Poster Presentation Society of Obstetric Medicine of Australia and New Zealand ASM 2026

Rare ‘Fraternal Twins’ - Phaeochromocytoma and Neurofibromatosis in Pregnancy (#153)

Sze Khim Hiew 1 , Elvina Ingrid 1 2 , Bill Fleming 3 , Kathy Paizis 2 , Joanne Said 4 , Priya Rao 5 , Nicole Sheridan 5 , Dev Kevat 6
  1. General Internal Medicine Department, Sunshine Hospital, Western Health, St. Albans, VIC, Australia
  2. Obstetric Medicine Department, Joan Kirner Women's and Children's Hospital, Western Health, St. Albans, VIC, Australia
  3. General and Endocrine Surgery, Footscray Hospital, Western Health, Footscray, VIC, Australia
  4. Maternal Fetal Medicine Department, Joan Kirner Women's and Children's Hospital, Western Health, St. Albans, VIC, Australia
  5. Anaesthetics Department, Sunshine Hospital, Western Health, St. Albans, VIC, Australia
  6. Endocrinology Department, Sunshine Hospital, Western Health, St. Albans, VIC, Australia

Introduction/Aim:

Hypertension in pregnancy affects 5-10% of pregnancies [1] and is most commonly caused by pregnancy-induced hypertensive disorders, including pre-eclampsia, or primary hypertension. However, secondary causes should also be considered, particularly in young patients with severe or atypical presentations, or, at early gestation at onset, as management differs significantly. This case report aims to provide clinical and investigation findings and highlight a management strategy for these rare conditions.

Method:

Review of clinical presentation and laboratory investigations.

Results:

This case report presents a 19-year-old primigravida woman who was referred at 11 weeks’ gestation with two months of headaches, fatigue and palpitations.  Notable examination features included severe hypertension, multiple café-au-lait spots and bilateral axillary freckling. The diagnostic process was advanced through metanephrine assay testing with results which were grossly elevated. Magnetic resonance imaging demonstrated a 60mm right sided adrenal lesion and nerve sheath tumours.  Hypertension was initially medically optimised with an alpha-adrenergic blocker prior to successful laparoscopic transperitoneal resection in the second trimester.  Blood pressure and metanephrine levels normalised post-operatively.  Histopathology confirmed the diagnosis of phaeochromocytoma and clinical diagnosis of neurofibromatosis Type 1 (NF1) was made. She underwent emergency caesarean section at 27 weeks 3 days’ gestation due to foetal distress, delivering a male infant weighing 715 gram.

Conclusion:

This case highlights the importance of considering secondary causes of hypertension in pregnancy and outlines the multidisciplinary approach to antenatal management of phaeochromocytoma.

  1. Hutcheon, J.A., S. Lisonkova, and K.S. Joseph, Epidemiology of pre-eclampsia and the other hypertensive disorders of pregnancy. Best Pract Res Clin Obstet Gynaecol, 2011. 25(4): p. 391-403.