Anaplastic lymphoma kinase-negative primary systemic anaplastic large cell lymphoma mimicking a ruptured epidermal cyst of the scalp: a case report and literature review

The incidence of anaplastic large cell lymphoma is 0.25 cases per 100,000 people. It usually causes lymphadenopathy and B symptoms; however, diverse cutaneous manifestations can also be observed. We report a rare case of anaplastic large cell lymphoma of the scalp, which presented similarly to a ruptured epidermal cyst. A 77-year-old woman visited the outpatient clinic complaining of scalp masses that had appeared 2 months before. One week before her visit, she had undergone incision and drainage at a local clinic but showed no improvement. Before surgery, facial magnetic resonance imaging revealed two suspicious ruptured cystic masses. Surgical excision was performed with a 1-cm free margin from the soft mass. Histopathology confirmed anaplastic lymphoma kinase-negative anaplastic large cell lymphoma. After wide excision and skin grafting for wound reconstruction, followed by consultation with a hemato-oncologist and radiation oncologist, chemotherapy was planned to prevent recurrence. Differentiating anaplastic lymphoma kinase-negative anaplastic large cell lymphoma of the scalp from a ruptured epidermal cyst-like mass proved challenging. We recommend considering the possibility of anaplastic large cell lymphoma if an epidermal cyst-like mass does not respond to antibiotics or conventional dressing, as illustrated by our rare case.