2014, Volume 30, Number 3, Page(s) 210-214
Co-Existence of a Large Ovarian Hemangioma and Microscopic Dysgerminoma in a 10-Year Old Child
Mehmet KEFELİ1, Filiz KARAGÖZ1, Anais MALPICA2
1Department of Pathology, Ondokuz Mayıs University, Faculty of Medicine, SAMSUN, TURKEY
2MD Anderson Cancer Center, Section of Gynecologic Pathology, Houston, TEXAS, USA
Keywords: Ovary, Neoplasms, Hemangioma, Dysgerminoma, Childhood
Ovarian hemangioma is an uncommon ovarian tumor occurring
in patients in different age groups. It is usually seen as small,
asymptomatic mass, which is detected incidentally. On rare occasions,
this type of tumor can be associated with other gynecological
neoplasms. In this report, we present the clinicopathologic features of
a case of ovarian hemangioma coexisting with multiple microscopic
foci of dysgerminoma in a 10-year-old child. The patient presented
with acute abdomen. Magnetic resonance imaging revealed a left
ovarian mass and subsequent left salpingo-oophorectomy was
performed. Grossly, the mass was 9.5 cm in diameter and contained
hemorrhage and necrosis. Microscopic examination showed that
the tumor was mostly composed of hemangioma. Additionally,
there were three microscopic foci of dysgerminoma between the
hemangioma and ovarian cortex. Immunohistochemical studies
showed that dysgerminoma foci were positive for PLAP, Oct4 and
CD117. To our knowledge, this is the first case of the above-described
association ever reported.
Hemangiomas are benign vascular tumors that are found
uncommonly in the gynecological tract. In the ovary, this
type of tumor is usually detected incidentally. It is usually
seen in children or young adults but it can occur in patients
with a wide age range, from 4 months to 63 years. Although
there is some controversy as to whether these represent
hamartomas or true tumors, it is likely that both are
responsible for their formation1,2
Ovarian hemangiomas are usually under 1.5 cm in the
largest dimension, and found asymptomatically. However,
a few reported cases have presented with acute abdomen
due to the large size of the neoplasm with secondary torsion1,2. Seldom ovarian hemangioma may also be associated
with other gynecological neoplasms. We present herein the
clinicopathologic features of a case of ovarian hemangioma
coexisting with multiple microscopic foci of dysgerminoma
in a 10-year-old child who presented with abdominal
pain. We also discuss the differential diagnosis of ovarian
hemangioma with respect to their association with other
gynecological tract neoplasms.
A 10-year-old girl was admitted to the hospital after one
month history of abdominal pain. Blood tests and hormonal
levels were within normal limits except placental alkaline
phosphatase (596 U/L), which was slightly elevated (Normal value <300 U/L). Magnetic resonance imaging (MRI) of the
abdomen and pelvis showed a 9.8 x 9.6 x 7.4 cm left ovarian,
solid mass with central necrosis and hemorrhage. These
features prompted the imaging impression of a malignant
mesenchymal tumor. A left salpingo-oophorectomy was
Gross examination showed that the left ovarian mass
measured 9.5 x 6 x 5.5 cm. It was circumscribed and had
an intact capsule (Figure 1). The tumor was tan-brown and
contained small cysts, especially beneath the capsule, and
it showed extensive necrosis and hemorrhage towards its
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|Figure 1: Gross appearance of the ovarian tumor, tan-brown in
color and with extensive necrosis and hemorrhage in the center.
Microscopically, most of the tumor showed the histological
features typically seen in a hemangioma (i.e., numerous
thin-walled, large, vascular spaces lined by a single layer
of bland endothelial cells and filled with red blood cells)
(Figure 2). There were areas of hemorrhage and necrosis,
especially in the center of the mass and the ovarian cortex
was mostly obliterated. In addition, there were three foci
of polyhedral cells with clear cytoplasm and prominent
nuclei between the hemangioma and the thinned ovarian
cortex (Figure 3). These cells were arranged in nests
and cords separated by fibrovascular septae containing
lymphocytes (Figure 4A,B) and they showed 1 to 2 mitoses
per high power field. These foci measured at 0.2, 0.3, 0.8
cm, respectively. Immunohistochemically, these cells
were cytoplasmic positive for vimentin, placental alkaline
phosphatase, CD-117 (Figure 5) and nuclear positive with
Oct4 (Figure 6), but negative for cytokeratin, calretinin,
inhibin, chromogranin A, synaptophysin, α-fetoprotein,
S-100, CD 30 and desmin.
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|Figure 2: An ovarian hemangioma is characterized by numerous
large calibers, thin-walled vascular spaces lined by a single layer of
endothelial cells (H&E, x100).
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|Figure 3: Hemangioma and dysgerminoma foci separated by
fibrous capsule (Left side hemangioma, right side dysgerminoma)
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|Figure 4A, B: Foci of dysgerminoma detected between the hemangioma and the ovarian cortex (H&E, x100 and x200).
