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Extramammary Paget's disease with the appearance of a nodule: a case report.

Wang X, Yang W, Yang J - BMC Cancer (2010)

Bottom Line: EMPD is mainly composed of intraepidermal Paget cells, and possesses variable clinical behaviors and histological appearances, leading to difficulty in the diagnosis of this disease.We here report a case of primary EMPD with the appearance of a nodule on the background of erythema.Using immunohistochemistry, the expressions of CK7, CK19, CK20, GCDFP-15, CEA, S-100 protein and bcl-2 were examined to elucidate the cellular differentiation of the carcinoma.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Dermatology, the Second Affiliated Hospital of Guangzhou Medical University, Guangzhou 510260, P. R. China. wenliny@21cn.com

ABSTRACT

Background: Extramammary Paget's disease (EMPD) remains a rare condition with only a limited number of cases reported in the literature. EMPD is mainly composed of intraepidermal Paget cells, and possesses variable clinical behaviors and histological appearances, leading to difficulty in the diagnosis of this disease.

Case presentation: We here report a case of primary EMPD with the appearance of a nodule on the background of erythema. Histological assessment showed Paget cell infiltration throughout the epidermis with dermal spread. Using immunohistochemistry, the expressions of CK7, CK19, CK20, GCDFP-15, CEA, S-100 protein and bcl-2 were examined to elucidate the cellular differentiation of the carcinoma.

Conclusion: According to the histological assessment, this case was diagnosed as primary EMPD with carcinoma cells invading into the dermis, but without lymph node infiltration.

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The clinical features of the patient with EMPD. Red firm mass on the centre of irregular warm eczematoid erythema covering the woman mons pubis.
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Figure 1: The clinical features of the patient with EMPD. Red firm mass on the centre of irregular warm eczematoid erythema covering the woman mons pubis.

Mentions: A 66-year-old Chinese woman presented with a slowly growing vulvar mass with pruritus. Two years prior to presentation, she noted a painless red firm nodule on her vulva. Subsequently, erythema around the nodule appeared. The nodule and erythema had gradually enlarged during the 2-year period. Physical examination revealed a 2 cm × 2 cm × 2 cm slightly erosive, nodular red firm mass on the centre of 5 cm × 7 cm area of irregular eczematoid erythema covering her mons pubis (Fig.1). There was no swelling of lymph nodes in bilateral groins. Biopsy of inguinal lymph node was negative. The complete mass and part of the erythema were excised for histological evaluation. The patient's medical history was unremarkable. Metastasis from other cancers was excluded by routine blood tests, cystoscopy, oesophagogastroduodenoscopy, colonoscopy, abdominopelvic computed tomography (CT), chest radiography and positron emission tomography-CT. All these examinations were normal. At 3-month follow-up, the patient had local mass recurrence and non-palpable inguinal lymph nodes.


Extramammary Paget's disease with the appearance of a nodule: a case report.

Wang X, Yang W, Yang J - BMC Cancer (2010)

The clinical features of the patient with EMPD. Red firm mass on the centre of irregular warm eczematoid erythema covering the woman mons pubis.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2921398&req=5

Figure 1: The clinical features of the patient with EMPD. Red firm mass on the centre of irregular warm eczematoid erythema covering the woman mons pubis.
Mentions: A 66-year-old Chinese woman presented with a slowly growing vulvar mass with pruritus. Two years prior to presentation, she noted a painless red firm nodule on her vulva. Subsequently, erythema around the nodule appeared. The nodule and erythema had gradually enlarged during the 2-year period. Physical examination revealed a 2 cm × 2 cm × 2 cm slightly erosive, nodular red firm mass on the centre of 5 cm × 7 cm area of irregular eczematoid erythema covering her mons pubis (Fig.1). There was no swelling of lymph nodes in bilateral groins. Biopsy of inguinal lymph node was negative. The complete mass and part of the erythema were excised for histological evaluation. The patient's medical history was unremarkable. Metastasis from other cancers was excluded by routine blood tests, cystoscopy, oesophagogastroduodenoscopy, colonoscopy, abdominopelvic computed tomography (CT), chest radiography and positron emission tomography-CT. All these examinations were normal. At 3-month follow-up, the patient had local mass recurrence and non-palpable inguinal lymph nodes.

Bottom Line: EMPD is mainly composed of intraepidermal Paget cells, and possesses variable clinical behaviors and histological appearances, leading to difficulty in the diagnosis of this disease.We here report a case of primary EMPD with the appearance of a nodule on the background of erythema.Using immunohistochemistry, the expressions of CK7, CK19, CK20, GCDFP-15, CEA, S-100 protein and bcl-2 were examined to elucidate the cellular differentiation of the carcinoma.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Dermatology, the Second Affiliated Hospital of Guangzhou Medical University, Guangzhou 510260, P. R. China. wenliny@21cn.com

ABSTRACT

Background: Extramammary Paget's disease (EMPD) remains a rare condition with only a limited number of cases reported in the literature. EMPD is mainly composed of intraepidermal Paget cells, and possesses variable clinical behaviors and histological appearances, leading to difficulty in the diagnosis of this disease.

Case presentation: We here report a case of primary EMPD with the appearance of a nodule on the background of erythema. Histological assessment showed Paget cell infiltration throughout the epidermis with dermal spread. Using immunohistochemistry, the expressions of CK7, CK19, CK20, GCDFP-15, CEA, S-100 protein and bcl-2 were examined to elucidate the cellular differentiation of the carcinoma.

Conclusion: According to the histological assessment, this case was diagnosed as primary EMPD with carcinoma cells invading into the dermis, but without lymph node infiltration.

Show MeSH
Related in: MedlinePlus