title: Pheochromocytoma associated with neurofibromatosis type 1: concepts and current trends.
abstract: BACKGROUND
  Neurofibromatosis Type 1(NF-1) has autosomal dominant inheritance with complete penetrance, variable expression and a high rate of new mutation. Pheochromocytoma occurs in 0.1%-5.7% of patients with NF-1.
  
  
  METHODS
  We present the case of a 37-year-old patient with laparoscopically resected pheochromocytoma. He was investigated for hypertension, flushing and ectopic heart beat. Abdominal CT and MRI revealed a mass measuring 8 x 4 cm in the right adrenal gland. The diagnosis of pheochromocytoma was confirmed by elevated 24-hour urine levels of VMA, metanephrines and catecholamines as well as positive MIBG scan. The patient presented with classic clinical features of NF-1, which was confirmed by pathologic evaluation of an excised skin nodule. The patient underwent laparoscopic right adrenalectomy through a transabdominal approach and was discharged on the second postoperative day, being normotensive. The patient is normotensive without antihypertensive therapy 11 years after the procedure.
  
  
  CONCLUSIONS
  Nowadays in the era of laparoscopy, patients with pheochromocytoma reach the operating theatre easier than in the past. Despite, the feasibility and oncological efficacy of the laparoscopic approach to the adrenals, continued long term follow-up is needed to establish the minimally invasive technique as the preferred approach. Furthermore, these patients should be further investigated for other neoplasias and stigmata of other neurocutaneous syndromes, taking into account the association of the familial pheochromo-cytoma with other familial basis inherited diseases.
authors.affiliation: Third Department of Surgery, G, Gennimatas Hospital, Athens, Greece. gnzografos@yahoo.com
authors.forename: George N
authors.initials: GN
authors.lastname: Zografos
authors.forename: George K
authors.initials: GK
authors.lastname: Vasiliadis
authors.forename: Flora
authors.initials: F
authors.lastname: Zagouri
authors.forename: Chrysanthi
authors.initials: C
authors.lastname: Aggeli
authors.forename: Dimitris
authors.initials: D
authors.lastname: Korkolis
authors.forename: Sophia
authors.initials: S
authors.lastname: Vogiaki
authors.forename: Matina K
authors.initials: MK
authors.lastname: Pagoni
authors.forename: Gregory
authors.initials: G
authors.lastname: Kaltsas
authors.forename: George
authors.initials: G
authors.lastname: Piaditis
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000473:pathology* / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D006801:Humans; D010535:Laparoscopy; D008279:Magnetic Resonance Imaging; D008297:Male; D009456:Neurofibromatosis 1 / Q000473:pathology* / Q000601:surgery; D010673:Pheochromocytoma / Q000473:pathology* / Q000601:surgery; D014057:Tomography, X-Ray Computed