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CASE REPORT
Year : 2020  |  Volume : 13  |  Issue : 4  |  Page : 406-409  

An unusual location of schwannoma in a 4-year-old child


1 Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha “O” Anusandhan University (Deemed to be), Bhubaneswar, Odisha, India
2 Medical Research Laboratory, IMS and SUM Hospital, Siksha “O” Anusandhan University (Deemed to be), Bhubaneswar, Odisha, India

Date of Submission16-Apr-2019
Date of Decision04-Jun-2019
Date of Acceptance03-Sep-2019
Date of Web Publication20-Jul-2020

Correspondence Address:
Santosh Kumar Swain
Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha “O” Anusandhan University (Deemed to be), Bhubaneswar, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_108_19

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  Abstract 


Schwannomas are relatively rare neoplasm and even rarer in the pediatric age group. The nerve sheath tumors originating from the nerves of the neck are extremely rare. We report a 4-year-old girl presenting with a history of an asymptomatic neck swelling for 6 months. The child was meticulously evaluated with imaging and fine-needle aspiration cytology. Complete surgical excision was done, and the histopathological examination confirmed the diagnosis. The child was completely recovered without any morbidity. Here, we present this case of schwannoma as it is rarely seen in the pediatric age group where the child was recovered completely. Hence, there is a possibility for complete cure and without complications of schwannoma in the neck of a child after complete excision of the tumor.

Keywords: Benign tumor, child, neck, schwannoma


How to cite this article:
Swain SK, Samal S. An unusual location of schwannoma in a 4-year-old child. Med J DY Patil Vidyapeeth 2020;13:406-9

How to cite this URL:
Swain SK, Samal S. An unusual location of schwannoma in a 4-year-old child. Med J DY Patil Vidyapeeth [serial online] 2020 [cited 2020 Aug 9];13:406-9. Available from: http://www.mjdrdypv.org/text.asp?2020/13/4/406/290153




  Introduction Top


Schwannoma or neurilemmoma is a benign neoplasm that arises from the Schwann cells of the nerve sheath. It is a slow-growing, encapsulated, and often solitary tumor.[1] Extracranially, around 25%–40% of the schwannomas are found in the soft tissues of the head and neck area.[2] This neoplasm is common in the second and third decades, but very rare in children of age <10 years.[3] The etiology of this tumor is still not known. This tumor is usually asymptomatic and clinical presentations depend on the size and site of the tumor. The treatment of choice is complete surgical excision. The surgical resection of the tumor may lead to complications such as hoarseness of voice, anesthesia in pharyngolaryngeal region, aspiration, and paralysis of cranial nerves which may be transient or permanent.[4] Recurrence is often rare after surgical excision of the tumor.[5] Here, we report a case of schwannoma in the neck of a 4-year-old child, who completely cured after surgical excision of the tumor without any complications.


  Case Report Top


A 4-year-old girl presented with an asymptomatic swelling at the right side of the neck for 6 months, which is increasing in size gradually to present one. On examination, it is nontender and firm in consistency and approximately 4 cm × 5 cm in size at the lower one-third of the sternocleidomastoid muscle [Figure 1]. It was mobile in the transverse direction, but not in the vertical direction. The child had no voice change, nasal regurgitation, and syncopal attack. The carotid pulsations were palpable just anterior to the mass. Examination of the oral cavity, oropharynx, and larynx was within normal limit. Ultrasonographic examination showed a 4 cm × 5 cm × 5 cm mass, with plenty of vascularity and displacing the internal jugular vein and internal carotid artery. Computed tomography (CT) scan revealed a mass displacing the jugular vein and carotid artery anteromedially with neck nodes enlarged [Figure 2]. Ultrasonography (USG)-guided fine-needle aspiration cytology (FNAC) revealed the report as schwannoma [Figure 3]. The neck of the patient was explored by an oblique incision along the anterior border of the sternocleidomastoid muscle. The tumor was arising from the vagus nerve and extending toward the skull base area. The tumor mass was excised completely [Figure 4] along with the vagus nerve. The child was recovered completely without any postoperative symptoms. The histopathological report confirmed the diagnosis of schwannoma. The histopathological report showed the encapsulated benign neurogenic tumor which was composed of spindle cells. Spindle cells revealed hypercellular and hypocellular areas giving rise to Antoni A and B types of patterns, prominent Verocay bodies [Figure 5]. The patient was discharged after 1 week and is under regular follow-up. The child was followed up at 6 months, 1 year, and 2 years, and there was no evidence of recurrence.
Figure 1: Clinical photograph of a 4-year-old child showing right side neck swelling (schwannoma)

