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Congenital dumbbell neuroblastoma mimicking birth trauma
  1. F Nejat1,
  2. S Zabihyan Sigarchi2,
  3. M IzadYar3
  1. 1Department of Neurosurgery, Children’s Hospital Medical Center, Tehran University of Medical Science, Tehran, Iran
  2. 2Department of Neurosurgery, Mashad University of Medical Science, Mashad, Iran
  3. 3Children’s Hospital Medical Center, Tehran University of Medical Science, Tehran, Iran
  1. Correspondence to:
 Dr F Nejat
 No 20, West 194 (Alaii Saqarloo) Street, 3rd square of Tehran pars, Tehran, Iran; nejatsina.tums.ac.ir

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Neuroblastoma is the commonest extracranial malignant tumour in children and neonates.1 It may involve the vertebral bodies or extend into the spinal canal, compressing the spinal cord, or spread into the retroperitoneal space, involving the lumbosacral plexus. Early diagnosis is important for treatment. We report two cases of congenital neuroblastoma mimicking obstetric related palsies.

Case 1

A 4 month old baby boy with a diagnosis of unilateral leg palsy due to birth trauma, despite normal vaginal delivery, was admitted because of a palpable abdominal mass. The infant’s left leg lacked spontaneous movement, was flaccid, and deep tendon reflexes were absent. He had poor rectal tone and dribbling of urine. The levels of urinary catecholamine derivatives were increased. Spinal magnetic resonance imaging (MRI) demonstrated a large retroperitoneal mass with thoracolumbar cord involvement. A diagnosis of neuroblastoma was made following biopsy of the abdominal mass. Multiagent chemotherapy proved effective in reducing the size of neuroblastoma. His left leg function returned after several months’ chemotherapy. At present, after two years he is free of disease, he can stand and walk with a brace, and his neurogenic bladder is managed with clean intermittent catheterisation.

Case 2

A 10 day old baby boy was seen for evaluation of right lower limb weakness. He had not moved this leg since birth. He was born at full term via a normal vaginal delivery with vertex presentation. He had a hyperextended thigh and decreased tone in the remainder of the leg. Other limbs were normal. Abdominal examination revealed a palpable mass in the right upper quadrant just lateral to the midline. Sonography of the abdomen revealed a unilateral retroperitoneal tumour adjacent to the right kidney with spinal cord involvement. A spinal MRI showed extensive spinal cord compression from T12 to L4 (fig 1). Biopsy of the paravertebral mass revealed neuroblastoma. The neonate was treated with multiagent chemotherapy. However, he developed paresis of the left leg within two weeks of starting chemotherapy. The spinal cord was therefore surgically decompressed through an osteoplastic laminotomy and the extradural mass was fully resected. Although there was partial recovery of left leg function the right limb remained plegic.

Figure 1

 Case 2: spinal magnetic resonance imaging scan revealed severe cord compression from T12 to L4 and a large intra-abdominal retroperitoneal mass.

Discussion

Birth trauma causing brachial plexus injury is relatively common where obstetric services are limited, but lumbosacral plexopathy after a normal vaginal delivery is extremely rare. Unilateral lower extremity palsy in a neonate must lead the primary care provider to consider other diseases. The combination of neurological deficits and an abdominal mass should alert the physicians to consider neuroblastoma. Early diagnosis can improve outcome,2,3 and neuroblastoma diagnosed even in the prenatal period has been reported to have excellent prognosis.4

Although congenital dumbbell neuroblastomas are rare, spinal cord compression and involvement of the peripheral nerves or nerve plexus are not uncommon with abdominal neuroblastoma.5 The incidence of intraspinal involvement of neuroblastoma varies between 6% and 24%.6,7 Intraspinal neuroblastoma is a direct extension of a peripheral tumour.8 Dumbbell neuroblastoma is the commonest malignant cause of spinal cord or nerve root compression in young children and is regarded as an unresectable tumour.7 Chemotherapy should be considered for patients with partial deficits and surgical decompression should be reserved for children with recent onset of severe neurological dysfunction or deterioration in a 24–72 hour period.7 We treated case 1 with chemotherapy only, because his neurological deficit had been observed four months ago and was not progressive. After disappearance of the retroperitoneal and intraspinal masses with chemotherapy the deficit improved partially. The second neonate’s condition deteriorated during chemotherapy, and surgical decompression resulted in recovery of one limb although the fixed deficit from birth, in the lower right extremity, did not change.

References

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Footnotes

  • Competing interests: none declared

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