Primary desmoplastic small round cell tumor of upper cervical lymph nodes

Desmoplastic small round cell tumors (DSRCT) are rare malignancies that typically arise in the abdominopelvic cavities. They are very uncommon in the head and neck region. We present a case of an 11-year-old Caucasian male with a primary cervical lymph node tumor in the neck. Fine-needle aspiration cytology, histopathologic examination, immunohistochemical staining, and molecular genetic testing led to the diagnosis of DSRCT. Due to the very limited number of cases reported and the lack of staging criteria, the preferred management approach remains uncertain.