Langerhans cell sarcoma of the skin in association with superficial atypical Langerhans cell proliferation

Langerhans cell sarcoma of the skin is a rare tumor with aggressive behavior. There are reports of Langerhans cell sarcoma involving the skin in patients with underlying systemic Langerhans cell histiocytosis. However, to our knowledge, sarcomatous transformation of skin Langerhans cell histiocytosis, has not been previously described. We report a case of Langerhans cell sarcoma of the skin representing a probable transformation from Langerhans cell histiocytosis. The lesion had a clinical and pathological biphasic pattern, presenting as a plaque and nodule in the lower leg of a 66 year-old female. The plaque area showed a superficial epidermotropic proliferation of atypical Langerhans cells. The associated deep nodule had an overtly sarcomatous Langerhans cell proliferation. The immunohistochemistry profile was characteristic of Langerhans cells in both components. We consider this case a unique example of deep nodular Langerhans cell sarcoma of the skin, in which the associated superficial Langerhans cell proliferation is consistent with the precursor lesion.