Anaplastic T-Cell Non-Hodgkin’s Lymphoma: Mucocutaneous Presentation

A 41-year-old male presented with a nodular, vegetating and ulcerated lesion of the upper lip, with two weeks of evolution and rapid growth. Biopsy revealed Primary Cutaneous Anaplastic T-Cell Non-Hodgkin's Lymphoma (pc-ALCL). ALCL represents mature T-cell neoplasms that express the lymphocyte activation marker CD30. This can be grouped based on the expression of the ALK marker (positive or negative) and by clinical presentation (systemic or localized). Localized forms of ALCL include primary cutaneous (pc) ALCL and breast implant-associated ALCL (BIA-ALCL). c-ALCL makes up 8% of cutaneous T-cell lymphoma; it is seen mostly in male adults. Clinically, pc-ALCL presents with nodular cutaneous lesions; the majority of the patients present with solitary or localised nodules or papules. Patients with pc-ALCL have an excellent prognosis, with a 5-year disease-specific survival  over 90%. [...]