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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 3  |  Issue : 2  |  Page : 38-40

Hidradenoma papilliferum of the vulva with extensive squamous metaplasia mimicking malignancy: Rare entity with diagnostic challenge


Department of Pathology, Gujarat Cancer and Research Institute, Ahmedabad, Gujarat, India

Date of Web Publication18-Sep-2019

Correspondence Address:
Dr. Ritwika Dabral
Department of Pathology, Gujarat Cancer and Research Institute, Civil Hospital Campus, Room Number 401, Asarwa, Ahmedabad, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/oji.oji_23_19

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  Abstract 


Hidradenoma papilliferum (HP) is an uncommon benign cutaneous adnexal neoplasm of the anogenital region commonly seen in middle-aged, Caucasian females. Anastomosing and branching tubules, lined by columnar cells, and a basal layer of myoepithelial cells are the characteristic histopathological features. We report a case of HP in a 60-year-old female presented with a vulvar lesion. The case was evaluated for malignancy, with the initial histopathology showing extensive squamous metaplasia but ultimately found to have HP.

Keywords: Hidradenoma papilliferum, squamous metaplasia, vulva


How to cite this article:
Jetly D, Dabral R, Goel D. Hidradenoma papilliferum of the vulva with extensive squamous metaplasia mimicking malignancy: Rare entity with diagnostic challenge. Oncol J India 2019;3:38-40

How to cite this URL:
Jetly D, Dabral R, Goel D. Hidradenoma papilliferum of the vulva with extensive squamous metaplasia mimicking malignancy: Rare entity with diagnostic challenge. Oncol J India [serial online] 2019 [cited 2019 Oct 23];3:38-40. Available from: http://www.ojionline.org/text.asp?2019/3/2/38/266980




  Introduction Top


Papillary hidradenoma also called as hidradenoma papilliferum (HP), as the name suggests, is a rare slow-growing, benign tumor of the modified apocrine glands, and most commonly occurs in the anogenital region of middle-aged females between the ages of 30 and 49 years.[1] Rarely, it affects other regions of the body as well, for example, the head and neck, breast, external ear canal, and eyelid, which is then called as ectopic HP[2] It commonly presents as an asymptomatic small-sized flesh to red-colored cystic nodule.

We present case a unique case of HP vulva which was misdiagnosed and treated as squamous cell carcinoma of vulva. This case is being reported because HP can coexist with SCC and itself has malignant potential.


  Case Report Top


A 60-year-old female attended the outpatient department of our hospital with complaints of ulcerated exophytic growth for 2 years and discharge per vaginum for 3 months. She was a bidi smoker for the past 5 years and was recently diagnosed with hypothyroidism for which she was under treatment. Her general and systemic examinations were unremarkable.

On local examination, she had an exophytic growth of size 5 cm × 4 cm × 3 cm, soft to firm in consistency, involving left labia majora and part of the left side of the mons pubis for the past 2 years, which was gradually increasing in size over the time and recently got ulcerated. It was associated with enlarged palpable, mobile, firm, and nontender inguinal lymph nodes, with the largest being 2 cm × 2 cm. Per speculum, bimaual, and per-rectal examinations were normal. A punch biopsy from an outside histopathology laboratory reported it to be moderately differentiated SCC.

Ultrasound of the abdomen and pelvis confirmed the presence of bilaterally enlarged inguinal lymph nodes.  Pap smear More Details examination was negative for intraepithelial lesion or malignancy and showed inflammation and atrophy. The histopathology slides were reviewed in our institute which also suggested moderately differentiated SCC.

A diagnosis of SCC was made, and the patient was planned for surgery. She underwent radical vulvectomy and inguinofemoral lymph node dissection. She was started on antibiotics, analgesics, and thromboprophylaxis, and her postoperative period was uneventful.

