Limits...
Extramammary Paget's disease affecting the external auditory canal: a case report.

Bomfim Fde A, Mendes Wde O, Veras IM, de Freitas JC, Castro AM, de Castro Junior FM - J Med Case Rep (2013)

Bottom Line: He had peripheral facial nerve paralysis and complete ipsilateral hearing loss.The tumor was considered inoperable because of the extensive skull base involvement, and he was referred for palliative radiotherapy.In all the three cases, the prognosis was unfavorable despite treatment.

View Article: PubMed Central - HTML - PubMed

ABSTRACT

Introduction: Extramammary Paget's disease is a rare histological type of intraepithelial adenocarcinoma that mainly affects apocrine sweat gland-rich areas. Predilection sites include the anogenital region and, less commonly, the axillae. These tumors rarely occur in non-apocrine regions. The aim of this case report is to describe a case of extramammary Paget's disease in the external auditory canal with extensive temporal bone involvement and skull base invasion.

Case presentation: A 40-year-old Caucasian man presented with a progressively growing, vegetating lesion localized in his right-hand external auditory canal. He had peripheral facial nerve paralysis and complete ipsilateral hearing loss. An incisional biopsy suggested extramammary Paget's disease, and the immunohistochemical analysis confirmed the diagnosis. The tumor was considered inoperable because of the extensive skull base involvement, and he was referred for palliative radiotherapy.

Conclusion: We report here the third case of extramammary Paget's disease affecting the external auditory canal to be described in the literature. In all the three cases, the prognosis was unfavorable despite treatment.

No MeSH data available.


Related in: MedlinePlus

Histopathological examination and immunohistochemical study. Detailed legend: (a) An extensive infiltrate with involvement largely concentrated in the deep epidermis. Epidermal hyperplasia and hyperkeratosis occurred to some degree (hematoxylin and eosin staining, original magnification ×40). (b) The tumor cells were large, with distinct borders, abundant pale cytoplasm, pleomorphic nuclei, and conspicuous nucleoli (hematoxylin and eosin staining, original magnification ×200). (c) Strong human epidermal growth factor receptor 2-positive membranous reactivity was detected in Paget’s cells in the epidermis (original magnification ×200). (d) Paget’s cells showing diffuse positivity for carcinoembryonic antigen (original magnification ×400). (e) Extramammary Paget’s disease manifested as cytokeratin-positive malignant cell epidermal infiltration (original magnification ×40). (f) Reactivity for gross cystic disease fluid protein of 15kDa (original magnification ×200).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4226198&req=5

Figure 2: Histopathological examination and immunohistochemical study. Detailed legend: (a) An extensive infiltrate with involvement largely concentrated in the deep epidermis. Epidermal hyperplasia and hyperkeratosis occurred to some degree (hematoxylin and eosin staining, original magnification ×40). (b) The tumor cells were large, with distinct borders, abundant pale cytoplasm, pleomorphic nuclei, and conspicuous nucleoli (hematoxylin and eosin staining, original magnification ×200). (c) Strong human epidermal growth factor receptor 2-positive membranous reactivity was detected in Paget’s cells in the epidermis (original magnification ×200). (d) Paget’s cells showing diffuse positivity for carcinoembryonic antigen (original magnification ×400). (e) Extramammary Paget’s disease manifested as cytokeratin-positive malignant cell epidermal infiltration (original magnification ×40). (f) Reactivity for gross cystic disease fluid protein of 15kDa (original magnification ×200).

Mentions: The incisional biopsy suggested EMPD (Figure 2a, b), but there was a possible differential diagnosis of amelanotic melanoma or pagetoid Bowen’s disease. The immunohistochemical study (Table 1) was positive for human epidermal growth factor receptor 2 (Her2 or Neu; Figure 2c), carcinoembryonic antigen (CEA; Figure 2d), cytokeratin-7 (CK7; Figure 2e), and gross cystic disease fluid protein of 15kDa (GCDFP-15; Figure 2f), confirming the diagnosis of EMPD. A computed tomography scan revealed an infiltrative lesion in his external right ear, with paravertebral and middle ear extensions that affected his internal carotid artery anterior wall and facial nerve, respectively (Figure 3a). Magnetic resonance imaging revealed an expansive tumor with imprecise limits and no cleavage plane with the carotid artery. An extension to the parapharyngeal space and an invasion of the middle fossa, associated with meningeal contrast enhancement, were observed (Figure 3b).


