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HEAD & NECK RADIOLOGY Table of Contents   
Year : 2000  |  Volume : 10  |  Issue : 1  |  Page : 31-32
Case report: Primary cervical neuroblastoma with intracranial extension in a seven-year male


1 Dept of Radiology, Pt B.D Sharma Postgraduate Institute of Medical Science, Rohtak , Haryana, India
2 Dept of ENT, Pt B.D Sharma Postgraduate Institute of Medical Science, Rohtak , Haryana, India
3 Dept of Pathology, Pt B.D Sharma Postgraduate Institute of Medical Science, Rohtak , Haryana, India

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Keywords: Neuroblastoma, Computed tomography, neck, neoplasms, infants and children.

How to cite this article:
Magu S, Mishra DS, Gulati SP, Marwah N, Kakkar V, Sukhija R. Case report: Primary cervical neuroblastoma with intracranial extension in a seven-year male. Indian J Radiol Imaging 2000;10:31-2

How to cite this URL:
Magu S, Mishra DS, Gulati SP, Marwah N, Kakkar V, Sukhija R. Case report: Primary cervical neuroblastoma with intracranial extension in a seven-year male. Indian J Radiol Imaging [serial online] 2000 [cited 2019 Nov 19];10:31-2. Available from: http://www.ijri.org/text.asp?2000/10/1/31/30629
Neuroblastoma is the most common extracranial solid malignancy in children, arising from undifferentiated precursor cells of the sympathetic nervous system. A cervical location is relatively uncommon (5%) as compared with other reported sites such as the abdomen (65%), throat (15%) and pelvis (5%) [1]. Tumors arising in the postnasal space and the adjacent soft tissues penetrate the skull base in the region of the foramen lacerum, involving vital structures in the ipsilateral parasellar region and within the cavernous sinus [2].

We would like to report a case of primary cervical neuroblastoma and its computed tomographic findings in a seven-year man.


   Case Report Top


A seven-year old boy presented with inability to open the mouth and difficulty to swallow for six months. He complained of pain in the right ear and dribbling of saliva. The pain was intermittent to begin with and had gradually become persistent.

Local examination of the right ear showed bulging of the tympanic membrane with a fluid level. Oral examination revealed bulging of the soft palate on the right side. Local examination revealed non-tender cervical lymphadenopathy on the right side of the neck. The patient was investigated for a suspected right parapharyngeal mass. Plain radiography of the base of the skull showed erosion of the right temporal bone. Contrast enhanced CT showed a mass in the right post styloid parapharyngeal space causing anterior displacement of the carotid artery with compression and anterior displacement of the internal jugular vein and airway lumen [Figure - 1]. Coronal CT revealed an enhancing parapharyngeal mass with destruction of the basal foramina and its intracranial extension into the right middle cranial fossa and the parasellar region [Figure - 2]. No calcification was seen within the mass.

The final pathological diagnosis was a mature "well-differentiated neuroblastoma."


   Discussion Top


Of all the malignant solid tumors in children, excluding intracranial neoplasms, 5-10% occur in the head and neck region [3]. Malignant lymphomas and soft tissue sarcomas are the most frequent neoplasms in children. Rhabdomyosarcomas, the commonest soft-tissue sarcomas in childhood [4] are the second most common malignant neoplasm of the head and neck region (17%). The primary sites for rhabdomyosarcoma include the orbit (35%), pharynx (23%), nasopharynx (4%), maxillary sinus (4%), angle of the jaw (8%), postauricular (4%) and the temporal bone (4%) [5].

Neuroblastoma, a malignant embryonic tumor of the nervous system with neuroblastic differentiation is the fourth most common malignancy in children [6],[7]. The majority (70%) of neuroblastomas that are discovered in the head and neck region are metastatic lesions to the cervical lymph nodes, cranial bones or retro-orbital area [8].

Despite the frequency of metastatic origin, primary neuroblastomas are the sixth most common head and neck intracranial neoplasms, 5-10% occur in the head and neck region [3]. Malignant lymphomas and soft tissue sarcomas are the most frequent neoplasms in children. Rhabdomyosarcomas, the commonest soft-tissue sarcomas in childhood [4] are the second most common malignant neoplasm of the head and neck region (17%). The primary sites for rhabdomyosarcoma include the orbit (35%), pharynx (23%), nasopharynx (4%), maxillary sinus (4%), angle of the jaw (8%), postauricular (4%) and the temporal bone (4%) [5].

Neuroblastoma, a malignant embryonic tumor of the nervous system with neuroblastic differentiation is the fourth most common malignancy in children [6],[7]. The majority (70%) of neuroblastomas that are discovered in the head and neck region are metastatic lesions to the cervical lymph nodes, cranial bones or retro-orbital area [8].

Despite the frequency of metastatic origin, primary neuroblastomas are the sixth most common head and neck malignancy in childhood [8]. Primary neuroblastomas of the neck usually arise in the cervical sympathetic ganglia [9]. They occur predominantly in the first three years of life with preponderance in the first 6 months [10].

