Primary breast lymphoma
ICD-10 C84.7 · ICD-11 2A81.Y

Treatment of Primary Breast Lymphoma Presenting as Primary Cutaneous Anaplastic Large Cell Lymphoma (pcALCL)

This protocol addresses the rare situation in which primary breast lymphoma takes the form of primary cutaneous anaplastic large cell lymphoma (pcALCL) — an indolent, ALK-negative subtype of ALCL that most commonly arises as localised skin nodules. When pcALCL presents as a primary breast lesion (PB-pcALCL), the clinical picture is uncommon and the published experience limited to isolated case reports.

pcALCL is considered a more indolent form of ALCL, typically ALK-negative, and usually presents as single or multiple localised skin nodules. Primary breast involvement (PB-pcALCL) is rarely reported. The described presentations include isolated breast lesions, sometimes accompanied by overlying skin changes, with the diagnosis confirmed histologically.

For this early-stage, localised presentation, the treatment approach involves a surgical intervention. The complete structured regimen — including the full sequence of management steps and follow-up strategy — is available in the protocol.

Full regimen details, clinical decision points, and evidence summary are in the structured protocol below.

Instant Access to Structured Evidence-Based Regimens
References
DOI: 10.1016/j.jpra.2022.01.004
pcALCL is a more indolent form of ALCL, ALK- and most commonly presents as single or multiple localised skin nodules.
Primary breast-pcALCL (PB-pcALCL) has been reported rarely in the literature; we found one case report of a 70-year-old woman who developed a single breast lesion, accompanied by skin discolouration, who was treated successfully with surgery and was followed up with active surveillance.
Early-stage pcALCL has a relatively good prognosis with most cases treatable with surgery, and some cases have been reported in which the tumour spontaneously regresses after biopsy.
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