Session: MON 292-305-MEN1, MEN2 & Pheochromocytomas
Poster Board MON-303
Ganglioneuroblastomas are the tumors of sympathetic nervous system. The presence of immature tissue in these tumors indicates potentially malignant behaviour. They are most often seen in the early childhood, especially less than 7 years of age and are extremely rare in adults, with only about 50 cases documented in people over the age of 20 years. Because of this, the clinical, radiographic and pathological behaviour is not clearly understood in adults.
To our knowledge, there are no reports of ganglioneuroblastoma presenting during pregnancy.
A 22 year-old lady around 31 weeks gestation, presented with 24 hour history of right flank pain. USG revealed possible 9cm right adrenal mass with haemorrhagic changes. She was fit and well. She did not have symptoms of headache, palpitation, excessive sweating or dizziness. Her BP was 120/80 mm of Hg.
Endocrine investigation revealed normal serum aldosterone : renin ratio, normal urinary cortisol excretion. Urinary normetadrenaline was 3786 nmol / 24 hrs (ref 0-3000) however urinary dopamine was markedly raised at 14,929 & 22,746 nmol / 24 hrs (ref – 0-2700), and urine – 3 -Methoxytyramine 11,365 nmol /24 hrs (ref 0-2300).
MRI (non-contrast) revealed 8.1 X 7.3 cm right suprarenal /adrenal heterogenous mass, exact nature and extent of the tumor could not be commented with certainty.
Clinically patient was thought to have dopamine secreting paraganglioma /pheochromocytoma, therefore small dose of phenoxybenzamine was started.
In view of her pregnant status, detailed CT scan / MRI scan with contrast / PET scan was not performed. Both mother and foetus were closely monitored and medical therapy continued. Patient underwent elective caesarean section at 36 weeks gestation and delivered healthy baby.
4 weeks postpartum, detailed CT scan confirmed presence of 17 x10 x 8 cm mass lying within retroperitoneum & right chest extending from vertebrae level of T9 to L2. PET scan features were in keeping with a paraganglioma. Patient underwent thoracotomy and excision of thoraco-abdominal tumor. She made uneventful recovery & histology confirmed ganglioneuroblastoma.
The rarity of ganglioneuroblastoma, along with its occurrence mainly in the paediatric population, warrants a great deal of suspicion in an adult patient with adrenal mass.
There is virtually no evidence available related to safe management of these patients during pregnancy as well as safety of vaginal delivery in this setting.
Therefore we feel it is best practice to manage medically in multidisciplinary setting until the foetus is viable using the same drug regimen as for regular surgical preparation of pheochromocytoma. Caesarean section with tumor removal in the same session is preferred if tumor is small and localised. However if there is uncertainty regarding extent of tumor, as in our case, surgical removal during postpartum period should be considered.
Nothing to Disclose: MSR
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