Histopathology of the specimen revealed an exophytic ulceroproliferative growth of size 5 cm × 4 cm × 3 cm involving the left labia majora and minora [Figure 1]. On microscopic examination, the final diagnosis of benign epithelial tumor with extensive squamous metaplasia was made, which was limited to the dermis and subcutaneous tissue [Figure 2]a and [Figure 2]b. The left inguinofemoral lymphadenectomy specimen revealed 3 cm × 2 cm × 2 cm large matted lymph nodes. Few lymph nodes were identified in the right inguinofemoral lymphadenectomy specimen. On microscopic examination, all the lymph nodes showed reactive hyperplasia. The extensive squamous metaplasia showing features of high nucleus cytoplasmic ratio, hyperchromasia, and slight overlapping led to the confusion of being malignant. Hence, immunohistochemical examination was applied to confirm the diagnosis which showed positivity to CK5/6, carcinoembryonic antigen, AE1, epithelial membrane antigen, and gross cystic disease fluid protein (GCDFP)-15 [Figure 3] and [Figure 4]. Thus, the diagnosis of HP was confirmed.
Figure 1: Specimen of radical vulvectomy showing exophytic growth involving the left labia majora and minora

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Figure 2: H and E stained section (×100 magnification) showing (a) Extensive squamous metaplasia and ducts with no invasion and (b) ducts lined by apocrine cells and fibrous stroma

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Figure 3: Immunostain positive for CK5/6, carcinoembryonic antigen, and AE1 (×100 magnification)

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Figure 4: Immunostain positive for Epithelial membrane antigen and gross cystic disease fluid protein 15 marker (×100 magnification)

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The patient was discharged, and she is asymptomatic in the follow-up period of 6 months.


  Discussion Top


HP is an uncommon, benign, cystic, and papillary tumor of the anogenital region, most commonly occurring in Caucasian females of the middle age group.[1],[3] In our case, the patient was 60 years old. In various studies, it has been found that 3%–17% of women with HP are >60 years of age.[4]

The most common site of involvement in the body is the anogenital region, especially the large lips of the vulva, which is four times more common than perianal occurrence. Also apart from the vulva, less frequently, the tumor may also be seen in the interlabial sulcus, clitoris, posterior fourchette, pubis, perineum, anal region, and in extragenital sites. In places other than the anogenital region, it is called as ectopic HP which is mainly reported in the head-and-neck region, followed by others such as the breast, eyelid, external ear, scalp, orbit, nose, breast, chest, and abdomen.[1],[5]

Clinically, HP presents as a single, asymptomatic, well-circumscribed, skin-colored, or reddish-brown nodule, with the size of the lesion varying from 0.5 to 1 cm. Rarely, symptoms of pain, burning sensation, discharge, bleeding, or pruritis develop mostly in long-standing tumor.[5]

Although the histological origin of papillary hidradenoma was previously thought to be an apocrine sweat gland, newer studies have hypothesized that it may have originated from the anogenital mammary-like glands.[6] Histologically, papillary hidradenoma is a dermal tumor. It represents an adenoma with apocrine differentiation. Stromal compression gives an appearance of well-circumscribed pseudocapsule. Within the tumor mass, tubular and cystic structures can be seen with cystic spaces intervened with papillary folds.[7] Sometimes, the cystic spaces are lined by a single layer of columnar cells which have eosinophilic cytoplasm and oval, hypochromatic nuclei. Dual layer of cells, namely inner secretory and outer cuboidal cell layer with myoepithelial cells, surrounds the luminal space. Associated adenomatous hyperplasia with cellular pleomorphism and an irregular papillary pattern of the acinus without lumen formation indicate its malignant potential. Squamous metaplasia was noted in 1% of various studies.[8]

Benign lesions to be considered in its differential diagnosis are hemorrhoidal disease, anorectal abscess, viral verrucous lesion, sebaceous cyst, mucous cysts, epidermal inclusion cysts, lipoma, and neurofibroma, and few malignant lesions such as metastatic papillary carcinoma, syringocystoadenocarcinoma papilliferum, and SCC should also be ruled out before its diagnosis.[3]

