipated.
Indeed, Jordan et al. [3], in 2002, described three cases that
demonstrated the features of intravenous leiomyomatosis.
The authors regarded this type of leiomyoma as a novel
entity and proposed the term cotyledonoid hydropic
intravenous leiomyomatosis. However, only in one of the
three cases reported by Jordan et al. was there an intimate
association of the tumorous cells with vessels as detected
macroscopically. Earlier, Norris and Pamley suggested
that the term leiomyomatosis should be applied only to
tumors in which intravascular extension could be detected
on gross inspection, while lesions with intravascular
growth seen only microscopically should be designated as
leiomyoma.
An extrauterine cotyledonoid part and intrauterine
dissecting fascicles of disorganized, swirled neoplastic
smooth muscle with hydropic degeneration and foci of
intravascular growth of the tumor characterize both neo-
plasms described in this report. These features are similar to
those detected in previously described cases of Sternberg
tumor [4, 5, 79]. In our cases, we did not find the
intravascular growth macroscopically; hence, following
the terminology suggested by Norris and Pamley, we
designated both neoplasms reported herein as cotyledonoid
dissecting leiomyoma of the uterus with intravascular
growth and not as cotyledonoid hydropic intravenous
leiomyomatosis.
Foci of an intravascular growth may cause confusion
and might imply a malignancy. The tumors however lack
nuclear atypia, mitotic activity, or coagulative tumor ne-
crosis, and, besides, well-differentiated smooth muscle cells
and the abundance of collagen fibrils in the matrix infer
the benign nature of the tumors. However, the small
number of reported cases allows no conclusion on the
biological course of the lesions. One of our patients was
alive and well 2 years after surgery. In the series of Jordan,
the follow-up (no evidence of disease at 5 years) was
available only for one of the three patients. Further studies
are needed to establish the exact biological nature of these
tumors.
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Virchows Arch (2007) 450:119121 121