Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is the second most common cutaneous T-cell lymphoma after mycosis fungoides. It is rare in children and typically manifests as solitary or localized reddish-brown nodules or tumors. Histologically, it is characterized by a diffuse dermal infiltrate of medium-to-large atypical anaplastic cells. Despite the frequently alarming clinical and histologic presentation, the disease pursues an indolent course with a favorable prognosis. Herein, we report a case of C-ALCL in an 8-year-old boy presenting with multiple ulcers on the extremities, with the largest measuring 4 cm × 4 cm. Histopathological examination revealed an angiocentric and angiodestructive infiltrate composed of large atypical lymphoid cells. To our knowledge, this distinctive histopathological pattern has not previously been reported in pediatric C-ALCL and represents an important diagnostic pitfall because of its close resemblance to type E lymphomatoid papulosis and extranodal NK/T-cell lymphoma. The boy underwent surgical excision with no recurrence observed during the 10-month follow-up.
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