Mammary Paget’s Disease: An Update

Abstract

Mammary Paget’s Disease is a non-invasive cutaneous malignancy of the breast involving the nipple-areolar complex that is commonly mistaken for benign breast conditions, leading to delay in diagnosis. This review article discusses Paget’s disease etiology, clinical presentation, differential diagnosis, diagnostic work-up, natural history and prognosis. This article also discusses evolving strategies for the surgical and non-surgical management of Paget’s disease.

Paget’s Disease of the Breast (PDB) – A Review

“…Two theories have been propounded to explain the possible pathogenesis of Paget's disease as following [6]:…”

“…The timely diagnosis of PDB requires awareness about the fact that if after four to six weeks course of topical corticosteroids, an apparently benign skin lesion does not show any signs of complete resolution or shows only marginal improvement , then a high-quality diagnostic breast imaging work-up and biopsy should be performed [6].…”

“…Whether the associated malignancy is identified by imaging studies or not, patients presenting with a persistent NAC rash should raise suspicion of PDB and must undergo a full-thickness biopsy of the nipple or areolar using a 2-4 mm diameter punch biopsy tool or else a wedge-shaped full-thickness incisional biopsy [6]. There are reports in literature wherein diagnosis have been achieved by quick and non-invasive means like scrape smear cytology test [25][26].…”

“…Superior aesthetic outcomes can be attained with oncoplastic surgical techniques (e.g., Grisotti mastopexy, Wise-Pattern mammaplasty) combined with a contralateral mammaplasty or mastopexy, to maintain breast symmetry. Nippleareolar reconstruction may be undertaken immediately or with a delay and dermatography (medical tattooing) may be performed to create a symmetrical, colourmatched NAC [6] .…”

“…Without any active treatment, PDB and the underlying breast lesions are likely to progress to invasive breast cancer, followed by lymph node and visceral metastasis [6]. The prognosis of PDB is dependent upon the initial clinical presentation and the presence or otherwise, of an underlying invasive ductal carcinoma or axillary node metastases.…”