Clinical Analysis Of Hereditary Pheochromocytoma

Objective Analyze the clinical features and treatment of hereditarypheochromocytoma, to improve the understanding of hereditary pheochromocytoma.Methods12cases of hereditary pheochromocytoma in Department of Urology ofFujian Province Hospital from2005January to2014February were analyzedretrospectively, to study the classification of hereditary pheochromocytoma, clinicalcharacteristics, diagnosis, treatment.Results12cases of hereditary pheochromocytoma are from27-60year old,average38.6years old. There were6males (50%),6females (50%). Including7cases of multiple endocrine neoplasia type2A (MEN-2A),2cases of MEN-2B,2cases of VHL disease,1case of neurofibromatosis type1(NF-1). In MEN-2A patients,5cases showed pheochromocytoma (PHEO) and thyroid carcinoma,2cases showedPHEO+thyroid cancer+hyperparathyroidism (HPT).2cases of MEN-2B showedPHEO+thyroid cancer and oral, lip mucosa lesions. In2cases of VHL disease，onecase showed PHEO+renal cell carcinoma, the other one showed PHEO+cerebellartumor.1case of NF-1patients showed PHEO+whole body skin white coffee spot anddermatofibroma. In12cases，9cases showed bilateral PHEO (75%),3cases showedunilateral (25%), the largest tumor diameter from2.8-7.0cm, average4.39cm.5caseshave family history (41.6%),7cases were no family history or family history isunknown (58.3%). In12cases,3cases (25%) showed persistent hypertension,5cases(41.6%) showed paroxysmal hypertension,2cases showed persistent hypertensionparoxysmal episodes (16.7%),2cases without hypertension (16.7%).7of themreceived laparoscopic operation,5of them underwent open operation to excise PHEO,7cases (58.3%) received postoperative follow-up,1case (14.3%) appeared tumorrecurrence.Conclusion Hereditary pheochromocytoma usually showed bilateral adrenal PHEO, and a variety of clinical manifestations, the clinical manifestation can beparoxysmal hypertension also showed persistent hypertension, biochemical andimaging examination is helpful to the diagnosis, operation to excise PHEO in treatinghereditary pheochromocytoma showed positive effect, but there is some recurrencerate，we shoud make a close follow-up after surgery.