DERMATOPATHOLOGY CASES: Self-Assessment Cases: Editor - Dr Sampurna Roy MD

Digital Images of interesting cases that will include the full spectrum of Dermatopathology, presented in the form of quiz.

The answer of the cases include related links and recent abstracts of articles.

Monday, January 23, 2012

Answer of Dermatopathology Case 113

Extramammary Paget's Disease

Visit: Dermatopathology Site
Visit: Pathology of Extramammary Paget's Disease


Extramammary Paget's disease: Evaluation of the histopathological patterns of Paget cell proliferation in the epidermis.J Dermatol. 2011 Nov;38(11):1054-7. doi: 10.1111/j.1346-8138.2011.01268.x. Epub 2011 Sep 23.
Extramammary Paget's disease (EMPD) is a rare malignant skin neoplasm. The prototypical pattern of tumor cell proliferation in the epidermis includes single cells and/or nest arrangements, mainly in the lower epidermis. Although other patterns have been recognized, they have not previously been investigated in detail. We aimed to examine the patterns of tumor cell proliferation in the epidermis. Surgical specimens were obtained from 38 patients with primary EMPD. We defined six patterns, in addition to the prototypical one: (i) glandular; (ii) acantholysis-like; (iii) upper nest; (iv) tall nest; (v) budding; and (vi) sheet-like. There were 26 males and 12 females (mean age, 75.0 years). Lesions were located on the scrotum (26 cases) and vulva (12). There were 22 in situ EMPD and 16 invasive EMPD. The frequencies of the different proliferation patterns were: glandular, 36.8%; acantholysis-like, 73.7%; upper nest, 68.4%; tall nest, 28.9%; budding, 47.4%; and sheet-like, 23.7%. Upper nest pattern and the presence of more than three patterns were significantly more frequent in invasive EMPD than in situ EMPD (P < 0.05). We identified the histopathological patterns of Paget cell proliferation in the epidermis in EMPD, and suggest that the characteristic patterns and the diversity of patterns could be associated with progression and dermal invasion in EMPD.

Primary Extramammary Paget's Disease Combined with Bowen's Disease in Vulva.Ann Dermatol. 2011 Oct;23(Suppl 2):S222-5. Epub 2011 Oct 31.
Extramammary Paget's disease (EMPD) is a uncommon neoplastic condition of apocrine gland-bearing skin and its occurrence in combination with Bowen's disease is very rare. The most common site of involvement is the vulva, although perineal, perianal, scrotal and penile skin may also be affected. EMPD is usually not combined with Bowen's disease. We report an interesting case of EMPD combined with Bowen's disease, which was confirmed by immunohistochemical stain.

Paget's disease of the vulva: Diagnosis and follow-up key to management; a retrospective study of 50 cases from Queensland.Gynecol Oncol. 2011 Jul;122(1):42-4. Epub 2011 Apr 17.
To review the clinical features, diagnosis, management, and outcomes for the 50 cases of Paget's disease (PD) of the vulva referred to Queensland Centre for Gynaecological Cancer between 1986 and 2009.
Vulvar PD cases from QCGC were reviewed and analyzed using the computer software Statistical Package for the Social Sciences (SPSS) 11.0.
Paget's disease (PD) of the vulva is uncommon. Of the 50 patients, 2 have died of their PD, 1 patient that had coexisting PD died of squamous cell vulva cancer, and 11 died of unrelated causes. The mean age at diagnosis was 67.6 years (range, 31 to 91). All cases were Caucasian. Time from onset of symptoms to diagnosis averaged 21 months. Not until a biopsy was performed was the diagnosis made. The most common presenting complaint was pruritis (27 cases, 54%). There was no identifiable "favored" site on the vulva for PD. Positive groin lymph nodes were found in 4 of the 10 cases who underwent node biopsy. Two who had poorly differentiated carcinoma in the nodes and PD died of disease within a year of diagnosis, one is alive three years later. The fourth case had coincidental PD and vulvar squamous cell carcinoma with squamous carcinoma groin nodes. Initial treatment was surgical.
The prognosis for primary extra-mammary PD of the vulva confined to the epidermis (IEP) is excellent. Early diagnosis and long term follow-up are the keys to successful management. The status of disease at the margins of surgical specimens does not reliably equate to patient long term outcomes.