<%server.execute "isdev.asp"%> A unique pediatric case of intratumoral hemorrhage in a sporadic vestibular schwannoma Diyora B, Bhende B, Kotecha N, Mulla M, Dhal G, Nayak N - J Pediatr Neurosci
home : about us : ahead of print : current issue : archives search instructions : subscriptionLogin 
Users online: 1050      Small font sizeDefault font sizeIncrease font size Print this page Email this page


 
  Table of Contents    
CASE REPORT
Year : 2019  |  Volume : 14  |  Issue : 3  |  Page : 165-168
 

A unique pediatric case of intratumoral hemorrhage in a sporadic vestibular schwannoma


Department of Neurosurgery, LTMMC & LTMG Hospital, Sion, Mumbai-400022, Mumbai

Date of Submission02-Apr-2018
Date of Decision19-Jul-2018
Date of Acceptance28-May-2019
Date of Web Publication27-Sep-2019

Correspondence Address:
Dr. Batuk Diyora
Department of Neurosurgery, LTMMC & LTMG Hospital, Sion, Mumbai-400022.
Mumbai
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpn.JPN_44_18

Rights and Permissions

 

   Abstract 

Vestibular schwannomas (VSs) are tumors that commonly occur in the eighth cranial nerve. They are usually associated with type 2 neurofibromatosis. They are uncommon in children, and sporadic cases of pediatric VS are even rarer. In general, VSs are benign lesions with less than 1% chance of intratumoral hemorrhage. Adult cases of hemorrhage in VS are well documented. We present the first pediatric case of intratumoral hemorrhage in VS in the absence of any features of NF in an 11-year-old child who complained of holocranial headache and sensorineural hearing loss in the left ear. We further discuss the pathogenesis and clinical features, and review the literature of intratumoral hemorrhage in patients of VS.


Keywords: Facial nerve dysfunction, intratumoral hemorrhage, pediatric vestibular schwannoma


How to cite this article:
Diyora B, Bhende B, Kotecha N, Mulla M, Dhal G, Nayak N. A unique pediatric case of intratumoral hemorrhage in a sporadic vestibular schwannoma. J Pediatr Neurosci 2019;14:165-8

How to cite this URL:
Diyora B, Bhende B, Kotecha N, Mulla M, Dhal G, Nayak N. A unique pediatric case of intratumoral hemorrhage in a sporadic vestibular schwannoma. J Pediatr Neurosci [serial online] 2019 [cited 2019 Dec 12];14:165-8. Available from: http://www.pediatricneurosciences.com/text.asp?2019/14/3/165/267988





   Introduction Top


Vestibular schwannomas (VSs) are benign, encapsulated tumors arising from the peripheral nerve sheath of superior division of vestibulocochlear nerve. They are generally seen in adults and are rare in children (0.9%–2.08% of all unilateral acoustic neurinomas). Although a majority of VS in adults are sporadic, only 5% cases are associated with neurofibromatosis type 2 (NF2). However, most cases in the pediatric age group are associated with NF2 and sporadic cases are rare.[1] Patients with VS usually present with sensorineural hearing loss (98%), tinnitus (70%), disequilibrium or vertigo (67%), and headache (32%). Hemorrhage is uncommon in VS: only less than 50 cases have been reported in the literature with almost all belonging to the adult age group.[2],[3],[4],[5] In addition, only four cases of repeated intratumoral hemorrhage (ITH) have been reported and their occurrence has also been found exclusively in adolescents and adults.[6] Such patients present with a sudden onset of headache, nausea, vomiting, and vertigo. Less common symptoms include altered sensorium, diplopia, ataxia, gait disturbance, and facial hypesthesia[7],[8] that are associated with a poor prognosis. However, no pediatric case of ITH in VS has ever been reported in the past. We hence present the first pediatric case of ITH in VS that had a good postsurgical outcome.


