Clival chordoma in an infant.

AA Goel, TD Nadkarni, MV Kirtane 
 Department of Neurosurgery and Otolaryngology, KEM Hospital and Seth GS Medical College, Parel, Mumbai.

Correspondence Address:
A A Goel
Department of Neurosurgery and Otolaryngology, KEM Hospital and Seth GS Medical College, Parel, Mumbai.

Full Text

::   Introduction

Chordomas are congenital tumours believed to originate from notochordal cell rests. More often these tumours present in the 3rd to 4th decade of life Occurrence of these tumours in childhood is rare[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15]. We encountered a 7 month, 16 day old child with a large clival chordoma, which was symptomatic since the age of 2 months. This is the youngest age on record for such a tumour to be symptomatic.

::   Case report

This boy developed noisy breathing, snoring and occasional apnoea with cyanosis when placed in a horizontal position since 2 months of age. In an upright position, he was asymptomatic but preferred a hyper extended neck posture. The frequency of apnoeic spells increased to three or four times a day as the child grew. When he was 7 month old, in a period of 10 days he developed three episodes of cyanosis followed by 28 hour period of unconsciousness and needed ventilatory assistance, arterial blood oxygen being grossly reduced at each time. Neurological examination was normal. Magnetic resonance imaging (MRI) showed a large retropharyngeal and clival mass [Figure:1].

Considering the age and strict midline location of the tumour a relatively simple transoral surgical approach (by MVK) was considered to be safer in this patient. Radical debulking of the jellylike soft and relatively avascular tumour was done. Histology revealed the classical features of chordoma [Figure:2] with tissue composed of lobules of large clear physaliphora cells with small central or peripheral nuclei. The child had no further episode of apnoeic spell and breathing was comfortable and noiseless and the neck posture returned to normal. Due to the patient's age, the tumour was not subjected to radiation treatment. The patient has not reported for further followup.

::   Discussion

Cranial chordomas are rare, histologically usually benign and slow growing tumour[16],[17],[18]. They present more often in the 3rd to 5th decade of life. Due to the characteristic site and pattern of growth, the tumours are usually large in size when first diagnosed and have relatively innocuous presenting symptoms, The location of these tumours in the depths and in proximity to vital neural and vascular structures makes surgical removal difficult and the ultimate clinical course malignant. Unlike the adult type, the intracranial chordomas in children have been seen to run an acute clinical course and show frequent metastasis, more often in the lungs[4],[8],[10],[12],[14],[15],[19]. In general, for chordomas a 5 year survival rate has been reported to be between 3050%[20],[21]. However, 8 of the 11 cases diagnosed in children less than 5 years of age died within 2 years from diagnosis[2],[3],[4],[5],[8],[10],[11],[14],[15],[19].

The youngest case of chordoma reported in the literature was in a 6-month-old child who presented with acute symptoms of obstructive hydrocephalus [15]. In our case, the tumour was symptomatic since the child was 2 month old. Most of the chordomas seen in Paediatric age group were in boys. The reason for the extreme male preponderance in young age group is unclear[7]. The common presenting symptoms include headache, progressive cranial nerve palsies[7],[8] widening of cranial sutures and enlarging head size[3],[15]. A typical presentation with the sole initial symptom of obstruction to respiratory passage due to the tumour bulk as, was seen in our case has not been recorded.

Radiological examination of patients with cranial chordoma often reveals a midline soft tissue mass around the clivus associated with osteolytic bone destruction and intralesional calcifications. MRI not only delineates the exact site and the extensions of the tumour, but also clearly defines the neural and vascular relationship essential for planning of the surgical strategy. The radiological picture of chordoma in the Paediatric age group simulates that in adults.

Although, good results with radical surgical excision by lateral infratemporal and transtemporal routes has been reported for adults[22],[23] feasibility and tolerance of such a treatment in Paediatric age group is yet to be seen. Anterior transoral and transpalatal routes although only debulking operations in most cases[24], appear to be short and safe alternative in selected infants. Immunological studies were not possible in the present case due to nonavailability of suitable antigens.

The role of radiotherapy for chordomas is not established, and its use in Paediatric age group can be debated. There are reports of successful outcome following this modality of treatment[22],[24]. Combination of a ‘radical debulking’ operation followed by high dose radiation therapy appears to be an effective mode of treatment in older children. Survival for 411 years without recurrence is recorded in children under 10 years of age who received such a combination of treatment [3],[5]. Chemotherapy appears to be of no value[6].

::   Acknowledgments

The authors acknowledge with gratitude the encouragement and support of Professor SK Pandya.

References