Management of pheochromocytoma during pregnancy from diagnosis to laparoscopic adrenalectomy. A case report and review of literature

INTRODUCTION: Pheochromocytoma is an endocrine tumour of chromaffin cells. It can be diagnosed either sporadically or in the context of hereditary syndromes (e.g. Von Hippel Lindau, Neurofibromatosis type 1 and multiple neuroendocrine neoplasia type 2). During pregnancy, its frequency is very low (about 0,007%). This tumour causes paroxysmal hypertension in 0,1-0,6% pregnant women, because of an overproduction of catecholamines. If undiagnosed and nontreated, it's associated with high maternal and fetal mortality (40-50%). We report the case of a 30-year-old female diagnosed with pheochromocytoma during pregnancy at week 31 of gestation. In a multidisciplinary team made of surgeons, gynaecologists, anaesthetists, geneticists and endocrinologists we evaluated the case and according to literature, we choose a surgical approach after childbirth: performing a laparoscopic right adrenalectomy. DISCUSSION: In pregnancy, pheochromocytoma is a rare clinical condition. Gold standard treatment is laparoscopic adrenalectomy. However, the optimum timing of surgery is a challenge. CONCLUSION: Timely diagnosis of pheochromocytoma in pregnant women with hypertension and appropriate therapeutic management can lead to improve maternal, fetal and neonatal outcomes. The multidisciplinary team is necessary to recognize the symptoms and to adopt the right pre - and post - operative treatment. Laparoscopic adrenalectomy after delivery is safe and feasible even though the surgical procedure should be performed by an experienced surgeon. KEY WORDS: Pheochromocytoma, Pregnancy, Laparoscopic adrenalectomy and pregnancy, Management of pheochromocytoma, Laparoscopic adrenalectomy, Adrenalectomy, Pregnancy and pheochromocytoma.