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 114

Deep Penetrating Nevus

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Abstract:

Deep penetrating nevus: a review.Arch Pathol Lab Med. 2011 Mar;135(3):321-6.
CONTEXT:
Deep penetrating nevus is a distinctive melanocytic lesion that may simulate melanoma both clinically and histologically.
OBJECTIVE:
To review clinical and histologic features of deep penetrating nevi and discuss their differential diagnosis, especially regarding melanoma.
DATA SOURCES:
The literature on deep penetrating nevi is reviewed and supplemented by our experiences with deep penetrating nevi.
CONCLUSIONS:
One or more disturbing histologic features may frequently be found in deep penetrating nevi, including asymmetry, plump but fairly regular nests of melanocytes in the dermis, cytologic atypia with some nuclear pleomorphism, a small to medium-sized eosinophilic nucleolus, absence of maturation, occasional presence of normal dermal mitoses, and a patchy mononuclear inflammatory cell infiltrate. Although unusual, such histologic features should not be regarded as a sign of malignancy in deep penetrating nevi.

Pigmented lesion pathology: what you should expect from your pathologist, and what your pathologist should expect from you.Clin Plast Surg. 2010 Jan;37(1):1-20.
The first part of this review examines the reliability of histologic diagnosis in pigmented lesions, as measured by concordance studies and medicolegal analysis. It emphasizes the role of clinicians in maximizing that reliability, by providing adequate clinical descriptions, using appropriate biopsy technique, and critically interpreting pathology reports. It identifies those entities that are especially problematic, either because they cannot be reliably recognized by the histopathologist or because their histology is a poor guide to their biologic behavior. The second part of the review is a guide to some of the more difficult and controversial pigmented lesions, including dysplastic nevus, spitzoid nevi and melanomas, cellular blue nevus, animal-type melanoma, and deep penetrating nevus.

Dermatopathology Case 114

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Case 114

A dark pigmented papule on the neck of a 25 year old female.

Answer of Dermatopathology Case 113

Extramammary Paget's Disease

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Visit: Pathology of Extramammary Paget's Disease

Abstract:

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.
OBJECTIVE:
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.
METHODS:
Vulvar PD cases from QCGC were reviewed and analyzed using the computer software Statistical Package for the Social Sciences (SPSS) 11.0.
RESULTS:
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.
CONCLUSIONS:
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.

Dermatopathology Case 113

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Case 113

A 54 year old woman with a thickened plaque on the vulva. The lesion is crusted with scale covering its surface.


Thursday, January 12, 2012

Answer of Dermatopathology Case 112

Pleomorphic Fibroma

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Abstract:

Pleomorphic fibroma of the skin: a benign neoplasm with cytologic atypia. A clinicopathologic study of eight cases.Am J Surg Pathol. 1989 Feb;13(2):107-13.
A clinicopathologic study of eight examples of polypoid and dome-shaped cutaneous fibrous lesions with sparse cellularity but striking nuclear atypia and rare mitotic figures is presented. Positive immunohistochemical staining for vimentin and actin supported the fibroblastic nature of these lesions. All eight cases were adults whose ages ranged from 33 to 67 years (mean 52 years). Five were women and three were men. Five lesions were located on extremities, two on the trunk, and one on the face and they measured from 4 to 16 mm in greatest dimension. The lesions were clinically followed from 4 months to 5 years. They all showed benign clinical behavior, with only one local recurrence in a lesion that had been incompletely removed. The nuclear atypia seen in these fibrous lesions may be similar to that which occurs in other benign mesenchymal neoplasms, such as pleomorphic lipoma, pleomorphic leiomyoma, ancient schwannoma, and variants of dermatofibroma with atypical cells. We suggest that "pleomorphic fibroma" is an appropriate term for this lesion based on its histologic differentiation, cytologic atypia, and benign clinical course.

Pleomorphic fibroma of the skin, a form of sclerotic fibroma: an immunohistochemical study.Clin Exp Dermatol. 1998 Jan;23(1):22-4.
We report a new case of pleomorphic fibroma of the skin arising on the face. The lesion was polypoid and fibrous, located on the dermis, and formed by coarse collagen bundles with sparse cells. It was also characterized by the presence of cellular atypia and pleomorphism without mitosis. Immunohistochemistry showed staining for vimentin and actin and negativity for S-100, CD34, CD68, alpha-1-antichymotrypsin and alpha-1-antitrypsin antigens, supporting a myofibroblastic origin. A few isolated cells also showed granular cytoplasmic detection of factor XIIIa. Although the disorder has been previously considered to be a tumour of preferential localization on the trunk or extremities, this does not always hold true as four of the 14 reported cases arose on the head. Pleomorphic fibroma is a benign condition and is we believe a variant of sclerotic fibroma.

Dermatopathology Case 112

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Case 112

A 51 year old female with a polypoid cutaneous lesion on the left extremity.