Abstract

Background: Although hemorrhages associated with cervical and thoracic intraspinal schwannomas are typically localized to the subarachnoid hemorrhages (SAH) or subdural hemorrhages (SDH) compartments, rare intratumoral bleeds may also occur.

Methods: In the literature, we found and analyzed multiple factors for 13 cases (e.g., epidemiological, clinical, and pathological) of cervical schwannomas with intratumoral hemorrhages (ITH). We added the 14^th case of a 35-year-old female with along segment cervical schwannoma with ITH who presented with acute quadriplegia and respiratory decompensation.

Results: These 14 patients averaged 51.77 years of age, 60% were male, and the tumor involved 2.83 segments. The incidence of SAH and ITH was noted in five cases each, while SDH’s were very rare. The pathological characteristics were consistent with the diagnosis of cellular schwannomas with S-100 positivity. The clinical outcomes were good (100%) in all the cases, including the one presented (modified McCormick score III).

Conclusion: Cervical schwannomas with ITH are rare, and the surgical outcomes in such patients are good-excellent (>90%). The histopathology is always of prime importance and decisive in establishing and confirming the etiology of such ITH.

Keywords: Cellular schwannoma, Intratumoral bleed, Schwannoma with bleed

INTRODUCTION

Schwannomas are some of the most common peripheral nerve sheath tumors.[ 1 ] They are classified, and outcomes/prognosis are closely tied to tumor size and location.[ 3 , 7 ] Spinal schwannomas very rarely present with acute neurological deterioration secondary to intratumoral hemorrhages (ITH); other hemorrhagic sites include tumor-related subarachnoid hemorrhages (SAH) or subdural hemorrhages (SDH). Here, we present review of 13 cases of ITH attributed to cervical spinal schwannoma in the literature and have added our 14^th cases to the series.

MATERIALS AND METHODS

We utilized (2020) two electronic databases (e.g., U.S. National Library of Medicine, National Institutes of Health [PubMed], and EMBASE) to identify studies of cervical schwannoma with ITH [ Table 1 ].

Table 1:

Previous reported cases of cervical schwannoma with bleed.

Literature review

We reviewed 13 cases from 13 articles regarding cervical ITHs attributed to spinal schwannomas, exclusive of our case. The following variables were studied: age, gender, predisposing factor, clinical presentation, size of the tumor, level of the tumor, hemorrhages, pathological tumor features, and outcomes (e.g., using the modified McCormick score).

Of the 13 previously reported cases of cervical schwannomas hemorrhages, spontaneous ITH were noted in five cases (38%), SAH in five cases (38%), and SDH with intratumoral bleed in two cases (15%), with one isolated case of SDH alone [ Tables 1, 2 ]. Patients averaged 51.77 years of age; our patient was 35 years old. There were eight males (61%) and five (39%) females; our patient was a female. Only four cases (30%) had attendant trauma/spinal manipulation as predisposing factors to the hemorrhage. Tumors involved an average of 2.83 levels. Histopathological findings in six cases showed a predominant Antoni A pattern, one case had an Antoni B pattern, while four had mixed patterns as in our case; in two cases, no pathological features were discussed. Outcomes were analyzed with the McCormick score; 12–13 cases showed significant improvement, while the outcome was not reviewed in one case.

Table 2:

Characteristics of study cohort of previous reported cases.

Case report

Case illustration

A 35-year-old female were presented with the sudden onset of quadriparesis over 1 week duration and 2 days of increased respiratory distress. On examination, she had a flaccid quadriparesis (motor 1/5 both upper extremities and 0/5 in both lower extremities) with a C7 sensory level and acute urinary retention. A contrast holo-neuraxis MRI showed a T1 hypointense, T2 heterogeneously hyperintense (e.g., with peripheral irregularity), and a non-enhancing central-large intradural extramedullary lesion from C2-T2 [ Figure 1a - c ]. Axial images showed extension into the right C2 neural foramen with widening/displacement of the spinal cord toward the left (e.g., suggestive of type III giant spinal schwannoma-Sridhar classification).[ 3 ] Gradient-recalled echo T2-weighted images additionally documented “blooming” in the lesion’s central region suggestive of blood degradation products.

Figure 1:

T2-weighted images of magnetic resonance imaging in sagittal (a), axial (b), and coronal (c) sections – a large mixed intensity intradural extramedullary spinal cord tumor from C2-D2 location and with significant compression of the spinal cord. A large brownish tumor anterolateral to the cord and pushing it to the left side (d), with intratumoral blood clots (e), and completely excised tumor (f).

