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Sudden neurological deterioration due to repeated intratumoral hemorrhage in a patient with a vestibular schwannoma
Journal of Clinical Neuroscience xxx (2018) xxx–xxx
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case report
Sudden neurological deterioration due to repeated intratumoral hemorrhage in a patient with a vestibular schwannoma Mostafa Fatehi Hassanabad ⇑, Ryojo Akagami Division of Neurosurgery, Vancouver General Hospital, 899, 12th Avenue West, Vancouver V5Z1M9, Canada
a r t i c l e
i n f o
Article history: Received 5 August 2018 Accepted 6 October 2018 Available online xxxx
a b s t r a c t Vestibular schwannomas (VS) are the most common tumors involving the cerebellopontine angle (CPA) and the internal auditory canal (IAC). These tumors are usually slow-growing and commonly present with cranial nerve dysfunction such as hearing loss. Repeated intratumoral hemorrhage (ITH) is extremely rare with only four cases previously reported. We report the case of a 30 year old female with a right sided CP angle tumor who presented with vertigo and ataxia due to ITH. Her symptoms initially improved; however, three weeks later, she had acute onset of facial palsy and imaging confirmed rebleeding. Surgical pathology reported typical features of schwannoma. A literature review performed using the PubMed and EMBASE databases yielded four previous reports. A summary of these cases is presented and the features of ITH are discussed. Patients affected by repeated ITH present with sudden headache and ataxia. Rapid worsening of cranial nerve dysfunction such as hearing loss or facial nerve palsy is suggestive of ITH. Ó 2018 Elsevier Ltd. All rights reserved.
1. Case presentation A 30 year old female with a prior history of Hodgkin’s lymphoma and gradual hearing loss was found to have a right CP angle mass in June 2016. An MRI in June 2016 further characterised the lesion and showed no significant change in size (Fig. 1). After consultation with a neurosurgeon, the patient elected to undergo watchful surveillance. In the ensuing months, the patient developed further hearing loss and hemifacial numbness (CN V2 and V3 distribution); however, follow-up MR imaging in January and June 2017 remained largely stable in size (Fig. 2). The development of headache, tinnitus and ataxia in late June 2017 prompted repeat MR imaging which demonstrated intratumoral hemorrhage and consequent enlargement of the tumor (Fig. 3). While the patient’s symptoms largely improved over the next week, she agreed to undergo neurosurgical treatment in July. However, the day before her scheduled procedure, the patient developed sudden headache and noticed rapidly progressive right facial nerve palsy. Urgent imaging confirmed repeat intratumoral hemorrhage (Fig. 4). The patient had intact level of consciousness throughout and her lower cranial nerves were not affected. Preoperatively, the patient had right-sided, House-Brackmann (HB) grade IV, facial nerve weakness, no hearing on the right ⇑ Corresponding author. E-mail address: [email protected] (M. Fatehi Hassanabad).
and decreased sensation in the right CN V2 and V3 distribution. Intraoperative neurophysiological monitoring was performed with CN V, CNVII and CN X nerve EMG monitoring. Bilateral brainstem auditory-evoked responses (BAERs) and CNVII motor evoked potentials (MEP) were also monitored. Good subtotal (>85%) resection of the tumor was achieved and a rind of tumor was left along the subarachnoid course of the facial nerve. Interestingly, the facial MEPs had slightly improved at the end of the procedure. Pathologic assessment demonstrated a benign spindle cell tumor characterized by areas of variable cellularity, focal Verocay body formation, and evidence of previous hemorrhage. The neurological exam remained stable in the post-operative period; however, her eye closure improved slowly. She was discharged to a rehabilitation center due to persistent ataxia. 2. Review of literature A search of the PubMed and EMBASE databases using the keywords ‘‘intratumoral hemorrhage” and ‘‘vestibular schwannoma”, limited to the English language, yields 20 articles. The search was further expanded to 31 articles using the citations of our initial search results. Most articles relate to patients presenting with intratumoral hemorrhage. Only four articles [3,4,6,9] described patients with repeated ITH and these reports are summarized in Table 1.
https://doi.org/10.1016/j.jocn.2018.10.052 0967-5868/Ó 2018 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Fatehi Hassanabad M, Akagami R. Sudden neurological deterioration due to repeated intratumoral hemorrhage in a patient with a vestibular schwannoma. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.052
2
M. Fatehi Hassanabad, R. Akagami / Journal of Clinical Neuroscience xxx (2018) xxx–xxx
Fig. 1. A. CT (without and with contrast) and B. MR imaging (T1 + Gad) acquired in June 2016 demonstrate an avidly enhancing extra-axial mass in the right CP angle. This lesion, which measures 2.1 2.2 1.9 cm, compresses the right cerebellar peduncle and extends into the IAC by 4 mm.
