Case of lymph node primary perivascular epithelioid cell tumor associated with leukoderma

Dear Editor, Perivascular epithelioid cell tumor, also known as PEComa, is a type of mesenchymal neoplasm. Here, we encountered an interesting case of primary PEComa in a cervical lymph node (LN) that was associated with leukoderma. A 63-year-old Japanese man was referred to us with a 3month history of a swollen LN in the left neck. The patient was a white collar worker belonging to an advertising agency unassociated with chemical agents. The patient had not used a cosmetic containing rhododendrol. Physical examination, including otological examination, revealed a swollen, movable, non-tender LN in the left neck, and several white macules measuring less than 40 mm in diameter on the forehead and neck (Fig. 1a). The areas of leukoderma had been increasing in number for 30 years. The patient had not been diagnosed with disease of the thyroid grand or anemia. Computed tomography detected a swollen LN measuring 16 mm 9 13 mm (Fig. 1b), but no other lesions. Histopathological examination of the resected swollen LN revealed that tumor cells occupied the majority of the LN (Fig. 1c). The tumor consisted of epithelioid