Clinical details:

This is a vulval scraping smear of 44 years old woman who presented with complaints of itching and hysterectomy fourteen years ago for DUB. Physical examination showed an ulcerated and painful 1x1 cm well circumscribed nodular exophytic growth on the labia majora.

Images:

Image 1 (Pap, 400x)

Image 2 (Pap, 400x)

Image 3 (Pap, 400x)

Image 4 (Pap, 200x)

Choose correct answer:

1) Low-grade adenocarcinoma originating in the sweat gland

2) Extra-mammary Paget’s disease

3) Papillary Hidradenoma of vulva

The correct answer is Papillary hidradenoma of vulva

Clinical details:

This is a vulval scraping smear of 44 years old woman who presented with complaints of itching and hysterectomy fourteen years ago for DUB. Physical examination showed an ulcerated and painful 1x1 cm well circumscribed nodular exophytic growth on the labia majora.

Description:

Cellular smear show many singly occurring, groups and sheets of benign glandular cells. They show moderate amount of cytoplasm. Nuclei are eccentrically placed with finely granular, evenly distributed chromatin pattern. The nuclear membrane is smooth. Conspicuous eosinophilic nucleoli are seen.

Discussion:

Low-grade adenocarcinoma shows overlapping clusters of normal-looking epithelial cells with loss of honeycomb pattern and nuclear crowding. Loss of polarity may be observed. The cells in this case are seen in flat sheets and few groups showing overlapping. However no loss of polarity is observed and the cells have benign morphology. The most important feature against a low-grade adenocarcinoma is the presence of prominent nucleoli.

The differential of Paget’s disease can be considered due to the location of the lesion and the presence of prominent nucleoli. The smear picture of Paget’s disease shows singly occurring or clusters of Paget’s cells with pale cytoplasm and indistinct cytoplasmic outline. The nuclei are enlarged with abnormal chromatin pattern. The signet ring morphology may also be noted. The case image shows rather dense abundant cytoplasm with distinct cell borders. The nuclear abnormality is not evident. This rules out the presence of Paget’s disease.

Papillary hidradenoma is a benign tumor of apocrine origin. They present as nodular, well-circumscribed growth, less than 2 cm in diameter on the labia majora. They may ulcerate. These lesions are most frequently diagnosed clinically or on biopsy. On cytology clumps of slightly pleomorphic glandular cells are seen. This is an abnormal finding in a vulval scraping smear. This should raise a suspicion and a possibility of hidradenoma of the vulva should be considered. Correlation with the clinical findings and biopsy confirmation is necessary to arrive at a definite diagnosis.

The point to remember is cytologically hidradenoma of the vulva should not be misinterpreted as a carcinoma.

Follow-up:

The local excision of the growth was done and the histology was Papillary hidradenoma of the vulva. The patient is well with no recurrence.

References:

  1. Schramm G: Diagnosis of a Papillary Hidradenoma of the Vulva by Simultaneous Cytology and Colposcopy Acta Cytol 1979; Jan-Feb 23: 57-60.
  2. Hustin: Identification of Papillary Hidradenoma of the Vulva by Imprint Cytology, Acta Cytol 1980; Sept-Oct 24:466-467
  3. Daniel F, Mahmoudi A, de Parades V, Flejou JF, Atienza P: An uncommon peri-anal nodule: Hidradenoma papilliferum, Gastroenterol Clinical Biol 2007; Feb 31 (2):166-168.
  4. Kazakov DV, Mikyskova I, Kutzner H, Simpson RH, et al: Hidradenoma Papilliferum with oxyphilic metaplasia: a clinicopathological study of 18 cases, including detection of human papillomavirus, Am J Dermatopathol 2005; April 27(2): 102-110.
  5. Smith FB, Shemen LJ, Guerrieri C, Ismail SS: Hidradenoma papilliferum of nasal skin, Arch Pathol Lab Med 2003; Feb 127(2): E86-88.
  6. Vang R, Cohen PR: Ectopic hidradenoma papilliferum: a case report and review of the literature, J Am Acad Dermatol 1999; July 41(1): 115-118.
  7. Kaufmann T, Pawl NO, Soifer I, Greston WM, Kleiner GJ : Cystic papillary hidradenoma of the vulva:Case report and review of the literature, Gynecol Oncol 1987; Feb 26 (2) : 240-245.
  8. Koss LG: Diseases of the Vagina and Vulva in Diagnostic Cytopathology and its Histopathologic Bases, 4th Ed.,J B Lippincott Company Philadelphia 1992:600-603.
  9. Johnson J : Tumours of the vulva and vagina In Diagnostic Cytopathology 2nd Ed., edited by Gray W & Mckee GT, Churchill Livingstone 2003: 813.

Case provided by: Mrs. Maya Uke