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Figure 2: Computed tomography scan of the neck showing schwannoma in the right side

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Figure 3: Fine-needle aspiration cytology showing fascicle of tumor cells in cytology smear of schwannoma (H and E, ×440)

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Figure 4: Photograph showing schwannoma excised in toto

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Figure 5: Photomicrograph of the excised schwannoma showing cellular (Antoni A) and hypocellular (Antoni B) regions and a palisade-style layout of the tumor cells (Verocay body) (H and E, ×200)

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  Discussion Top


A schwannoma is an encapsulated and solitary tumor that arises from the Schwann cells of the peripheral nerve sheath. In the neck, the retrostyloid compartment of the parapharyngeal space is the most common location for schwannoma.[6] Although the neck mass or swelling is common in children, the majority of the etiologies are reactive lymph nodes. Neurogenic tumors are extremely rare in the pediatric age group, and it accounts for 2% of the benign nonlymphadenomatons lesions.[7] In the neck, this tumor often arises from the cranial nerves such as vagus, glossopharyngeal, accessory or hypoglossal nerve, or in some cases from sympathetic chain. Although these tumors are benign in nature, they can also undergo malignant transformation.[8] The history of painless mass, mobile laterally, but immobile vertically with nonpulsatile in nature often gives suspicion of schwannoma. Most of the schwannomas are asymptomatic and may present with dysphagia or throat pain by its pressure effect on the surrounding structures. Clinical presentations due to nerve compression are rare as the nerve trunk often lies in the loose fascial compartment and schwannomas by nature are usually noninfiltrative.[9]

FNAC has an important role for the diagnosis of head and neck tumors. However, the ability of the FNAC for diagnosing neurogenic tumors is poor. It can give correct diagnosis in only 25% of head and neck schwannomas.[10] Schwann cell is common to both schwannoma and neurofibromas, which can be differentiated only under a microscope.[11] CT scan, magnetic resonance imaging (MRI), and angiography often confirm the diagnosis. The contrast-enhanced CT is helpful to determine the size, extent, and vascularity of the tumor. Hence, it is useful for surgical approach. A tumor on contrast CT pushing the internal carotid artery or common carotid artery is in favor of schwannoma arising from sympathetic chain or vagus nerve.[12] MRI is a routine imaging for schwannoma, which shows hypointense on T1- and hyperintense on T2-weighted images, depending on the cellularity of the tumor.[13] Incisional biopsy is discouraged due to the vascularity nature of the tumor, and there is a chance of uncontrolled bleeding and may become difficult to remove the tumor due to obliteration of the tissue plane in the neck. Complete surgical excision and histopathological examination confirm the diagnosis of schwannoma. The histopathological report shows two main patterns: Antoni A and Antoni B. Antoni A tissue is characterized by Verocay bodies. There is palisading of the nuclei about a central mass of cytoplasm whereas the Antoni B shows loosely arranged stroma where fibers and cells form no distinctive pattern. A mixed pattern of both types can also be seen in some cases.

The treatment of choice includes complete surgical excision of the tumor mass. Cautious surgical excision includes extracapsular peeling or even intracapsular encluation of the tumor from the nerve with preserving of the neural function.[14] If the tumor is known to be schwannoma, it is better to open the capsule and shell out the tumor, so leaving the capsular nerve fibers undisturbed and avoid functional deficits. The neurological functions can be known by using a nerve stimulator or doing surgery under a microscope for performing intracapsular encluation. There is no significant difference in the recurrence rate between the complete tumor resection including nerve fibers and the intracapsular encluation.[15] In this surgery, preservation of the nerve is usually advisable. However, in case of large tumor, the nerve may be sacrificed for achieving total removal of the schwannoma. Sometimes, the salvaged nerve can be repaired using greater auricular nerve or sural nerve. Resection of the tumor along with nerve does not always result in postoperative neurological deficit. After surgery, if the neurological deficit present are usually of partial loss of sensory or motor action. Moreover, the nerves giving rise to the tumor are often nonfunctional at the time of the surgery, and the risks leading to disabling neurological deficits after sacrificing these nerves are also very minimal.