   Case Report Top


An 11-year-old boy presented to the emergency room complaining of holocranial headache and vomiting since the evening before. Few hours before the onset of his symptoms, he was playing in the playground and suffered a minor trauma. He was treated by the family physician with medications that provided symptomatic relief. However, he experienced a recurrence of the headache the next morning along with difficulty in walking because of loss of balance with a tendency to fall on his left side, for which he was referred to our institute. On examination, he was found conscious, oriented, and had normal vital parameters. Cranial nerve examination revealed no abnormalities except sensorineural deafness in the left ear. Cerebellar signs such as heel–knee test, finger nose test, and dysdiadochokinesia were positive on the left side. Power, tone, and sensations were normal in both upper and lower limbs. Babinski sign was positive on both sides. Funduscopy examination revealed bilateral mild papilledema. Audiometric testing confirmed left-sided moderate grade sensorineural deafness. Computed tomography (CT) scan of the brain showed a well-defined lesion in the left cerebellopontine angle with central hypodensity and peripheral enhancement [Figure 1]A. Magnetic resonance imaging (MRI) of the brain revealed a 3.5 × 4.2 × 3cm sized extra-axial mass lesion in the left cerebellopontine angle, which was well defined, heterogeneously enhancing on contrast. Lesion appeared homogenous hyperintense on Fluid-attenuated inversion recovery (FLAIR) sequences [Figure 1]B and heterogeneous hyperintense on T1-weighted images [Figure 1]C with central hypointensity. The lesion was extending laterally into the internal auditory meatus and compressing the fourth ventricle medially.
Figure 1: Post contrast CT scan of the brain showing heterogeneous enhancing mass lesion in left cerebellopontine (CP) angle (A). MRI brain images showing a 3.5 × 4.2 × 3cm size left CP angle mass lesion, which appeared homogenous hyperintense on Fluid-attenuated inversion recovery sequences (B) and heterogeneous hyperintense on T1-weighted images (C) with central hypointensity. Postoperative CT scan showing complete excision of lesion (D). Postoperative MRI showing complete excision of lesion on T1-weighted images (E) and on post contrast study (F)

Click here to view


He underwent left retro mastoid suboccipital craniectomy in the lateral position. The dura was opened vertically near the lateral margin of the craniectomy. Cerebellum was found to be full and tense. By gently retracting the cerebellum upward and medially, the left cerebellomedullary cistern was opened and the cerebrospinal fluid was drained slowly till complete relaxation of the cerebellum was achieved. On gently retracting the cerebellum medially, a grayish red tumor capsule was visualized. On opening the tumor capsule, a gush of hemorrhagic fluid under pressure was drained out. The solid component of the tumor was excised with microsurgical techniques. The internal auditory meatus was drilled and the part of the tumor extending into the meatus was also excised after identifying and preserving the facial nerve at its anterosuperior location. Complete excision of the tumor was ensured and hemostasis was confirmed. The dura was closed watertight and the closure was carried out in layers.

Postoperatively, his headache and vomiting subsided immediately and his walking improved. He had House–Brackmann grade 3 facial weakness, which improved to grade 5 at the end of 4 weeks. Histopathological examination of the specimen showed Antoni A and B cells with areas of hemorrhage within the tumor [Figure 2]. Immediate postoperative CT scan showed complete excision of lesion [Figure 1]D. Postoperative MRI showed complete excision of lesion on T1 weighted images [Figure 1]E and on post contrast study [Figure 1]F. At 1-year follow-up, the patient was completely asymptomatic with normal left facial nerve function.
Figure 2: Photomicrograph showing Antoni A and Antoni B cells with evidence of ITH

Click here to view



   Discussion Top


VSs are benign tumors that commonly affect the eighth cranial nerve and hence are most commonly found in the cerebellopontine angle.[5] They have a strong association with NF2, which is an autosomal-dominant disorder. These tumors are more common in adults and are specifically most prevalent in the fifth and sixth decade of life. Their prevalence in children less than 9 years of age is less and is estimated to account for 0.9% of all acoustic schwannomas. Very rarely however, VS may occur sporadically in a child without any features of NF2.[1],[4] Acoustic schwannomas associated with NF-2 account for 2%–4% of all acoustic schwannomas with the mean patient age being younger than that of sporadic schwannomas.[2] Sporadic VS usually presents with sensorineural hearing loss (98%), tinnitus (70%), disequilibrium or vertigo (67%), and headache (32%).[7],[8],[9] Unlike in adults, the early symptoms of VS are easily neglected in children. Hence in children, the tumors are often identified by the more overt signs caused by the space occupying nature of the tumor, which include headache, vomiting, and loss of balance. This delay in diagnosis of months to years is the reason for the large size of such tumors in children.[1]

