Diagnostic delays and nuanced surgical management in perianal Paget’s disease: a case report and review of current literature

TL;DR

This case report discusses the challenges in diagnosing and surgically managing perianal Paget’s disease, emphasizing the need for early recognition and a multidisciplinary approach.

Contribution

The paper presents a real-world case highlighting diagnostic delays and surgical complexities in perianal Paget’s disease.

Findings

The patient experienced a 2-year delay in diagnosis due to misinterpretation as an inflammatory rash.

Surgical excision resulted in positive margins, prompting a multidisciplinary discussion on further treatment options.

Early recognition and a team-based approach are critical for managing this rare and challenging disease.

Abstract

Perianal Paget’s disease is an uncommon and difficult to treat disease due to the high risk of positive margin at resection and high risk for recurrence. It can arise as a primary tumor or secondary to underlying pelvic visceral malignancy and is often initially misdiagnosed as an inflammatory perianal rash until proper pathologic diagnosis has been confirmed. We present the case of a 76-year-old woman who presented with 2 years of a perianal rash unsuccessfully treated with various topical interventions. Eventually, a biopsy was performed revealing extramammary Paget’s disease and she underwent surgical excision of the perianal lesion. Operative pathology unfortunately exhibited positive margins and we detail the multidisciplinary discussion of further management options in this complicated clinical dilemma. This case highlights the importance of early recognition of this disease and a multidisciplinary approach to its treatment.

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