Extramammary Paget's disease affecting the external auditory canal: a case report.

Bomfim Fde A, Mendes Wde O, Veras IM, de Freitas JC, Castro AM, de Castro Junior FM - J Med Case Rep (2013)

Histopathological examination and immunohistochemical study. Detailed legend: (a) An extensive infiltrate with involvement largely concentrated in the deep epidermis. Epidermal hyperplasia and hyperkeratosis occurred to some degree (hematoxylin and eosin staining, original magnification ×40). (b) The tumor cells were large, with distinct borders, abundant pale cytoplasm, pleomorphic nuclei, and conspicuous nucleoli (hematoxylin and eosin staining, original magnification ×200). (c) Strong human epidermal growth factor receptor 2-positive membranous reactivity was detected in Paget’s cells in the epidermis (original magnification ×200). (d) Paget’s cells showing diffuse positivity for carcinoembryonic antigen (original magnification ×400). (e) Extramammary Paget’s disease manifested as cytokeratin-positive malignant cell epidermal infiltration (original magnification ×40). (f) Reactivity for gross cystic disease fluid protein of 15kDa (original magnification ×200).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4226198&req=5

Figure 2: Histopathological examination and immunohistochemical study. Detailed legend: (a) An extensive infiltrate with involvement largely concentrated in the deep epidermis. Epidermal hyperplasia and hyperkeratosis occurred to some degree (hematoxylin and eosin staining, original magnification ×40). (b) The tumor cells were large, with distinct borders, abundant pale cytoplasm, pleomorphic nuclei, and conspicuous nucleoli (hematoxylin and eosin staining, original magnification ×200). (c) Strong human epidermal growth factor receptor 2-positive membranous reactivity was detected in Paget’s cells in the epidermis (original magnification ×200). (d) Paget’s cells showing diffuse positivity for carcinoembryonic antigen (original magnification ×400). (e) Extramammary Paget’s disease manifested as cytokeratin-positive malignant cell epidermal infiltration (original magnification ×40). (f) Reactivity for gross cystic disease fluid protein of 15kDa (original magnification ×200).
Mentions: The incisional biopsy suggested EMPD (Figure 2a, b), but there was a possible differential diagnosis of amelanotic melanoma or pagetoid Bowen’s disease. The immunohistochemical study (Table 1) was positive for human epidermal growth factor receptor 2 (Her2 or Neu; Figure 2c), carcinoembryonic antigen (CEA; Figure 2d), cytokeratin-7 (CK7; Figure 2e), and gross cystic disease fluid protein of 15kDa (GCDFP-15; Figure 2f), confirming the diagnosis of EMPD. A computed tomography scan revealed an infiltrative lesion in his external right ear, with paravertebral and middle ear extensions that affected his internal carotid artery anterior wall and facial nerve, respectively (Figure 3a). Magnetic resonance imaging revealed an expansive tumor with imprecise limits and no cleavage plane with the carotid artery. An extension to the parapharyngeal space and an invasion of the middle fossa, associated with meningeal contrast enhancement, were observed (Figure 3b).

Bottom Line: He had peripheral facial nerve paralysis and complete ipsilateral hearing loss.The tumor was considered inoperable because of the extensive skull base involvement, and he was referred for palliative radiotherapy.In all the three cases, the prognosis was unfavorable despite treatment.

View Article: PubMed Central - HTML - PubMed

ABSTRACT

Introduction: Extramammary Paget's disease is a rare histological type of intraepithelial adenocarcinoma that mainly affects apocrine sweat gland-rich areas. Predilection sites include the anogenital region and, less commonly, the axillae. These tumors rarely occur in non-apocrine regions. The aim of this case report is to describe a case of extramammary Paget's disease in the external auditory canal with extensive temporal bone involvement and skull base invasion.

Case presentation: A 40-year-old Caucasian man presented with a progressively growing, vegetating lesion localized in his right-hand external auditory canal. He had peripheral facial nerve paralysis and complete ipsilateral hearing loss. An incisional biopsy suggested extramammary Paget's disease, and the immunohistochemical analysis confirmed the diagnosis. The tumor was considered inoperable because of the extensive skull base involvement, and he was referred for palliative radiotherapy.

Conclusion: We report here the third case of extramammary Paget's disease affecting the external auditory canal to be described in the literature. In all the three cases, the prognosis was unfavorable despite treatment.

No MeSH data available.


Related in: MedlinePlus