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 ::  Abstract
 ::  Introduction
 ::  Case report
 ::  Discussion
 ::  Acknowledgments
 ::  References
 ::  Article Figures

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CASE REPORT
Year : 1996  |  Volume : 42  |  Issue : 2  |  Page : 51-3

Clival chordoma in an infant.


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.

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Source of Support: None, Conflict of Interest: None


PMID: 0009715301

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 :: Abstract 

An unusual case of clival chordoma seen in a 7 month 16 day old infant is presented. The literature on this subject, clinical course of such tumours and the management strategy in paediatric age group is reviewed.


Keywords: Apnea, etiology,Case Report, Chordoma, congenital,diagnosis,surgery,Cranial Fossa, Posterior, Human, Infant, Newborn, Magnetic Resonance Imaging, Male, Posture, Skull Neoplasms, congenital,diagnosis,surgery,


How to cite this article:
Goel A A, Nadkarni T D, Kirtane M V. Clival chordoma in an infant. J Postgrad Med 1996;42:51

How to cite this URL:
Goel A A, Nadkarni T D, Kirtane M V. Clival chordoma in an infant. J Postgrad Med [serial online] 1996 [cited 2019 Sep 23];42:51. Available from: http://www.jpgmonline.com/text.asp?1996/42/2/51/454





  ::   Introduction Top


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 Top


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 2-8 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 jelly-like 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 follow-up.


  ::   Discussion Top


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 30-50%[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 non-availability 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 4-11 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 Top


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

 
 :: References Top

1.   Back to cited text no. 1    
2.Adson AW, Kernohan JW, Woltman HW. Cranial and cervical chordomas. A clinical and histological study. Arch Neurol Psychiatry 1935; 33:241-261.  Back to cited text no. 2    
3.Bartel AD, Heilbronn YD. Transcervical removal of a clivus chordoma in a 2-year-old child. Reversal of quadriplegia and bullbar paralysis Acta Neurochir 1970; 23:127-133  Back to cited text no. 3    
4.Becker LE, Yates AJ, Hoffman HJ, Norman MG. Intracranial chordoma in infancy Case report. J Neurosurg 1975; 42:349-352  Back to cited text no. 4    
5.Brooks LJ, Afshani E, Hicialgo C, Fisher J. Clivus chordoma with pulmonary metastases appearing as failure to thrive. Am J Dis Child 1981; 135:713-715.  Back to cited text no. 5    
6.Cummings BJ, Hodson DI, Bush RS. Chordoma: The results of megavoltage radiation therapy. Int J Radial Oncol Biol Phys 1938; 9:633-642  Back to cited text no. 6    
7.Fink FRI, Ausserer B, Schrochcnadel W, Pallus AK, Frommhold H, Mikuz G. Clivus chordoma in a 9 year old child. Case report and review of the literature. Pediatr Hematol Oncol 1987; 4:91-100.  Back to cited text no. 7    
8.Handa J, Suzukt F, Hirofumi N, Koyama T. Clivus chordomas in childhood. Surg Neurol 1987; 28:58-62  Back to cited text no. 8    
9.Kaneko Y, Sato Y, Iwaki T, Shin RW, Tateishi J, Fukui M, et al. Chordoma in early childhood: A clinicopathological study. Neurosurgery 1991; 29:442-446  Back to cited text no. 9    
10.Krayenbuht H, Yasargil MG. Cranial chordomas. Prog Neutol Surg 1975; 6:380-434  Back to cited text no. 10    
11.Nakamura Y, Becker LE. Marks A. S100 protein in human chordoma and human and rabbit notochord Arch Pathol Lab Med 1983; 107:118-120  Back to cited text no. 11    
12.Nolte K. Maliganant intracranial chordoma and sarcoma of the clivus and infancy. Pediatr Radiol 1979; 8:1-6.  Back to cited text no. 12    
13.Plase JPP, Borges JM, Nudelman M, Lefevre AB, Sallum J. Unusual subarachnoid metastasis of an intracranial chordoma in infancy. Child's Brain 1978; 4:25-26  Back to cited text no. 13    
14.Raffei G, Wright DC, Gutin PH, Wilson CB. Cranial chordomas, clinical presentation and results of operative and radiation therapy in twenty-six patients. Neurosurgery 1985; 17:703-710  Back to cited text no. 14    
15.Sassin JF. Chutorian AM. Intracranial chordoma in children Arch Neurol 1967; 17:89-93.  Back to cited text no. 15    
16.Schneegans E, Mandigaz M. Hydrocepgalie non communicate due a un chordome malin. Bull Soc Pediatr (Paris) 1938; 436:535-538  Back to cited text no. 16    
17.Larson TC III, Houser OW, Laws ER Jr. Imaging of cranial chordomas Mayo Clin Proc 1987; 62:886-893.  Back to cited text no. 17    
18.Rich A, Schiller A, Suit HD, Mankin HJ. Clinical and pathologic review of 48 cases of chordoma. Cancer 1985; 56:182-187.  Back to cited text no. 18    
19.Russell DS, Rubinstein U. Chondrosarcomas and chordomas. In: Pathology of tumours of the nervous system. Baltimore: Williams & Wilkins; 1989, pp 819-821  Back to cited text no. 19    
20.Sibley RK, Day DL, Dehiner LP, Trueworthy RG. Metastasizing chordoma in early childhood a pathological and immunohistochemical study with review of the literature. Pediatr Pathology 1987; 7:287-301.  Back to cited text no. 20    
21.Heffeifinger MJ, Dahlin DC, MacCarty CS, Beabout JW. Chordomas and cartilaginous tumours at the skull base Cancer 1973; 23:410-420.  Back to cited text no. 21    
22.Higinbotham NL, Phillips RF, Farr HW, Hustu HO. Chordoma. Thirty-five year study at Memorial hospital Cancer 1967; 11:1841-1850.  Back to cited text no. 22    
23.Sen CK, Selkhar LN, Schramm VL Jr, Jenecka JP. Chordoma and chondrosarcoma of the cranial base an 8-year experience. Neurosurgery 1989; 26:931-941  Back to cited text no. 23    
24.Goel A. Middle fossa subgasserian ganglion approach to clival chordomas. Acta Neurochir (Wien) 1995; 136:212-216  Back to cited text no. 24    
25.Erbengi A, Tekkok IH, Acikgoz B. Posterior fossa chordomas with special reference to transoral surgery Neurosurg Rev 1991; 14:23-28.   Back to cited text no. 25    


    Figures

[Figure - 1], [Figure - 2]

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INTERNATIONAL JOURNAL OF CANCER. 2000; 87 (1): 68-72
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