Fig. 2. Axial MR imaging; T1+Gad (A) and T2-weighted (B) from early June 2017 exhibit a stable mass in the right CPA. Imaging from late June 2017; T1+Gad MR images (C-E) and T2-weighted (F) show interval enlargment of the tumor secondary to intratumoral hemorrhage.
Fig. 3. T1+Gad MR images (A-C) and T2-weighted (D) obtained in late June 2017 show interval enlargment of the tumor secondary to intratumoral hemorrhage.
Please cite this article in press as: Fatehi Hassanabad M, Akagami R. Sudden neurological deterioration due to repeated intratumoral hemorrhage in a patient with a vestibular schwannoma. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.052
3
M. Fatehi Hassanabad, R. Akagami / Journal of Clinical Neuroscience xxx (2018) xxx–xxx
Fig. 4. CT (non-contrast) obtained in mid July 2017 (three weeks after initial bleed) shows further enlargement (3 2.7 2.9 cm3) of the tumor after repeat intratumoral hemorrhage.
Table 1 Existing reports of patients with vestibular schwannoma with repeated ITH. Patient (Age/gender)
Symptoms at initial hemorrhage
Symptoms at repeated hemorrhage
Interval between bleeds
Final tumor size
Post-op HB grade
Post-op hearing
56M [4]
Severe HL, tinnitus
10 m
3 4 5 cm3
II or III
Lost
35F [3]
Severe HL, tinnitus, ataxia, vomiting, sudden HA None
Sudden HA, CN V, VII, IX, X palsy Sudden HA, vomiting, ataxia
1–2 m
4 4 4 cm3
II
NR
Sudden HA, ataxia, papilledema Sudden HA, nausea, ataxia Sudden HA, CN VII palsy
3m
14.3 cm3
V
NR
III IV
Lost Lost
59M* [6] 15M [9] Present case
Severe HL, tinnitus Tinnitus, ataxia, sudden HA
2–3 m 3w
3
3.1 2.3 2.2 cm 3 2.7 2.9 cm3
HB = House-Brackmann, M = male, F = female, NR = not reported. * This patient suffered ITH three times. The initial bleed was asymptomatic and the interval reported is between the 2nd and 3rd bleed.
3. Discussion Hemorrhage into vestibular schwannomas is an uncommon (<1% of cases) cause of acute neurological deterioration [1,2,8]. This rarity is partially explained by the slower rate of growth in these tumors compared with more aggressive neoplasms. Repeated ITH is even more infrequent with only four cases reported to date. A recent review found there was no clear association between ITH and patient age (average age of onset 51.3 y), gender or tumor location [7]. However, the hemorrhagic tumors were larger than average VS. Other factors thought to correlate with bleeding risk include cellularity and increased vascularity [7]. The average age of patients with repeated ITH is 39y (15–59) and the tumors were quite large (average volume 35.5 cm3) and increased vascularity was noted. The time interval between bleeding episodes was uncharacteristically short in the present case (3 weeks vs several months). The current report re-affirms the clinical impact of repeated ITH; all patients presented with severe sudden headache and four cases report onset of ataxia. Rapid worsening of cranial nerve dysfunction, such as hearing loss, is a hallmark of ITH [5]. As expected, the onset of facial nerve palsy significantly increases after ITH. This is reported to occur in 6% of all patients with VS, 31% of patients with ITH and in 2 of 5 cases of repeated ITH. Due to the increased morbidity, physicians should consider close observation and early intervention in patients whose clinical presentation is suggestive of ITH.
