[Cancerous Peutz-Jeghers syndrome with disseminated metastases]

This report of a patient with Peutz-Jeghers syndrome is unusual in a number of ways: the patient, a 49-year-old woman, was first seen with deep thrombophlebitis of the leg which appeared to be part of a paraneoplastic syndrome. The patient had an adenocarcinoma originating in a hamartoma of the terminal ileum, with ovarian and lymph-node metastases. During the course of the disease the patient was found to have metastatic disease in the cervical lymph nodes which responded, temporarily, to chemotherapy. The authors review the literature on metastatic Peutz-Jeghers syndrome. Although the rate of metastatic disease is low, they believe that all patients with Peutz-Jeghers syndrome deserve a close long-term follow-up.