Case description
A 34-year-old-man admitted to the local hospital with the complaints of headache and marked hypertension. Laboratory investigation revealed elevated levels of norepinephrine and dopamine in the plasma. Computed tomography (CT) revealed a 4×8cm tumor of the urinary bladder, while there was no evidence of metastatic disease at this stage. Pathological diagnosis of paraganglioma was obtained by surgery.
At the age of 43 years, laboratory workup showed elevated plasma dopamine and F18-FDG-PET demonstrated a recurrence of paraganglioma in the clavicle, thoracic vertebrae and rib. Uptake of 123I-MIBG was not detected in the tumors. After external radiation therapy to these sites and 4 cycles of combination chemotherapy with cyclophosphamide, vincristine and dacarbazine, the patient was followed carefully without any additional treatment. Plasma level of dopamine increased gradually and bone metastasis showed a slow progression on imaging. FDG-PET revealed a metastatic lung tumor, pleural dissemination and cervical lymph node metastasis between 46-47 years of age.
At the age of 49 years, FDG-PET demonstrated gradual growth of existing metastatic lesions, and new uptake at esophagus. Esophagogastroduodenoscopy (EGD) showed a 6mm mass at the middle intra-thoracic esophagus and biopsy was done. Hematoxylin and eosin staining of the biopsy specimen demonstrated the classic Zellballen pattern of paraganglioma with partial staining of synaptophysin and chromogranin A, suggesting metastasis of paraganglioma.
Conclusion
Little has been reported about metastasis to esophagus of paraganglioma. Differential diagnosis between esophageal carcinoid and metastasis of paraganglioma is difficult pathologically. We will report a case of paraganglioma which arised from urinary bladder initially, and was suspected to metastasize to esophagus.