In intracranial tumors, hemorrhage is reported to be approximately 1.4%–10% and is more common in malignant and highly vascular lesions such as high-grade glioma, metastatic tumor, melanoma, and pituitary tumors.[3],[10],[11] In slow-growing benign tumors such as meningioma, VS, and low-grade glioma, such hemorrhage is rare. In VS, hemorrhage is reported in <1% of the patients. Since the initial report of ITH in VS by McCoyd et al.[12] in 1974, more than 50 cases of hemorrhagic VS have been reported in the literature in the form of case reports and case series.[2] Our sporadic case of an 11-year-old boy is one of its kind as this is the only pediatric case reported with ITH in an acoustic schwannoma. ITH causes a sudden increase in the size of the tumor. The most common presenting symptoms are sudden-onset headache, nausea, vomiting, and vertigo.[3] The less common symptoms may include altered sensorium, diplopia, ataxia, gait disturbance, and facial hypesthesia.[7],[8]

Although several mechanisms have been proposed, the exact etiology of ITH in VS is unknown. It has been widely postulated that tumor-induced angiogenesis produces arterial walls lacking media, which predisposes this vasculature to rupture. Occlusion of intratumoral vessels and necrosis of distal vessels are other mechanisms of ITH. High mindbomb homolog (MIB) index reflects rapid tumor growth due to high tumor doubling rate. Occasionally ITH associated with high MIB index and rapid growth of tumor is blamed for ITH.[13] ITH is not always associated with rapid growth rate.[14]

In adults, several predisposing factors for tumoral bleeding have been noted in acoustic schwannoma such as the mean tumor diameter (a mean diameter of 3.7cm is associated with hemorrhage),[8] cystic VS,[15] anticoagulation,[8] previous history of stereotactic radiation,[16] minor trauma,[17] strenuous exercise, or even severe effort.[14] Increased vascularity, thinning of tumor-associated vasculature, and mixed Antoni A and B cellularity are histopathologic characteristics of hemorrhagic VS that have been suggested to correlate with hemorrhage.[3],[18],[19]

Treatment options for cases of VS include observation, radiation therapy, and surgical resection. The treatment approach of a VS presenting with ITH has not been clearly defined. Because of the limited number of reported cases available in literature at present, each case needs to be approached individually. Surgical excision is the best option available.[20],[21] Prognosis of these patients after surgical excision is good. However, ITH in adult VS is associated with significant morbidity and mortality. The reported incidence of facial nerve palsy and mortality is much higher in all cases of VS compared to that of patients with hemorrhagic VS.[17] As sporadic VSs in pediatric population are rare, decision for the best treatment options is complicated.[22] They can be best treated by microsurgical resection with good clinical outcome. Equally good long-term results can be achieved by radiation therapy. However, ITH in sporadic case of pediatric VS should be best treated with microsurgical excision.


   Conclusion Top


Although unilateral VSs in children are rare, they do occur in the absence of features of NF. ITH in VS has been reported multiple times in the adult population, but no such pediatric documentation exists. However, in view of our case, such a possibility exists. Pathologic microvasculature within VS predisposes ITH following minor trauma. ITH in VS potentially increases the risk of neurologic deficit, especially hearing loss and facial dysfunction, hence demanding special attention to ensure good surgical outcome. Such a good clinical outcome including facial preservation can be achieved with microsurgical excision.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Mazzoni A, Dubey SP, Poletti AM, Colombo G. Sporadic acoustic neuroma in pediatric patients. Int J Pediatr Otorhinolaryngol 2007;71:1569-72.  Back to cited text no. 1
    
2.
Samii M, Matthies C, Tatagiba M. Management of 1000 vestibular schwannoma (acoustic neuromas): auditory and facial nerve functions after resection of 120 VSs in patients with NF-2. Neurosurgery 1992;40:696-706.  Back to cited text no. 2
    
3.
Asari S, Katayama S, Itoh T, Tsuchida S, Furuta T, Ohmoto T. Neurinomas presenting as spontaneous intratumoral hemorrhage. Neurosurgery 1992;31:406-11; discussion 411-2.  Back to cited text no. 3
    
4.
Allcutt DA, Hoffman HJ, Isla A, Becker LE, Humphreys RP. Acoustic schwannomas in children. Neurosurgery 1991;29:14-8.  Back to cited text no. 4
    
5.
Guinto Balanzar G, Guinto-Nishimura Y. Intratumoral hemorrhage in vestibular schwannomas. World Neurosurg 2014;82:599-600.  Back to cited text no. 5
    