Conflict of interest None declared. References [1] Chou D, Sampath P, Brem H. Hemorrhagic vestibular schwannoma: an unusual clinical entity. case report. Neurosurg Focus 1998;5(3). e9. [2] Dehdashti AR, Kiehl TR, Guha A. Vestibular Schwannomas presenting with haemorrhage: clinical presentation and histopathological evaluation of an unusual entity. Br J Neurosurg 2009;23(4):431–6. [3] Kim SH, Youm JY, Song SH, Kim Y, Song KS. Vestibular schwannoma with repeated intratumoral hemorrhage. Clin Neurol Neurosurg 1998;100(1):68–74. [4] Kurata A, Saito T, Akatsuka M, et al. Acoustic neurinoma presenting with repeated intratumoral hemorrhage. Case report. Neurol Med Chir (Tokyo) 1989;29:328–32. [5] Maimone G, Ganau M, Nicassio N, Cambria M. Clinical and radiological aspects of cerebellopontine neurinoma presenting with recurrent spontaneous bleedings. Surg Neurol Int 2013;4:67. [6] Mandl ES, Vandertop WP, Meijer OW, Peerdeman SM. Imaging-documented repeated intratumoral hemorrhage in vestibular schwannoma: a case report. Acta Neurochir (Wien) 2009;151(10):1325–7. [7] Niknafs YS, Wang AC, Than KD, Etame AB, Thompson BG, Sullivan SE. Hemorrhagic vestibular schwannoma: review of the literature. World Neurosurg 2014;82(5):751–6. [8] Sugihara S, Kinoshita T, Matsusue E, Fujii S, Ogawa T. Multicystic acoustic schwannoma with intratumoral hemorrhage: a report of two cases. Magn Reson Med Sci 2004;3(2):101–4. [9] Takeuchi S, Nawashiro H, Otani N, Sakakibara F, Nagatani K, Wada K, Osada H, Shima K. Vestibular schwannoma with repeated intratumoral hemorrhage. J Clin Neurosci 2012;19(9):1305–7.
Please cite this article in press as: Fatehi Hassanabad M, Akagami R. Sudden neurological deterioration due to repeated intratumoral hemorrhage in a patient with a vestibular schwannoma. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.052
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case report
Sudden neurological deterioration due to repeated intratumoral hemorrhage in a patient with a vestibular schwannoma Mostafa Fatehi Hassanabad ⇑, Ryojo Akagami Division of Neurosurgery, Vancouver General Hospital, 899, 12th Avenue West, Vancouver V5Z1M9, Canada
a r t i c l e
i n f o
Article history: Received 5 August 2018 Accepted 6 October 2018 Available online xxxx
a b s t r a c t Vestibular schwannomas (VS) are the most common tumors involving the cerebellopontine angle (CPA) and the internal auditory canal (IAC). These tumors are usually slow-growing and commonly present with cranial nerve dysfunction such as hearing loss. Repeated intratumoral hemorrhage (ITH) is extremely rare with only four cases previously reported. We report the case of a 30 year old female with a right sided CP angle tumor who presented with vertigo and ataxia due to ITH. Her symptoms initially improved; however, three weeks later, she had acute onset of facial palsy and imaging confirmed rebleeding. Surgical pathology reported typical features of schwannoma. A literature review performed using the PubMed and EMBASE databases yielded four previous reports. A summary of these cases is presented and the features of ITH are discussed. Patients affected by repeated ITH present with sudden headache and ataxia. Rapid worsening of cranial nerve dysfunction such as hearing loss or facial nerve palsy is suggestive of ITH. Ó 2018 Elsevier Ltd. All rights reserved.