Local excision with a margin will be sufficient for its diagnosis, treatment, and cure.[9] In the present case, a large ulceroproliferative exophytic mass, old age of presentation, clinical and radiological suggestion of bilaterally enlarged inguinofemoral nodes went in favor of malignant lesion of the vulva. Furthermore, histologically, extensive squamous metaplasia was seen, which may be because of irritation and repetitive infection caused by a long-standing lesion which led to a diagnosis of SCC. Complex papillary glandular pattern, with stratification and some degree of pleomorphism and mitotic activity, stimulate carcinoma.

Immunohistochemistry will help to differentiate between HP and SCC. SCC shows immunoreactivity for high-molecular-weight keratin, while HP does not. GCDFP-15 positivity supported the diagnosis of HP as it comes positive in tumors of apocrine gland and lacrimal gland origin.[10]

Hence, HP with squamous metaplasia and HP with associated SCC should be differentiated with utmost care. This is the first case in the literature where HP was confused with SCC of vulva due to extensive squamous metaplasia.


  Conclusion Top


The diagnosis of HP should be kept in mind in any anogenital lesion. Also as we have seen it can occur with extensive squamous metaplasia, or with concomitant malignant carcinoma and even it itself has the propensity to convert into malignant carcinoma, every care must be taken to prevent both over and underdiagnosis. Role of expert histopathological review and immunohistochemistry is a must.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Kambil SM, Bhat RM, D'Souza DC. Hidradenoma papilliferum of the vulva. Indian Dermatol Online J 2014;5:523-4.  Back to cited text no. 1
  [Full text]  
2.
Vang R, Cohen PR. Ectopic hidradenoma papilliferum: A case report and review of the literature. J Am Acad Dermatol 1999;41:115-8.  Back to cited text no. 2
    
3.
Docimo S Jr., Shon W, Elkowitz DE. Bartholin's abscess arising within hidradenoma papilliferum of the vulva: A case report. Cases J 2008;1:282.  Back to cited text no. 3
    
4.
Woodworth H Jr., Dockerty MB, Wilson RB, Pratt JH. Papillary hidradenoma of the vulva: A clinicopathologic study of 69 cases. Am J Obstet Gynecol 1971;110:501-8.  Back to cited text no. 4
    
5.
5. Kondo RN, Melhado IP, Moreira CR, Crespigio J. Ectopic hidradenoma papilliferum. An Bras Dermatol 2018;93:474-5.  Back to cited text no. 5
    
6.
Al-Faky YH, Al-Mosallam AR, Al-Sohaibani MO. Periocular hidradenoma papilliferum. Saudi J Ophthalmol 2009;23:211-3.  Back to cited text no. 6
    
7.
Klein W, Chan E, Seykora JT. Tumours of the epidermal appendages. In: Elder DE, editor. Lever's Histopathology of Skin. 10th ed. New Delhi: Wolters Kluwer (India); 2009.p. 851-909.  Back to cited text no. 7
    
8.
Konstantinova AM, Michal M, Kacerovska D, Spagnolo DV, Stewart CJ, Kutzner H, et al. Hidradenoma papilliferum: A clinicopathologic study of 264 tumors from 261 patients, with emphasis on mammary-type alterations. Am J Dermatopathol 2016;38:598-607.  Back to cited text no. 8
    
9.
Kazakov DV, Mikyskova I, Kutzner H, Simpson RH, Hes O, Mukensnabl P, et al. Hidradenoma papilliferum with oxyphilic metaplasia: A clinicopathological study of 18 cases, including detection of human papillomavirus. Am J Dermatopathol 2005;27:102-10.  Back to cited text no. 9
    
10.
Elbendary A, Cochran E, Xie Q, Kabigting F, Pereira L, Elston DM, et al. Hidradenoma papilliferum with oncocytic metaplasia: A histopathological and immunohistochemical study. Am J Dermatopathol 2016;38:444-7.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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