6.
Takeuchi S, Nawashiro H, Otani N, Sakakibara F, Nagatani K, Wada K, et al. Vestibular schwannoma with repeated intratumoral hemorrhage. J Clin Neurosci 2012;19:1305-7.  Back to cited text no. 6
    
7.
Odabasi AO, Buchman CA, Morcos JJ. Tumor-associated hemorrhage in patients with acoustic neuroma. Am J Otol 2000;21:706-11.  Back to cited text no. 7
    
8.
Vellin JF, Bozorg Grayeli A, Kalamarides M, Fond C, Bouccara D, Sterkers O. Intratumoral and brainstem hemorrhage in a patient with vestibular schwannoma and oral anticoagulant therapy. Otol Neurotol 2006;27:209-12.  Back to cited text no. 8
    
9.
Gaini SM, Giovanelli M, Motti EDF, Tomei G, Villani R. Acoustic neurinomas in infancy. Mod Probl Paediat 1977;18:90-3  Back to cited text no. 9
    
10.
Honda M, So G, Kaminogo M, Abe K, Nagata I. Massive intratumoral hemorrhage of ependymoma of the fourth ventricle. Childs Nerv Syst 2005;21:926-9.  Back to cited text no. 10
    
11.
Wakai S, Yamakawa K, Manaka S, Takakura K. Spontaneous intracranial hemorrhage caused by brain tumor: its incidence and clinical significance. Neurosurgery 1982;10:437-44.  Back to cited text no. 11
    
12.
McCoyd K, Barron KD, Cassidy RJ. Acoustic neurinoma presenting as subarachnoid hemorrhage. Case report. J Neurosurg 1974;41:391-3.  Back to cited text no. 12
    
13.
Ohta S, Yokoyama T, Nishizawa S. Massive haemorrhage into acoustic neurinoma related to rapid growth of the tumour. Br J Neurosurg 1998;12:455-7.  Back to cited text no. 13
    
14.
Diyora B, Sharma A, Badhe P, Nayak N. Hemorrhage in acoustic neurinoma. Neurol India 2010;58:329-30.  Back to cited text no. 14
[PUBMED]  [Full text]  
15.
Ganslandt O, Fahrig A, Strauss C. Hemorrhage into cystic vestibular schwannoma following stereotactic radiation therapy. Zentralbl Neurochir 2008;69:204-6.  Back to cited text no. 15
    
16.
Karampelas I, Alberico RA, Plunkett RJ, Fenstermaker RA. Intratumoral hemorrhage after remote subtotal microsurgical resection and gamma knife radiosurgery for vestibular schwannoma. Acta Neurochir (Wien) 2007;149:313-6.  Back to cited text no. 16
    
17.
Kojima Y, Tanaka N, Kuwana N. Massive hemorrhage in acoustic neurinoma after minor head trauma—case report. Neurol Med Chir (Tokyo) 1990;30:972-6.  Back to cited text no. 17
    
18.
Brady AP, Stack JP: Case report: magnetic resonance demonstration of haemorrhagic acoustic neuroma. Clin Radiol 1994;49:61-3.  Back to cited text no. 18
    
19.
Castillo R, Watts C, Pulliam M. Sudden hemorrhage in an acoustic neuroma. Case report. J Neurosurg 1982;56:417-9.  Back to cited text no. 19
    
20.
Niknafs YS, Wang AC, Than KD, Etame AB, Thompson BG, Sullivan SE. Hemorrhagic vestibular schwannoma: review of the literature. World Neurosurg 2014;82:751-6.  Back to cited text no. 20
    
21.
Walcott BP, Sivarajan G, Bashinskaya B, Anderson DE, Leonetti JP, Origitano TC. Sporadic unilateral vestibular schwannoma in the pediatric population. Clinical article. J Neurosurg Pediatr 2009;4:125-9.  Back to cited text no. 21
    
22.
Wang J, Xu Y, Lei T, Zeng L. Treatment decision–making for sporadic small vestibular schwannoma in a pediatric patient: a case report and literature review. Oncol Lett 2015;9:2371-3.  Back to cited text no. 22
    


    Figures

  [Figure 1], [Figure 2]



 

Top
Print this article  Email this article
 
 
  Search
 
  
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (959 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
    References
    Article Figures

 Article Access Statistics
    Viewed175    
    Printed15    
    Emailed0    
    PDF Downloaded7    
    Comments [Add]    

Recommend this journal