1. Case presentation A 30 year old female with a prior history of Hodgkin’s lymphoma and gradual hearing loss was found to have a right CP angle mass in June 2016. An MRI in June 2016 further characterised the lesion and showed no significant change in size (Fig. 1). After consultation with a neurosurgeon, the patient elected to undergo watchful surveillance. In the ensuing months, the patient developed further hearing loss and hemifacial numbness (CN V2 and V3 distribution); however, follow-up MR imaging in January and June 2017 remained largely stable in size (Fig. 2). The development of headache, tinnitus and ataxia in late June 2017 prompted repeat MR imaging which demonstrated intratumoral hemorrhage and consequent enlargement of the tumor (Fig. 3). While the patient’s symptoms largely improved over the next week, she agreed to undergo neurosurgical treatment in July. However, the day before her scheduled procedure, the patient developed sudden headache and noticed rapidly progressive right facial nerve palsy. Urgent imaging confirmed repeat intratumoral hemorrhage (Fig. 4). The patient had intact level of consciousness throughout and her lower cranial nerves were not affected. Preoperatively, the patient had right-sided, House-Brackmann (HB) grade IV, facial nerve weakness, no hearing on the right ⇑ Corresponding author. E-mail address: [email protected] (M. Fatehi Hassanabad).
and decreased sensation in the right CN V2 and V3 distribution. Intraoperative neurophysiological monitoring was performed with CN V, CNVII and CN X nerve EMG monitoring. Bilateral brainstem auditory-evoked responses (BAERs) and CNVII motor evoked potentials (MEP) were also monitored. Good subtotal (>85%) resection of the tumor was achieved and a rind of tumor was left along the subarachnoid course of the facial nerve. Interestingly, the facial MEPs had slightly improved at the end of the procedure. Pathologic assessment demonstrated a benign spindle cell tumor characterized by areas of variable cellularity, focal Verocay body formation, and evidence of previous hemorrhage. The neurological exam remained stable in the post-operative period; however, her eye closure improved slowly. She was discharged to a rehabilitation center due to persistent ataxia. 2. Review of literature A search of the PubMed and EMBASE databases using the keywords ‘‘intratumoral hemorrhage” and ‘‘vestibular schwannoma”, limited to the English language, yields 20 articles. The search was further expanded to 31 articles using the citations of our initial search results. Most articles relate to patients presenting with intratumoral hemorrhage. Only four articles [3,4,6,9] described patients with repeated ITH and these reports are summarized in Table 1.
https://doi.org/10.1016/j.jocn.2018.10.052 0967-5868/Ó 2018 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Fatehi Hassanabad M, Akagami R. Sudden neurological deterioration due to repeated intratumoral hemorrhage in a patient with a vestibular schwannoma. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.052
2
M. Fatehi Hassanabad, R. Akagami / Journal of Clinical Neuroscience xxx (2018) xxx–xxx
Fig. 1. A. CT (without and with contrast) and B. MR imaging (T1 + Gad) acquired in June 2016 demonstrate an avidly enhancing extra-axial mass in the right CP angle. This lesion, which measures 2.1 2.2 1.9 cm, compresses the right cerebellar peduncle and extends into the IAC by 4 mm.
Fig. 2. Axial MR imaging; T1+Gad (A) and T2-weighted (B) from early June 2017 exhibit a stable mass in the right CPA. Imaging from late June 2017; T1+Gad MR images (C-E) and T2-weighted (F) show interval enlargment of the tumor secondary to intratumoral hemorrhage.
Fig. 3. T1+Gad MR images (A-C) and T2-weighted (D) obtained in late June 2017 show interval enlargment of the tumor secondary to intratumoral hemorrhage.
Please cite this article in press as: Fatehi Hassanabad M, Akagami R. Sudden neurological deterioration due to repeated intratumoral hemorrhage in a patient with a vestibular schwannoma. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.052
3
M. Fatehi Hassanabad, R. Akagami / Journal of Clinical Neuroscience xxx (2018) xxx–xxx
Fig. 4. CT (non-contrast) obtained in mid July 2017 (three weeks after initial bleed) shows further enlargement (3 2.7 2.9 cm3) of the tumor after repeat intratumoral hemorrhage.
