Ganglioneuroblastoma is a variant of neuroblastoma tumours with mature ganglion cell differentiation which occurs commonly in cervical, mediastinal and retroperitoneal locations. Approximately 90% of ganglioneuroblastomas are seen in children younger than five years old. There are 50 adult cases of ganglioneuroblastomas reported to date. Our patient is the 51st case and she is the first to be diagnosed in pregnancy. Our patient's tumour site was the thoraco-abdominal retroperitoneal space adjacent to her kidney. This tumour is generally diagnosed incidentally or by compression presentations, i.e. pain or neurological symptoms. Our patient's ultrasound scan appearances triggered suspicion of an atypical mass after presenting with loin pain. Caesarean section with tumour removal in the same session is preferred if tumour is small and localized. However in this case, there was uncertainty regarding the extent of the tumour. Therefore, the patient underwent thoracotomy after delivery to excise the thoraco-abdominal tumour, which extended from the level of the T9 to L2 vertebrae.
Introduction Phaeochromocytoma, a catecholamine-producing endocrine tumour, is a life-threatening condition to the mother and fetus. The incidence of phaeochromocytoma in pregnancy is extremely rare, 1 in 54 000 pregnancies1. If it remains undiagnosed and untreated, maternal and fetal mortality amounts to 40–50%2 3. Classically, physicians search for the tumour in hypertensive patients with paroxysmal symptoms such as headache, sweating or palpitations. However, our patient presented atypically and would have been left undiagnosed. Case presentation A 21-year-old presented after a fall at 24 weeks gestation, with loin pain on the contralateral side to the fall. This prompted an ultrasound scan that demonstrated a 9 cm ‘haematoma’ above the right kidney. As she was claustrophobic, she declined magnetic resonance imaging (MRI). A repeat ultrasound was performed which showed the mass to be unchanged. Suspicion arose and an MRI under sedation was performed (as recommended to look for adrenal/renal mass in pregnancy4). This confirmed an 8 cm × 7 cm mass superior to the right kidney. An endocrinologist reviewed her and serum and urine biochemistry investigations were performed. Her urinary 3-methoxytyramine and serum dopamine levels were raised5 6 indicating possible phaechromocytoma. She did not need α-adrenoceptor blockade, as she remained asymptomatic throughout her pregnancy. She was referred to a tertiary hospital where an elective Caesarean section and surgical tumour removal were performed simultaneously. Conclusion Diagnosing phaeochromocytoma in an asymptomatic pregnant patient is challenging. However, when diagnosed, a multi-disciplinary team approach (obstetrician, surgeon and endocrinologist) is vital in the management of this rare disorder. References Lenders JWM. Phaeochromocytoma and pregnancy: a deceptive connexion. European Journal of Endocrinology 2012;161:143–150. Schenker JG & Chowers I. Phaeochromocytoma and pregnancy. Review of 89 cases. Obstetrical and Gynecological Survey 1971;26:739–747. Dean RE. Phaeochromocytoma and pregnancy. Obstetrics and Gynecology 1958;11: 35–42. Lenders JW, Eisenhofer G, Mannelli M, et al. Phaeochromocytoma. Lancet 2005;366:665–675. Peleg D, Munsick RA, Diker D, Goldman JA & Ben-Jonathan N. Distribution of catecholamines between fetal and maternal compartments during human pregnancy with emphasis on L-dopa and dopamine. Journal of Clinical Endocrinology and Metabolism 1986;62:911–914. Lenders JW, Pacak K, Walther MM, Linehan WM, Mannelli M, Friberg P, Keiser HR, Goldstein DS & Eisenhofer G. Biochemical diagnosis of phaeochromocytoma: which test is best? Journal of the American Medical Association 2002;287: 1427–1434.
Background As part of the ‘Jimma Exchange Scheme’, staff from Nottingham University Hospitals NHS Trust travelled to Jimma, Ethiopia to observe working practices in the Obstetrics department of the University Hospital in November 2009. Objectives To provide skills training for residents, midwives and medical students and to audit the unit's obstetric outcomes for the preceding 6 months. Methods Skills training was performed with small groups of participants of mixed skill levels, focusing on emergencies including neonatal resuscitation, breech delivery, postpartum haemorrhage and shoulder dystocia. Audit was performed by retrospective data collection from the theatre records and maternity unit discharge book with difficulty due to large amounts of missing data. Audit results 1019 women delivered 1027 babies over 6 months. 14 maternal deaths occurred (1374 per 100 000 maternities). 117 fetal deaths (11.4% of births). 68 peripartum hysterectomies (6.7% of women). 390 caesarean sections (38%). Birthweight <2.5 kg 7%. Birthweight >4.0 kg 2.2%. Recommendations Development of guidelines to improve practice. Change in working practices to allow greater supervision of trainees and involvement of midwifery staff, allowing better interdisciplinary working. Improvements in documentation of outcomes, facilitating further audit, establishing whether changes have improved outcomes. Conclusions The Nottingham visitors had a wonderful and productive visit. They received a great welcome and generous hospitality. There were many positives in the visit and at the top of that list were the enthusiasm, knowledge and clinical skill of the junior doctors. We have made suggestions for future collaboration and improvement in care for pregnant women and their babies.
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