Surgery

The patient underwent a C3-T3 Laminoplasty. After opening the dura, a giant dark brown subarachnoid mass was found attached to the right C2 nerve root and the spinal cord was displaced toward the left. The tumor was soft in consistency and accompanied by a lobulated dark-brown hemorrhage (e.g., with the altered thickness of blood clots of different ages). The tumor was easily suckable, and a good cleavage plane could develop circumferentially around the tumor, which was approximately 7.5 cm in size [ Figure 1d - f ]. The tumor was first internally decompressed under intraoperative monitoring in a piecemeal manner using the cavitron ultrasonic aspirator, finally facilitating gross total, en bloc removal.

Histology

The histological examination revealed a cellular schwannoma with mixed Antoni A and Antoni B cells and degenerative atypia; however, mitotic activity was not discernible [ Figure 2a - d ]. There were areas of hemorrhage and fibrin deposition. Sections were further subjected to immunohistochemistry, which showed strong and diffuse staining for S 100 [ Figure 2e ], while the EMA was negative [ Figure 2f ].

Figure 2:

Illustrated as – (a) Sections showing hypercellular Antoni Type A and hypocellular Antoni Type B areas (H and E, ×10), (b) with interspersed dilated, irregular shaped blood vessels (H and E, ×4), (c) cells are having oval to spindle shape nuclei, fibrillary cytoplasm with minimal atypia (H and E, ×40), (d) degenerative changes with smudgy chromatin, however, mitotic activity is not seen (H and E, ×40), (e) immunohistochemistry showing diffuse and strong S100 positivity and (f) While EMA is negative.

Postoperative course

The postoperative course was uneventful as the patient’s status improved; motor power became 3/5 in both upper and lower extremities, and her respiratory distress regressed. By the time of discharge, she could walk with one person’s support (e.g., motor power still 3–4/5). Her neurological status remained mostly unchanged at the 3 months follow-up (modified McCormick score III).

DISCUSSION

ITH occurring within the cervical spinal are very rare. We reviewed 13 prior cervical schwannomas, five of which were ITH. Of interest, cervical schwannomas equally present with ITH and SAH, while SDHs are rare. Outcomes of ITH within cervical schwannomas appear independent of size, age, or type of bleed, and preoperative neurological status.

The mechanism of hemorrhage in such tumors is variously reported as attributed to; ectatic hyalinized vessels undergoing spontaneous thrombosis[ 5 ] distal tumor necrosis, hemorrhage, endothelial proliferation in vascular lesions, and/or recanalization by meningeal vessels within the necrotic tumor, or traction along the spinal axis (e.g., on nerve roots’ vascular attachments).[ 2 , 3 , 5 ] The pathological factors have also been studied previously with different names as ancient schwannoma to cellular schwannoma, which has explained the degenerative and hemorrhagic nature of certain pathologically distinct schwannomas.[ 3 - 6 ]

CONCLUSION

The suspicion of an ITH within a cervical schwannoma must be considered when patients develop the acute onset of quadriparesis/plegia without an attendant history of coagulopathy, hypertension, or spinal manipulation/trauma. The histopathology is the final determinant of the lesion.

Declaration of patient consent

Patient’s consent not required as patients identity is not disclosed or compromised.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Hilton DA, Hanemann CO. Schwannomas and their pathogenesis. Brain Pathol. 2014. 24: 205-20

2. Jenkins AL, Ahuja A, Oliff AH, Sobotka S. Spinal schwannoma presenting due to torsion and hemorrhage: Case report and review of literature. Spine J. 2015. 15: e1-4

3. Jinnai T, Hoshimaru M, Koyama T. Clinical characteristics of spinal nerve sheath tumors: Analysis of 149 cases. Neurosurgery. 2005. 56: 510-5

4. Mills B, Marks PV, Nixon JM. Spinal subarachnoid hemorrhage from an ancient schwannoma of the cervical spine. Br J Neurosurg. 1993. 7: 557-9

5. Ng PY. Schwannoma of the cervical spine presenting with acute haemorrhage. J Clin Neurosci. 2001. 8: 277-8

6. Parmar H, Pang BC, Lim CC, Chng SM, Tan KK. Spinal schwannoma with acute subarachnoid hemorrhage: A diagnostic challenge. AJNR Am J Neuroradiol. 2004. 25: 846-50

7. Sridhar K, Ramamurthi R, Vasudevan MC, Ramamurthi B. Giant invasive spinal schwannomas: Definition and surgical management. J Neurosur. 2001. 94: 210-5