Table 1 Existing reports of patients with vestibular schwannoma with repeated ITH. Patient (Age/gender)
Symptoms at initial hemorrhage
Symptoms at repeated hemorrhage
Interval between bleeds
Final tumor size
Post-op HB grade
Post-op hearing
56M [4]
Severe HL, tinnitus
10 m
3 4 5 cm3
II or III
Lost
35F [3]
Severe HL, tinnitus, ataxia, vomiting, sudden HA None
Sudden HA, CN V, VII, IX, X palsy Sudden HA, vomiting, ataxia
1–2 m
4 4 4 cm3
II
NR
Sudden HA, ataxia, papilledema Sudden HA, nausea, ataxia Sudden HA, CN VII palsy
3m
14.3 cm3
V
NR
III IV
Lost Lost
59M* [6] 15M [9] Present case
Severe HL, tinnitus Tinnitus, ataxia, sudden HA
2–3 m 3w
3
3.1 2.3 2.2 cm 3 2.7 2.9 cm3
HB = House-Brackmann, M = male, F = female, NR = not reported. * This patient suffered ITH three times. The initial bleed was asymptomatic and the interval reported is between the 2nd and 3rd bleed.
3. Discussion Hemorrhage into vestibular schwannomas is an uncommon (<1% of cases) cause of acute neurological deterioration [1,2,8]. This rarity is partially explained by the slower rate of growth in these tumors compared with more aggressive neoplasms. Repeated ITH is even more infrequent with only four cases reported to date. A recent review found there was no clear association between ITH and patient age (average age of onset 51.3 y), gender or tumor location [7]. However, the hemorrhagic tumors were larger than average VS. Other factors thought to correlate with bleeding risk include cellularity and increased vascularity [7]. The average age of patients with repeated ITH is 39y (15–59) and the tumors were quite large (average volume 35.5 cm3) and increased vascularity was noted. The time interval between bleeding episodes was uncharacteristically short in the present case (3 weeks vs several months). The current report re-affirms the clinical impact of repeated ITH; all patients presented with severe sudden headache and four cases report onset of ataxia. Rapid worsening of cranial nerve dysfunction, such as hearing loss, is a hallmark of ITH [5]. As expected, the onset of facial nerve palsy significantly increases after ITH. This is reported to occur in 6% of all patients with VS, 31% of patients with ITH and in 2 of 5 cases of repeated ITH. Due to the increased morbidity, physicians should consider close observation and early intervention in patients whose clinical presentation is suggestive of ITH.
Conflict of interest None declared. References [1] Chou D, Sampath P, Brem H. Hemorrhagic vestibular schwannoma: an unusual clinical entity. case report. Neurosurg Focus 1998;5(3). e9. [2] Dehdashti AR, Kiehl TR, Guha A. Vestibular Schwannomas presenting with haemorrhage: clinical presentation and histopathological evaluation of an unusual entity. Br J Neurosurg 2009;23(4):431–6. [3] Kim SH, Youm JY, Song SH, Kim Y, Song KS. Vestibular schwannoma with repeated intratumoral hemorrhage. Clin Neurol Neurosurg 1998;100(1):68–74. [4] Kurata A, Saito T, Akatsuka M, et al. Acoustic neurinoma presenting with repeated intratumoral hemorrhage. Case report. Neurol Med Chir (Tokyo) 1989;29:328–32. [5] Maimone G, Ganau M, Nicassio N, Cambria M. Clinical and radiological aspects of cerebellopontine neurinoma presenting with recurrent spontaneous bleedings. Surg Neurol Int 2013;4:67. [6] Mandl ES, Vandertop WP, Meijer OW, Peerdeman SM. Imaging-documented repeated intratumoral hemorrhage in vestibular schwannoma: a case report. Acta Neurochir (Wien) 2009;151(10):1325–7. [7] Niknafs YS, Wang AC, Than KD, Etame AB, Thompson BG, Sullivan SE. Hemorrhagic vestibular schwannoma: review of the literature. World Neurosurg 2014;82(5):751–6. [8] Sugihara S, Kinoshita T, Matsusue E, Fujii S, Ogawa T. Multicystic acoustic schwannoma with intratumoral hemorrhage: a report of two cases. Magn Reson Med Sci 2004;3(2):101–4. [9] Takeuchi S, Nawashiro H, Otani N, Sakakibara F, Nagatani K, Wada K, Osada H, Shima K. Vestibular schwannoma with repeated intratumoral hemorrhage. J Clin Neurosci 2012;19(9):1305–7.
Please cite this article in press as: Fatehi Hassanabad M, Akagami R. Sudden neurological deterioration due to repeated intratumoral hemorrhage in a patient with a vestibular schwannoma. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.052