Many patients have systemic disorders, predominantly MEN2B, followed by Cowden syndrome and NF1, presenting thyroid and adrenal tumors, trichilemmomas and “cobblestone” tongue lesions, or cutaneous cafe’-au-lait spots and neurofibromas (4–6, 16, 17, 21–24). The gene discussed is RET; the disease is plexiform neurofibroma.