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anaplastic large cell lymphoma, CD30 lymphoproliferative disorders
Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a CD30 positive lymphoproliferative disorder, which is the second most common group of cutaneous T-cell lymphomas. It is common in adults 50-70 years of age with a male to female ratio 2:1. Here, we report a 14-year-old Caucasian female who presented with a painless, growing, friable, hemorrhagic nodule on her right medial buccal cheek of 2 weeks’ duration. The lesion was traumatized during a basketball game and significant bleeding was noted. A shave biopsy was performed on the 1.7 cm x 1.3 cm lesion. Histopathology demonstrated epidermal necrosis, acanthosis, large atypical lymphocytes with a number mitoses, prominent eosinophils, and smaller lymphocytes. Immunohistochemical staining revealed CD30 positivity of much of the tumor infiltrate, but was not diffusely positive. Rare cells were granzyme positive. ALK-1, TdT, EBER, CD1a were negative. The diagnosis was confirmed by a second dermatopathologist. The patient was referred to oncology. Labs were within normal limits and PET/CT scan was negative for metastasis. The lesion started to spontaneously regress after the shave biopsy. Spontaneous regression of C-ALCL occurs in 20-42% of cases and usually has an indolent course, therefore, aggressive treatment is not appropriate. In most cases, solitary or localized lesions are treated with surgical excision or radiotherapy. Although, about half of cases reoccur, but are not life threatening. This unusual case of C-ALCL provides an additional example of a rare clinical presentation in an adolescent female and reiterates the important histopathological findings for diagnosis.
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