Patients present with a smooth, solid, rubbery, painless mass in the neck, dysphagia, Horner's syndrome, stridor, cyanosis, difficulty in swallowing and food aspiration due to compression on the pharynx [11].

Prior to modern imaging, upward growth of such tumors was difficult to demonstrate. Bone erosion in the region of the foramen lacerum was the only plain film sign of intracranial extension of the carcinoma of the postnasal space. This information alters the management, changing the grading to T4, thus requiring a larger field during radiation therapy [2].

Intracranial extension of a cervical neuroblastoma via the jugular foramen or carotid canal with potential involvement of cranial nerves IX, XI, XII has been noted [1]. The foramen lacerum may provide access to the intracranial cavity for tumors in the postnasal space [2]. MRI has better soft-tissue resolution and also demonstrates well the relationship of the tumor to vascular structures [11].

In a report of two cases of cervical neuroblastoma, MRI demonstrated a homogenous, hyperintense, well-defined mass with a density similar to that of the spinal cord, which was suggestive of its neurologic origin [12]. This supports the earlier observation by Casselman et al [11] who observed that high signal intensity is seen on T1W, T2W and PDW images. The mass is isointense with the cervical cord on T1W, T2W and PDW images.

Abramson et al in a report of cervical neuroblastoma in eleven infants have observed that MRI has been the method of choice for delineating the extent of disease. Ultrasonographic studies are generally nonspecific and demonstrate a solid, hypoechoic complex mass, frequently with accompanying enlarged nodes. Although small calcifications can sometimes be seen in the tumors, color Doppler frequently demonstrates the displacement of the jugular vein and carotid artery around the tumor [13].

Correlating the CT findings along with the age, the possibilities of rhabdomyosarcoma, Ewing's tumor and neuroblastoma should be kept in mind. In the present case it was difficult to be sure about the entry route into the cranial cavity; foramen lacerum or ovale because of their proximity. This distinction is unimportant since spread occurs to the same vital structures by either route [2].

 
   References Top

1.Bousvaros A, Kirks DR, Grossman H. Imaging of neuroblastoma:an overview. Pediatr Radiol 1986; 16: 89-106.   Back to cited text no. 1  [PUBMED]  
2.Phelps PD, Beale DJ. The Foramen Lacerum A route of access to the cranial cavity for malignant tumors below the skull base. Clin Radiol 1992; 46: 179-183.   Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Pearson ADJ, Nesbit ME. Connective tissue malignancies of the head and neck region. In: McQuarie DG, Adams GL, Shons AR, Brownie GA (eds). Head and Neck Cancer. Clinical decisions and management principles¬ Chicago: Year Book Medical Publishers, 1986; 423-429.   Back to cited text no. 3    
4.Soule EH, Mahour GH, Mills SD et al . Soft tissue sarcomas of infants and children. A clinico-pathological study of 13µ cases. Mayo Clin Proc 1968; 43: 313-326.   Back to cited text no. 4    
5.Newman AN, Rice DH. Rhabdomyosarcoma of the head and neck. Laryngoscope 1984; 94: 234-239.   Back to cited text no. 5    
6.Marsden HB. Childhood tumors. In: Duncan W (ed). The pathology and natural history of paediatric oncology: Recent results in cancer research, New York: Springer-Verlag, 1983; 11-25.   Back to cited text no. 6    
7.Young JL, Miller RW. Incidence of malignant tumors in US children. J Pediatr 1975; 86: 254-258.   Back to cited text no. 7    
8.Jaffe BF. Paediatric head and neck tumors. A study of 17¸ cases. Laryngoscope 1973; 83: 1644-1651.   Back to cited text no. 8    
9.Coleman AJ, Fryer CJH, Elwood JM et al . Neuroblastoma: Influence of age at diagnosis, stage, tumor site and sex on prognosis. Cancer 1980; 46: 1896-1901.   Back to cited text no. 9    
10.Or JD. Cervical neuroblastoma in childhood: A better prognosis? Clinical Oncol 1978; 4: 353-358.   Back to cited text no. 10    
11.Casselman JW, Smet MH, Van Damme B, Lemahieu SF. Primary Cervical Neuroblastoma: CT and MR Findings. JCAT 1988; 12: 684-686.   Back to cited text no. 11    
12.Tai CF, Lee KS, Chen IH. Primary cervical Neuroblastoma in Infants. J Formos Med Assoc 1997; 96: 561-564.   Back to cited text no. 12    
13.Abramson SJ, Berdon WE, Ruzal-Shapiro C, Stolar C, Garvin J. Cervical neuroblastoma in eleven infants - a tumor with favorable prognosis. Pediatr Radiol 1993; 23: 253-257.   Back to cited text no. 13    

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Correspondence Address:
Sarita Magu
Residence Superintendent Boy's Hostel Pt.B.D. Sharma PGIMS, Rohtak 124001, Haryana
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2]

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