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|Figure 5: Dysgerminoma foci are positive for CD 117
immunostaining (DAB, x200).
The final histological diagnoses were cavernous hemangioma
and minute foci of dysgerminoma. No additional therapy was given and after a follow-up of 24 months the patient is
alive with no evidence of disease.
Hemangiomas are benign vascular tumors that are rarely
seen in the ovary. Less than 50 cases have been reported so
far. They are usually small, under 1.5 cm in largest dimension
and usually detected incidentally. However, a few reported
cases have been associated with symptoms such as acute
lower abdominal pain, massive ascites, elevated CA-125
and pleural effusion (pseudo-Meigs’ syndrome)2-5
The ovary has very rich and complex vasculature in the
medulla. Therefore it’s not surprising that hemangiomas are generally seen in the hilus or medulla and may be
confused with normal vasculature. Various vascular
tumors and their mimics have been reported in the
ovary. Therefore, the differential diagnosis of ovarian
hemangioma encompasses normal medullary vasculature,
lymphangioma, hemangioendothelioma, angiosarcoma
and the prominent vascular component of a teratoma.
Since the ovarian medulla is rich of vascularized fibroelastic
connective tissue, which may sometimes proliferate, the
distinction between a small hemangioma and vascularized
ovarian medulla can be challenging. To complicate this
quandary, the normal blood vessels of the medulla can
become numerous and closely packed in postmenopausal
individuals. However, these vascular alterations are often
small and more diffuse than hemangiomas and do not result in a distinct mass formation. Moreover, lymphatic vessels
and nerve fibers are usually seen admixed with proliferated
vessels in a vascularized medulla. Additionally, the ovarian
medulla may have a few clusters of hilus cells which
ovarian counterpart of testicular Leydig cells. They may
seen between or around the proliferated vessels in medulla
but not in hemangiomas. It is of note that these cells are
only present during the fetal life and are not identifiable in
childhood. However, they do reappear again at puberty and
are demonstrable in all postmenopausal term2,6.
Ovarian lymphangiomas are also very rare and less than 20
cases have been reported so far1,7,8. Microscopically,
they are characterized by the presence of thin-walled
vascular structures, which are lined by flattened endothelial cells and contain pale homogeneous eosinophilic fluid
materials. Endothelial cells are positive for CD31 and D2-
40 by immunohistochemically in lymphangiomas1,2.
Primary ovarian hemangioendotheliomas have been
reported in three cases with different subtypes (kaposiform,
infantile and epithelioid subtypes)9-11. Two cases
(kaposiform and infantile subtypes) have been reported in
neonates, and the other case (epithelioid subtypes) has been
reported in a 20 year-old patient. Microscopically tumors
contained tightly packed, frequently dilated capillary blood
vessels, which were lined with spindle cells, or plump,
epithelioid cells depend to tumor subtypes. Those tumors
had some cellular area, mild atypia and some mitosis but
none of the mitosis atypical forms. Immunohistochemically,
tumor cells were positive with vascular markers. In the
presented case, vascular structures were thin-walled and
large sized, and lined by a single layer of bland endothelial
cells. However, no cellular areas, mitosis or other worrisome
features are noted throughout the vascular component of
Primary ovarian angiosarcomas are also rare neoplasms,
less than 40 cases reported to date, and some of those have
been associated with mature cystic teratoma12. They are
usually large and contain blue-brown, hemorrhagic and
necrotic regions, which may be associated with an invasive
growth into the adjacent structures. Microscopically, they
display a variable size of vascular spaces lined by atypical
endothelial cells that show increased mitotic activity
including atypical mitotic figures as well as pleomorphism
and papillary tufting. Hemangiomas do not have these
nuclear features. Therefore their absence is important for
the distinction of these entities1,2.
While benign and malignant vascular neoplasms can arise
in the background of teratomas, florid reactive vascular
proliferations mimicking hemangiomas were also reported
in teratomas. These regions were usually accompanied with
a neural tissue component. In those cases, the vascular
structures can be variable; small to large vascular structures
lined by oval to spindle shaped endothelial cells that exhibit
mild atypia can be encountered in this context. Therefore,
before making a diagnosis of hemangioma, adequate
sampling is critical to distinguish it from a teratoma13-15. We examined 35 samples from mass to make
differential diagnosis of hemangioma and to determine for
size of disgerminoma.
Ovarian hemangiomas rarely co-exist with other genital
tract malignancies such as endometrial and ovarian
carcinoma16-18. We found only two cases of hemangioma associated with other ovarian tumors. The first case was
that of a 32-year-old patient with ovarian hemangioma
and mucinous cystadenoma17. The second case was that
of a 65-year-old with an ovarian serous carcinoma and
an incidental small capillary hemangioma detected in the
To our knowledge, this is the first reported case of a large
ovarian hemangioma associated with foci of dysgerminoma.
Although a large hemangioma is very unusual, it should be
considered in the differential diagnosis of an ovarian mass
and it may be coexisting with other ovarian tumors.
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