here’s a breakdown of the provided text, focusing on the key facts about Paget’s disease of the breast:
What is Paget’s Disease of the Breast?
it’s a rare form of breast cancer.
It originates in the nipple and areola.
It’s characterized by eczematous lesions on the nipple.
How is it Detected?
Palpation: A mass is felt in 80% of cases.
Mammography: In 20% of cases,no mass is palpable,but mammography may show anomalies.
Clinical Suspicion: It should be suspected in patients with unilateral, erythematous nipple lesions that don’t respond to topical corticosteroids.
Diagnostic Tools:
Biopsy: This is the definitive diagnostic tool. It will show the presence of Paget cells in the epidermis.
Dermatoscopy: Can be helpful by showing irregular vessels,blue-gray dots/globules,bright white structures,and pink/brown areas without structure. Though, it’s not highly specific.
Immunohistochemistry: Useful for confirmation and includes markers like CK7 (+diffuse), polyclonal CEA (+), p63 (-), HER2 (+), EMA (+), SOX10 (-), Melan-A (-), S100 (-).
differential Diagnosis:
It’s crucial to differentiate Paget’s disease from benign conditions and other malignant lesions. Benign: Eczema, allergic contact dermatitis, radiodermatitis, erosive adenomatosis of the nipple, Toker cell hyperplasia.
Malignant: Bowen’s disease, basal cell carcinoma, malignant melanoma.
Key Point for Diagnosis: If lesions don’t improve after 3 weeks of topical corticosteroids, a biopsy should be considered.
Tumor staging:
paget’s disease itself doesn’t change the stage of the underlying neoplasia.
If no invasive cancer or ductal carcinoma in situ (DCIS) is found,Paget’s disease is classified as pTis (Paget).
Treatment:
The dermatologist’s role is primarily in diagnosis.
Surgery is the treatment of choice.
Mastectomy or conservative surgery followed by radiotherapy are the most accepted treatments.
Hormonal treatment (tamoxifen, aromatase inhibitors) and other adjuvant systemic treatments are determined by the underlying neoplasia.
The prognosis depends on the underlying neoplasia.
Extramammary cutaneous Paget Disease:
This is a rarer form that affects areas with apocrine glands (vulva, perineum, scrotum, perianal zone). It’s considered a primary cutaneous adenocarcinoma.Case Example:
The text describes a patient who had a biopsy confirming Paget’s disease. She was referred to gynecology, where an underlying neoplasm was found. She underwent tumorectomy, radiotherapy, and anastrozole treatment, and is now doing well.
the text emphasizes the importance of clinical suspicion, biopsy for definitive diagnosis, and the multidisciplinary approach to managing Paget’s disease of the breast, as it is indeed frequently enough associated with an underlying breast malignancy.
