Ectopic Extramammary Paget's Disease Research Articles

Solitary ectopic extramammary Paget's disease in the epigastric region.

Solitary ectopic extramammary Paget's disease in the epigastric region.

Ectopic extramammary Paget disease in thoracic location

Extramammary Paget disease (EMPD) is a rare skin cancer that affects areas with a high concentration of apocrine glands including genital, axillary, and anal skin. When it affects other locations it is called ectopic extramammary Paget disease (E-EMPD) and is uncommon. To date, there are only 45 case reports to the best of our knowledge. The clinical manifestation is typically a soft, red or bright pink patch or plaque with scattered white islands of hyperkeratosis and erosion. Diagnostic confirmation requires conventional histology with immunohistochemistry. The importance of immunohistochemical staining for the diagnosis of primary neoplasia, without underlying malignancy, is highlighted. We report the first Latin American confirmed case, to our knowledge, of primary E-EMPD in a 55-year-old man with a 1-year history of asymptomatic thoracic plaque.

Ectopic Extramammary Paget Disease Occurring on the Nose

Ectopic Extramammary Paget Disease Occurring on the Nose

Ectopic Extramammary Paget’s Disease: Case Report and Literature Review

Extramammary Paget's disease that occurs in non-apocrine-bearing regions is referred to as ectopic and has been rarely reported. A 62-year-old man presented with a slowly progressive, asymptomatic light-brown plaque on his back. Histopathological examination revealed the presence of large pale cells with prominent nuclei, which proliferated diffusely and focally in the epidermis. Immuno-histochemically the tumour cells were positive for CK7, GCDFP-15, CEA, and p63. Based on these findings, we diagnosed the tumour as ectopic extramammary Paget's disease. We reviewed the English and Japanese literature and found 29 previously reported cases of ectopic extramammary Paget's disease, including our case, with a predominance of occurrence in the Asian population. The germinative milk line is known to be a possible site where extramammary Paget's disease occurs. Like-wise, some germinative apocrine-differentiating cells might exist on the trunk preferentially in Asians. Attention should be paid to the development of ectopic as well as triple or quadruple EMPD in Asians.

Extramammary Paget’s disease presenting as alopecia neoplastica

Extramammary Paget's disease (EMPD) is a rare malignant neoplasm with a predilection for the apocrine-rich anogenital skin and less commonly for the axilla. The tumor rarely occurs in non-apocrine bearing regions where it is referred to as ectopic EMPD. Here, we report a case of a patient that presented with a poorly circumscribed, erythematous plaque with patchy alopecia on the scalp. Histology showed pagetoid infiltration of the epidermis by atypical single and nested cells with abundant amphophilic cytoplasm with nuclear hyperchromasia, extending along the adnexae. Immunohistochemistry showed that the tumor cells stained positively for mucicarmine, periodic acid schiff, cytokeratin-7, polyclonal carcinoembryonic antigen, epithelial membrane antigen, gross cystic disease fluid protein, androgen receptor and Her-2-neu; and negatively for S-100, HMB-45, CDX-2, thyroid transcription factor-1, estrogen receptor and progesterone receptor, thus, establishing the diagnosis of ectopic EMPD. Subsequent workup showed no underlying malignancy. To our knowledge, we report the second case, and the first in the English literature, of an ectopic EMPD involving the scalp without any associated malignancy.

左上腹部に生じた異所性乳房外パジェット病の1例

65歳, 女性。既往歴として子宮体癌, 小腸消化管間葉系腫瘍があり2003年4月子宮全摘術, 腫瘍摘出術が施行されている。約13年前より腹部に出現した皮疹が徐々に拡大してきたため当科を受診した。左側腹部に径40×27mm大, 表面平滑, 境界明瞭, 周辺部が褐色調を呈する紅褐色局面を認め, 一部に赤色の点状変化がみられた。皮膚生検を施行したところ, HE染色にて表皮内に胞体の明るい異形細胞がみられたため表在拡大型悪性黒色腫が疑われた。しかし, 特殊染色を施行したところ腫瘍細胞は, HMB-45 (-) , S-100 (-) , CK7 (+) , CEA (+) , アルシャンブルー (+) であったため, 本症例を異所性乳房外パジェット病と診断した。全身検索から内臓病変や転移所見は否定的であったため, 病変部より3cm離して拡大切除術を施行した。2008年6月の現在で約2年を経過するが, 腫瘍の再発転移はみられない。

Treatment of double ectopic extramammary Paget's disease of bilateral chest with imiquimod 5% cream

Treatment of double ectopic extramammary Paget's disease of bilateral chest with imiquimod 5% cream

Extramammary Paget's Disease with Bowenoid Histologic Features Accompanied by an Ectopic Lesion on the Upper Abdomen

We present a 79-year-old man who suffered from extramammary Paget's disease (EMPD) with bowenoid histological features accompanied by an ectopic EMPD lesion on his abdomen. He had had an erythematous plaque on his genital region for three years. Based on a biopsy specimen, he was referred to our hospital with the histological diagnosis of Bowen's disease. The histological findings of the genital lesion obtained by surgical resection showed typical areas of Paget's cells adjacent to areas characteristic of Bowen's disease. Immunohistochemical findings showed CEA and CK7 positive tumor cells in both areas, so the atypical cells showing the bowenoid pattern could be regarded as tumor cells of Paget's disease. Immunohistochemical staining for CEA and CK7 along with multiple biopsies can be helpful in making the diagnosis of Paget's disease with bowenoid histologic features.

Ectopic extramammary Paget's disease affecting the upper abdomen

Ectopic extramammary Paget's disease affecting the upper abdomen

Ectopic extramammary Paget's disease affecting the upper abdomen

We present 57-year-old man in whom ectopic extrammary Paget's disease (EMPD) affected the upper abdomen. Although the clinical appearance was suggestive of Bowen's disease or superficial basal cell epithelioma (BCE), the biopsy specimen showed EMPD histologically. Only 12 cases of ectopic EMPD have been reported (including this case). In our 20 year experience of 129 EMPD, this is the first ectopic case. Thus, the frequency of ectopic EMPD is 0.78% (one of 129) in our study. The male/female ratio in the reported 12 cases is 2: 1, nearly the same as EMPD in general (2.1: 1, in our 129 cases). The mean age of the 12 patients is 65.8 years, which is not significantly different from ordinary EMPD (66.4 years, in our 129 cases). Comparing ectopic EMPD to ordinary EMPD, clinically and histologically, we could find no difference. As they appear to be the same disease, ectopic and ordinary EMPD may share similar origins and mechanisms of occurrence. We support the hypothesis that Paget's cells originate from the remaining pluripotential germinative cells which are able to differentiate into many kinds of secreting glands.