Case Series

Benign Vulvar Adnexal Tumours - A 5 Year Study in a Tertiary

Care Hospital
Kanwardeep Kaur Tiwana; M.D.1, Sarita Nibhoria; M.D.1, Harpreet Kaur; M.D.2,
Akanksha Bajaj; M.D.1, Richa Phutela; M.D.1

1 Department of Pathology, Guru Gobind Singh Medical College, Faridkot, Punjab, India
2 Department of Obstetrics and Gynaecology, Guru Gobind Singh Medical College, Faridkot, Punjab, India

Received April 2015; Revised and accepted November 2015

Abstract
Despite of the fact that the vulva contains a high density of apocrine and anogenital mammary glands in
addition to eccrine glands and folliculosebaceous units, the benign vulvar adnexal tumours are rare.
Though the varied clinical presentation and diverse histopathological spectrum of vulvar neoplasms has
amazed the pathologists, only few studies have been reported in literature. The present five year study
consists of only five cases of benign vulvar neoplasms depicting their rarity. Hidradenomapapilliferum
and syringoma were the most common entities followed by Chondroidsyringoma. The aim of our study is
to explore and highlight the histopathological diversity of benign vulvar adnexal tumours reflecting the
relative frequency of these structures.

Keywords: Vulvar Adnexal Tumours, Hidradenoma Papilliferum, Syringoma

Introduction1 Materials and methods

The vulva is composed of labia majora, labia minora, In the present five year study all the cases presenting
clitoris, vulvar vestibule, vestibulovaginal bulbs, with vulvar lesions were consecutively screened
urethral meatus, Bartholin's and Skene's glands. Also during the period of 2010 to 2015. The haematoxylin
vulvar region contains high density of apocrine and and eosin stained histopathological sections of vulvar
anogenital mammary like glands in addition to biopsies in the Pathology department were
eccrine glands and pilosebaceous units. However reevaluated. Physical and vulvar examination details
despite the high density of these units the tumours were obtained from the patient files and
derived from them are very low. The incidence of histopathology requisition form. The clinical
benign vulvar adnexal tumours has not been presentation and histopathological details were
highlighted much. The aim of the present study is to evaluated and diagnosis was confirmed. As the
highlight these benign vulvar adnexal tumours so that diagnosis was made purely on histopathological
it would reflect the relative frequency of these grounds, no ancillary aids were required
structures (1).
Results
In the present five year study a total of five cases of
Correspondence: benign vulvar adnexal tumours were identified. The
Kanwardeep Kaur Tiwana, H. No. 75 Medical College Campus patient age ranged between 22-60 years with mean
Faridkot, Punjab, 151203, India age of 42.8 years. Hidradenoma papilliferum (40%)
Email: [email protected]

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 Tiwana et al.

Table 1: Characteristics of reported cases

Age Clinical presentation Histopathological Diagnosis Percentage
42 years Indurated cyst Hidradenoma papilliferum 40%
32 years Sessile polyp Hidradenoma papilliferum
60 years Pruritus Syringoma 40%
40 years Pruritus Syringoma
22 years Painful tender lesion Chondroid syringoma 20%

and Syringoma (40%) were the most common structures (Figure 3).
tumours followed by Chondroid syringoma (20%) on
histopathological examination.
A wide range of clinical presentation was seen in
all the five cases and important point was that tumour
was not clinically suspected. Syringomas (40%)
presented with pruritus, Hidradenoma papilliferum
presented as indurated cyst and sessile polyp and
Chondroid syringoma presented as painful nodular
lesion (Table 1).

Hidradenoma papilliferum
It is derived from anogenital mammary glands.
Histopathologically the tumour has papillary and Figure 2: Syringoma (H&E X100)
glandular architecture. The epithelial cells are
columnar having pale eosinophilic cytoplasm and are
surrounded by myoepithelial layer (Figure 1).

Figure 3: ChondroidSyringoma exhibiting

cartilaginous differentiation (H&E X450)

Figure 1: HidradenomaPapilliferum (H&E X100) Discussion

Vulvar region contains dense apocrine glands,
Syringoma anogenital mammary like glands along with eccrine
It is a benign eccrine neoplasm. Histopathologically it glands and folliculosebaceous units. However the
consists of small epithelial cysts and dilated duct like frequency of adnexal tumours (benign as well as
spaces lined by two rows of cells, inner epithelial and malignant) is very low. Their incidence has not been
outer myoepithelial cells. The ductular structures reported much. This field despite being uncommon has
form comma like structures (Figure 2). not been explored. The aim of this study is to highlight
benign adnexal tumours of the vulva as the spectrum
ChondroidSyringoma (Mixed tumour of vulva) of various vulvar adnexal lesions appears to reflect the
It is a rare tumour of vulva arising from sweat glands frequency of the underlying glandular elements.
or Bartholin's glands. Histopathologically the tumour There have been very few studies done which
contains structures of epithelial and myoepithelial have addressed this issue. Only one study done by
cells associated with myxoid or cartilaginous Baker et al objectively evaluated the vulvar adnexal

