Mahapatra et al.

, J Womens Health, Issues Care 2013, 2:5

http://dx.doi.org/10.4172/2325-9795.1000120
Journal of Women’s
Health, Issues & Care

Case Report a SciTechnol journal

Cut-section was grayish white with solid to mucoid areas (Figure 1).
Glomus Tumor in Vulva with Microscopic examination revealed tumor cells arranged in sheets in

Uncertain Malignant Potential
Smita Mahapatra1*, Asaranti Kar2, Tushar Kar3 and Upasana
Das2
Abstract
Glomus tumors of female genital tract are rare. Herein we
report a case of glomus tumor in the vulva in a 39 years female
presenting with a 4 cm painful vulvar growth. The mass was
totally excised. The histopathological diagnosis was supported
with immunohistochemistry and considering the size of the tumor
more than 2 cm, the final diagnosis of glomus tumor with uncertain
malignant potential was made. After the excision, the patient had
no recurrence, on follow up. To the best of our knowledge only very
few cases of glomus tumor with uncertain malignant potential have
been described in vulva till date.
Keywords
Glomus tumor; Vulva
a hyalinized mixed stroma (Figure 2). The monotonous round cells
had indistinct cell borders, pale eosinophilic cytoplasm, round central
nuclei (Figure 3). The cells were arranged around the blood vessels at
the periphery of the tumor (Figure 4). There was no cellular atypia
or mitosis. It was histopathologically diagnosed as glomus tumor in
vulva. Again for further confirmation, immunohistochemistry (IHC)
was performed. The tumor showed positivity for smooth muscle actin
(+++) (Figure 5) but it was negative for cytokeratin. Thus, diagnosis
was confirmed as glomus tumor in vulva. Considering the size of
the tumor more than 2 cm, the final diagnosis of glomus tumor with
uncertain malignant potential was given. On follow up for duration
of 12 months, the patient had responded to the treatment, there was
no further recurrence of the tumor and she was symptom free.

Introduction
Glomus tumor is an unusual, benign soft tissue tumor arising
from neuromuscular cells of the glomus apparatus which function
Figure 1: Gross measuring 3×2×1 cm, cut section showing grayish solid to
as temperature regulation via arteriovenous shunting of blood. It is
mucoid areas.
found in deep dermis of the extremities [1]. They can occur anywhere
in the body and are occasionally found in gastrointestinal, respiratory
and female genital tracts. In the female reproductive tract, glomus
tumor has been described in the external genitalia [2,3], vagina [4,5],
uterine cervix [6] and myometrium [7]. We are presenting this a
case as of glomus tumor in vulva with uncertain malignant potential
which is extremely rare.
Case Report
A 39 year female presented with a painful swelling in vulva for
the last six months. The swelling was 4 cms in diameter, firm, with
overlying skin being normal. The swelling was clinically diagnosed
as bartholin’s cyst. In pervaginal (PV) examination, there was no Figure 2: Tumor cells are arranged in sheets surrounding blood vessels in a
hyalinized mixed stroma (H&E, X400).
abnormal discharge/bleeding, no history of amenorrhea, cervix
was parous and fornices were free. Introitus was normal and no
abnormality was detected in vagina. Her hematological parameters
were within normal limit. The mass was completely excised and was
submitted for histopathological examination.
Grossly, it was smooth, well circumscribed and of 3×2×1 cm3 size.

*Corresponding author: Dr. Smita Mahapatra, Department of Transfusion

Medicine, S.C.B. Medical College, N-1/256, IRC Village, Nayapalli,
Cuttack-753007, Orissa, India, Tel: 91-9437094138; E-mail: doctorsmita@
rediffsmail.com
Figure 3: Monotonous tumor cells with central nuclei and pale eosinophilic
Received: February 28, 2013 Accepted: September 06, 2013 Published: cytoplasm (H&E, X400).
September 11, 2013

