Co-occurrence of apocrine adenocarcinoma and invasive mammary-type ductal carcinoma in extramammary Paget disease of the axilla

Abstract

Extramammary Paget disease (EMPD) is an uncommon malignancy that occurs in apocrine gland-rich areas of the body. EMPD of the axilla is rare, but a few cases have been reported. Some cases of EMPD have been reported with underlying apocrine adenocarcinoma; rarely, mammary-type ductal carcinoma can accompany EMPD. Here, we report a very rare case of EMPD with apocrine adenocarcinoma and invasive mammary-type ductal carcinoma. A 55-year-old woman was referred with a brownish pigmented plaque accompanying an area of ulceration in the left axilla. A preoperative biopsy indicated Paget disease, and an additional evaluation was performed to determine whether it was of primary or secondary origin. A wide excision was made, and the axilla was reconstructed using a thoracodorsal artery perforator flap. The biopsy showed apocrine adenocarcinoma and invasive mammary-type ductal carcinoma with pagetoid spreading. The patient had no evidence of recurrence or other postoperative complications.

Atypical cells on reflectance confocal microscopy may not represent melanoma: A case of axillary pigmented extramammary Paget disease

“…The true diagnosis of PEMPD was elucidated after immunohistochemistry (IHC) was performed for markers expressed in melanocytic and Paget cells. To our knowledge, this is the first case of PEMPD occurring in the axilla to be described on RCM, and the fifth reported case of axillary PEMPD in the English literature 5,6,8,14 . It is also the first case of PEMPD to be described explicitly in an African American patient.…”

“…Immunostaining for CK7-the most sensitive and specific marker for EMPD 3 , as well as CK8/18, helped to identify the round-oval single and nested cells as Paget cells. In other reported cases of PEMPD, Paget cells were most commonly positive for: CK7, 2,3,[5][6][7][8][9][10][11][12][13][14]16 carcinoembryonic antigen (CEA), 1,2,4,5,[10][11][12]14,16 cytokeratin CAM 5.2, 1,5,7,8,10,12,16 epithelial membrane antigen (EMA), 3,5,8,12,16 and gross cystic disease fluid protein 15 (GCDFP-15). [5][6][7]12 Dendritic melanocytes were generally positive for human melanoma black 45 (HMB-45), 1,2,4,5,[7][8][9]11,12 melanoma antigen/melanoma antigen recognized by T cells...…”

“…To our knowledge, this is the first case of PEMPD occurring in the axilla to be described on RCM, and the fifth reported case of axillary PEMPD in the English literature. 5,6,8,14 It is also the first case of PEMPD to be described explicitly in an African American patient.…”

“…PEMPD has been reported in men and women, of white and Asian races, with presentation in the fifth to ninth decades of life (average age 62). [1][2][3][4][5][6][7][8][9][10][11][12][13][14] Lesions were primarily located in the genital area (vulva, scrotum, and perineum), and less commonly on the axilla, abdomen, and thigh. Most were clinically interpreted as other pigmented neoplasms, including seborrheic keratosis, atypical melanocytic nevus, and melanoma.…”

“…There are few well‐described cases of PEMPD in the literature to date, most of which highlight its diagnostic challenges. PEMPD has been reported in men and women, of white and Asian races, with presentation in the fifth to ninth decades of life (average age 62) 1‐14 . Lesions were primarily located in the genital area (vulva, scrotum, and perineum), and less commonly on the axilla, abdomen, and thigh.…”