  Conclusion Top


Schwannoma is a benign and slow-growing tumor in the head and neck region. These tumors are commonly seen in adults, but extremely rare among children. Although such tumors are rare in children, it should be considered as a differential diagnosis in a pediatric patient presenting with a lateral neck mass. FNAC is inconclusive in many cases. Imaging methods such as CT scan and MRI are very useful investigations for confirming the diagnosis. The treatment is complete surgical excision, which gives rise to complete cure and without any complications. A close follow-up is required after surgery of this tumor.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Karaca CT, Habesoglu TE, Naiboglu B, Habesoglu M, Oysu C, Egeli E, et al. Schwannoma of the tongue in a child. Am J Otolaryngol 2010;31:46-8.  Back to cited text no. 1
    
2.
Ma Q, Song H, Zhang P, Hou R, Cheng X, Lei D. Diagnosis and management of intraparotid facial nerve schwannoma. J Craniomaxillofac Surg 2010;38:271-3.  Back to cited text no. 2
    
3.
Nakasato T, Kamada Y, Ehara S, Miura Y. Multilobular neurilemmoma of the tongue in a child. AJNR Am J Neuroradiol 2005;26:421-3.  Back to cited text no. 3
    
4.
Gaikwad N, Sathe NU, Wadkar G, Chiplunkar D. Schwannoma of the cervical vagus nerve in a child: A case report. Indian J Otolaryngol Head Neck Surg 2013;65:188-91.  Back to cited text no. 4
    
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Mevio E, Gorini E, Lenzi A, Migliorini L. Schwannoma of the tongue: One case report. Rev Laryngol Otol Rhinol (Bord) 2002;123:259-61.  Back to cited text no. 5
    
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Cashman E, Skinner LJ, Timon C. Thyroid swelling: An unusual presentation of a cervical sympathetic chain schwannoma. Medscape J Med 2008;10:201.  Back to cited text no. 6
    
7.
Aggarwal G, Satsangi B, Shukla S, Lahoti BK, Mathur RK, Maheshwari A. Rare asymptomatic presentations of schwannomas in early adolescence: Three cases with review of literature. Int J Surg 2010;8:203-6.  Back to cited text no. 7
    
8.
Endo M, Yamamoto H, Harimaya K, Kohashi K, Ishii T, Setsu N, et al. Conventional spindle cell-type malignant peripheral nerve sheath tumor arising in a sporadic schwannoma. Hum Pathol 2013;44:2845-8.  Back to cited text no. 8
    
9.
Anil G, Tan TY. Imaging characteristics of schwannoma of the cervical sympathetic chain: A review of 12 cases. AJNR Am J Neuroradiol 2010;31:1408-12.  Back to cited text no. 9
    
10.
Lahoti BK, Kaushal M, Garge S, Aggarwal G. Extra vestibular schwannoma: A two year experience. Indian J Otolaryngol Head Neck Surg 2011;63:305-9.  Back to cited text no. 10
    
11.
Awasthi SK, Dutta A. Cervical sympathetic chain schwannoma: A case report. Indian J Otolaryngol Head Neck Surg 2011;63:292-4.  Back to cited text no. 11
    
12.
Iacconi P, Faggioni M, De Bartolomeis C, Iacconi C, Caldarelli C. Cervical sympathetic chain schwannoma: A case report. Acta Otorhinolaryngol Ital 2012;32:133-6.  Back to cited text no. 12
    
13.
Bocciolini C, Dall'olio D, Cavazza S, Laudadio P. Schwannoma of cervical sympathetic chain: Assessment and management. Acta Otorhinolaryngol Ital 2005;25:191-4.  Back to cited text no. 13
    
14.
Kim SH, Kim NH, Kim KR, Lee JH, Choi HS. Schwannoma in head and neck: Preoperative imaging study and intracapsular enucleation for functional nerve preservation. Yonsei Med J 2010;51:938-42.  Back to cited text no. 14
    
15.
Nacef I, Kedous S, Attia Z, Touati S, Gritli S. Cervical sympathetic chain schwannoma: A case report. Egypt J Ear Nose Throat Allied Sci 2014;15:169-72.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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