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Citation: Mahapatra S, Kar A, Das U, Kar T (2013) Glomus Tumor in Vulva with Uncertain Malignant Potential. J Womens Health, Issues Care 2:5.
Figure 4: Tumor cells arranged around blood vessels have centrally located
nuclei with pale cytoplasm (H&E X 400).
Figure 5: Immunostaining positive for smooth muscle actin (IHC).
Discussion
Glomus tumor was initially described by Masson in 1924 typically
involving the skin of the hand and especially the subungual region of
the finger [8]. This tumor is a small benign neoplasm derived from
the specialized cell of the glomus body, intimately associated with and
surrounding arteriovenous anastomosis. The most important site is
the subungual region, deep dermis and subcutis of upper and lower
extremities, where arteriovenous anastomoses are numerous. Glomus
tumor arising from female genital tract is rare. In vulva less than 10
cases have been reported in English literatures mostly as isolated case
reports [9,10]. These tumors arise in females in 27-53 years age group
with size being 1.0 cm or smaller. The patient with vaginal tumor of
5 cm size is present with vaginal bleeding [4]. The vulvar and clitoral
lesions are painful, but other glomus tumors are incidentally detected
during hysterectomy.
Glomus tumor is typically composed of 3 components: glomus
cells, vasculature and smooth muscle cells. Again, they can be
subcategorized as solid glomus tumor with poor vasculature and
scanty smooth muscle component, glomangioma with prominent
vascular component and glomangiomyoma with prominent vascular
and smooth muscle component. Out of this solid tumor is the
commonest (75%) followed by glomangioma and glomangiomyoma
(5%) [11].
The causes of subcutaneous nodule like nodular hidradenoma,
hemangiopericytoma, intradermal nevus and melanoma can be a
differential diagnosis of glomus tumor. Both glomus tumor and
nodular hidradenoma may show uniform aggregates of epitheliod
cells in a myxoid stroma with variable number of blood vessels. But, in
glomus tumor the tumor cells are arranged around the blood vessels
at the periphery of the tumor as seen in our case lacking ductular
differentiation and epithelial mucin production [11]. Hidradenoma
immunohistochemically stain for cytokeratin, carcinoembryonic
antigen and epithelial membrane antigen, while glomus tumor is
Volume 2 • Issue 5 • 1000120
doi:http://dx.doi.org/10.4172/2325-9795.1000120
negative for these. In our case, the tumor was negative for cytokeratin.
In haemangiopericytoma, there is presence of mixture of spindled and
epithelioid cells, staghorn vessels and indistinct cell borders which
are negative for muscle specific marker actin which was positive in
our case. The possibility of intradermal nevus and melanoma were
excluded due to absence of proliferation of melanocytic cells.
Rarely, when glomus tumors exihibit large pleomorphic nuclei
without increased cellularity and mitotic activity, they are referred
as symplastic glomus tumors. The atypical changes are due to
degenerative changes. Glomangiomatosis which is a rare and benign
morphologic variant of glomus tumors show infiltrative growth of
vessels whose walls are invested by glomus cells [12].
Majority of glomus tumors are benign which are cured by
simple local excision. It is diagnosed as malignant when large in
size (>2.0 cm), deep location or moderate to high nuclear grade and
increased mitotic figures (>5/50 high power fields) or the presence
of atypical mitotic figures. In malignancy, the metastasis exceeds
25% [12]. Glomus tumors with uncertain malignant potential are
diagnosed when they exhibit any one of the following features; high
mitotic activity with superficial location, large size, deep location.
Thus, considering the size of the tumor more than 2 cm, our case
was diagnosed as glomus tumor with uncertain malignant potential.
Glomus tumor is typically immunopositive for actin, occasionally
for desmin [13], thus actin and myosin immunohistochemical stains
highlight benign glomus tumor than malignant counterpart, whereas
opposite is true for vimentin [14].
Simple surgical excision is curative as seen in our case which was
followed for 12 months and was found disease free. The previous
reported case of vulvar glomus tumor of 3 cm diameter had a disease
free survival for more than 10 years [15]. Though rare while dealing
with painful vulvar growth, glomus tumor has to be kept in mind
for the better management of the patient Local recurrence may occur
after incomplete excision.
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Citation: Mahapatra S, Kar A, Das U, Kar T (2013) Glomus Tumor in Vulva with Uncertain Malignant Potential. J Womens Health, Issues Care 2:5.

doi:http://dx.doi.org/10.4172/2325-9795.1000120

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Author Affiliations Top

1
Department of Transfusion Medicine, SCB Medical College, Cuttack, Orissa,
India
2
Department of Pathology, SCB Medical College, Cuttack, Orissa, India
3
Department of Obstetrics and Gynaecology, SCB Medical College, Cuttack,
Orissa, India

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