Medication
Medication Summary
The goals of pharmacotherapy are to reduce morbidity and prevent complications. A Cochrane review of aminolevulinic acid photodynamic therapy for mammary and extramammary Paget disease identified 21 retrospective and two prospective noncomparative studies with a total of 99 patients. The results suggest that this modality might be considered as adjunctive therapy for selected patients. [36]
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Topical Skin Products
Class Summary
These agents provide a photodynamic treatment for low-risk malignant cells and may improve morbidity.
Aminolevulinic acid (Levulan Kerastick)
Aminolevulinic acid is a metabolic precursor of the photosensitizer protoporphyrin lX. When exposed to light of appropriate wavelength and energy, it induces local cytotoxicity through a photodynamic reaction.
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Media Gallery
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Schematic diagram of female breast depicting widely accepted concept of pathogenesis of mammary Paget disease. Malignant Paget cells are derived from luminal lactiferous ductal epithelium (A) of breast tissue with retrograde extension of cancerous Paget cells into epidermis of overlying nipple (B). Enlarged circle shows details that reveal thickening both of lining epithelium of breast duct and of nipple skin.
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Biopsy-proven Paget disease involving nipple of 56-year-old woman. Patient noted erythematous, swollen, enlarged nipple with focal ulceration and oozing; occasional serosanguineous discharge and bleeding were present. Patient was later found to have palpable breast mass and mammography results positive for subareolar microcalcification; no auxiliary lymphadenopathy was found. Patient was treated by simple mastectomy. At 5-year follow-up, she was alive, without recurrent or metastatic tumor.
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Mammary Paget disease (PD) affecting 48-year-old woman. Patient had experienced prolonged history of chronic eczematous dermatitis of nipple and areolar area for several years. Lesion did not respond to topical treatment, and it progressively distorted nipple with expansion into surrounding skin. Note markedly scaly, crusted, and deformed nipple with thickened, irregularly outlined adjacent nipple-areola complex. Excisional biopsy confirmed diagnosis of mammary PD. Patient developed infiltrating ductal carcinoma of underlying breast tissue with axillary lymph metastasis. She was treated by mastectomy and radiation. No metastatic tumor was noted in axillary lymph node. Patient was alive and well 3 years after treatment.
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Nipple invagination, deformed nipple-areola complex, marked erythema, and alternating hyperpigmentation and hypopigmentation noted in adjacent skin of breast in 65-year-old woman with biopsy-proven Paget disease. Note focal scaling of previous biopsy site. Nipple changes were associated with intraductal carcinoma of breast. Patient was treated by conservative excision of lesion and lumpectomy for in situ carcinoma. No recurrence or metastatic disease was noted at 6-year follow-up.
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Photomicrograph of malignant melanoma in situ of skin displays prominent intraepidermal pagetoid spread. Note that melanoma cells are present in all layers of epidermis, mostly in single units. Cytoplasm of melanoma cells is vacuolated. Moderate upper dermal chronic inflammatory infiltrate is present (hematoxylin-eosin, original magnification ×250). S-100 protein and homatropine methylbromide immunostains are positive in melanoma cells, whereas carcinoembryonic antigen is negative. No epithelial mucin is seen in these tumor cells.
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Photomicrograph of mammary Paget disease lesion. Note nests of malignant Paget cells predominantly involving lower layers of epidermis. Cytoplasm of tumor cells contains abundant pale-staining, granular, mucinous material. Occasional small glandular structures can be seen within malignant cell nests (hematoxylin-eosin, ×100).
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Composite photomicrograph of mammary Paget disease depicting nests, islands, and individual tumor cells in epidermis (left; hematoxylin-eosin, ×250), along with tumor cells stained positive for carcinoembryonic antigen (CEA) (right; immunostain with anti-CEA, ×250).
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Low-power view of transmission electron micrograph displaying malignant Paget cells in lower layer of epidermis. Note large Paget cell containing ovoid nucleus (N), scanty nuclear chromatin, large nucleolus, and abundant pale-staining cytoplasm with smooth and rough endoplasmic reticulum (arrow), scattered enlarged mitochondria, free ribosomes, and lysosomes. No desmosomal attachments are seen between Paget cells and adjacent keratinocytes. Tonofilaments are seen in keratinocytes (uranyl acetate and lead citrate, ×5,500).
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Cytoplasm of malignant Paget cell is packed with numerous rounded, membrane-bound mucin granules with various electron densities (uranyl acetate and lead citrate, ×12,000.)
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Photomicrograph of intraductal carcinoma of breast underneath Paget disease of nipple in 56-year-old woman. Note expansion of ductal lumen, which is filled with irregularly sized tumor cells of ductal epithelial origin. Nuclear hyperchromatism and gland-in-gland (cribriform) pattern are evident. Tumor was detected by positive mammogram result depicting focus of microcalcification beneath nipple.
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