200 Vol. 9, No. 4, November 2015 jfrh.tums.ac.ir Journal of Family and Reproductive Health
 Benign Vulvar Adnexal Tumours

lesions, they retrospectively retrieved a 32 year data Livingstone; 1995.

and reevaluated all the vulvar adnexal lesions 2. Baker GM, Selim MA, Hoang MP. Vulvar Adnexal
consisting of 189 cases. 2 In this study we evaluated Lesions: A 32-Year, Single- Institution Review From
only the benign adnexal vulvar tumours over the Massachusetts General Hospital. Arch Pathol Lab Med
period of 5 years and total of five cases were found. 2013; 137:1237-46.
The patient age ranged between 22-60 years with 3. Woodworth H, Docketty MB, Wilson RB, Pratt JH.
mean age being 42.8 years. Papillary Hidradenoma of the vulva: a
Hidradenomapapilliferum and syringoma were the clinicopathologic study of 69 cases. Am J
most common tumours which is in discordance with ObstetGynecol 1971; 110:501-8.
study done by Baker et al where 4. Parks A, Branch KD, Metcalf J, Underwood P, Young
Hidradenomapapilliferum (60%) was the most J. Hidradenomapapilliferum with mixed
common tumour followed by syringoma (22%) (2). histopathologic features of
Hidradenomapapilliferum was first reported by syringocystadenomapapillerum and anogenital
Worth in 1878 and the most common sites are vulva mammary –like glands: report of a case and review of
and perianal regions (3). This tumour is derived from the literature. AM J Dermatopathol 2012; 34 :104-9.
anogenital mammary like glands (4-7). In vulva the 5. Nishie W, Sawamura D, Mayuzumi M, Takahashi S,
sites of involvement include labia minora (50%), Shimizer H. Hidradenomapapilliferum with mixed
labia majora (40%), fourchette (7%) and clitoris (3%) histopathologic features of
(8). This distribution directly reflects the distribution syringocystadenomapapilliferum and anogenital
of anogenital mammary like glands. mammary-like glands. J CutanPathol 2004;31:561-4.
Syringoma is a benign eccrine gland tumour and it is
6. vanderPutte SC. Mammary like glands of the vulva and
very rare. But in the present study it was the most
their disorder. Int J GynecolPathol 1994; 13:150-60.
common tumour along with Hidradenomapapilliferum.
7. vanderPutte SC, van Gorp LH. Adenocarcinoma of the
It presented as pruritic lesion in all cases which is the
mammary- like glands of the vulva: a concept unifying
most common presentation in the study done by Huang
et al (9). In the present study all cases were incidental sweat gland carcinoma of the vulva, carcinoma of
findings. This is in concordance with study done by supernumerary mammary glands and extramammary
Baker et al where 41% of cases of syringoma were Paget’s disease. J CutanPathol 1994; 21:157-63.
incidental findings. These observations suggest that 8. Scurry J, vanderPutte SC, Pyman J, Chetty N, Szabo R.
prevalence of syringomas may be underestimated as Mammary like gland adenoma of the vulva: a review of
they were mostly asymptomatic. 46 cases. Pathology 2009; 41:372-8.
Chondroidsyringoma is a benign mixed tumour 9. Huang YH, Chuang YH, Kuo TT, Yang LC, Hong HS.
and has histological features of mixed tumour of Vulvar syringoma: a clinicopathologic and
salivary gland. It is a very rare tumour with only few immunohistochemical study of 18 patients and results
cases reported in literature (10-14). These tumours of treatment. J Am AcadDermatol 2003; 48 :735-9.
are considered benign but due to paucity of data it is 10. Ordonez NG, Manning JT, Luna MA. Mixed tumour of
difficult to determine the natural history at this site. the vulva: a report of two cases probably arising in
Bartholin’s gland. Cancer 1981; 48:181-6.
Conclusion 11. Dykgraaf RH, van Veen MM, van Bekkum-de Jonge
The spectrum of various vulvar tumours reflects the EE, Gerretsen J, de Jong D, Burger CW. Pleomorphic
relative frequency of the underlying glandular adenoma of the vulva: a review illustrated by a clinical
elements. This study is done to explore the field which case. Int J Gynecol Cancer 2006; 16:920-3.
is rare and has not been explored much so that natural 12. Rorat E, Wallach RC. Mixed tumors of the vulva:
progression of these tumours could be assessed. clinical outcome and pathology. Int J GynecolPathol
1984; 3:323-8.
Acknowledgments 13. Wilson D, Woodger BA. Pleomorphic adenoma of the
There is no conflict of interests to declare. vulva. J Obstet Gynaecol Br Commonw 1974; 81:1000-2.
14. Chome J, Giard R. Case report of an unusual tumor of
References the vulva: epithelioma of rearranged stroma or so-
1. Williams PL, Bannister LH, Berry MM, et al. Gray’s called mixed tumor [in French]. Bull Fed
Anatomy. 38thed. New York, NY: Churchill SocGynecolObstet Lang Fr 1956; 8:562-5.

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