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Polyradiculopathies from schwannomatosis.

Jia Y, Kraus JA, Reddy H, Groff M, Wong ET - Open Neuroimag J (2011)

Bottom Line: There were multiple peripheral nerve tumors detected by magnetic resonance imaging (MRI) at the left vestibular nerve, cauda equina, right radial nerve, thoracic paraspinal nerve, and brachial plexi.Several resected tumors have features of schwannomas, including hypercellular Antoni A areas, hypocellular Antoni B areas, Verocay bodies, and hyalinized blood vessels.The specimens are also positive for immunohistochemical staining for INI1 with diffuse nuclear staining.

View Article: PubMed Central - PubMed

Affiliation: Division of Gerontology, Department of Medicine, Beth Israel Deaconess Medical Center.

ABSTRACT
We describe a case of schwannomatosis presenting as radicular pain and numbness in multiple radicular nerve distributions. There were multiple peripheral nerve tumors detected by magnetic resonance imaging (MRI) at the left vestibular nerve, cauda equina, right radial nerve, thoracic paraspinal nerve, and brachial plexi. Several resected tumors have features of schwannomas, including hypercellular Antoni A areas, hypocellular Antoni B areas, Verocay bodies, and hyalinized blood vessels. The specimens are also positive for immunohistochemical staining for INI1 with diffuse nuclear staining. The findings are consistent with sporadic form of schwannomatosis. This case highlights the importance of using MRI and INI1 immunohistochemistry to differentiate familial schwannomatosis, neurofibromatosis 2 (NF2)-associated schwannomatosis, and sporadic schwannomatosis.

No MeSH data available.


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Immunohistochemical staining for INI1 of the schwannoma removed from the patient’s right radial nerve. There is diffuse immunoperoxidase positivity in the nuclei. Original magnification X 200.
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Figure 5: Immunohistochemical staining for INI1 of the schwannoma removed from the patient’s right radial nerve. There is diffuse immunoperoxidase positivity in the nuclei. Original magnification X 200.

Mentions: A 49-year-old previously healthy Chinese man presented with radicular pain and numbness in the S1 distribution of the left leg. There was no bowel or bladder problem, saddle anesthesia, or weakness. He underwent a gadolinium-enhanced lumbosacral MRI that showed a 1.7 cm mass compressing the conus of the spinal cord at the level of T12 and L1 (Fig. 1A and 1B, arrows). There was another smaller enhancing mass, measuring 0.7 cm, at L4 (Fig. 1A, arrow). In order to remove mass effect exerted on the conus, he underwent a posterior laminectomy for the surgical removal of the mass at T12-L1. The histological feature of the mass was consistent with schwannoma, World Health Organization grade 1. There were hypercellular Antoni A areas (*), hypocellular Antoni B areas (**), Verocay bodies (arrows), and hyalinized blood vessels (arrowhead) (Fig. 2). Postoperatively, his radicular pain resolved but the S1 numbness remained. He had no family history of schwannoma. But because his past history was notable for removal of another schwannoma from the right superficial paraspinal region, this prompted further staging evaluations to look for schwannomas at other body sites. A head MRI revealed a gadolinium-enhancing mass at the cerebellopontine angle that infiltrates into the left internal auditory canal, measuring 2.7 cm in diameter, and the neuroradiographic features of this mass was consistent with a vestibular schwannoma (Fig. 3, arrow). There was also a palpable nodule in his right inner arm and compression of this nodule resulted in shooting pain radiating in the radial nerve distribution. An MRI of the brachial plexi showed a 2.0 cm enhancing mass involving the right radial nerve and multiple smaller gadolinium-enhancing lesions measuring 0.8 to 1.3 cm causing nodular thickening of the brachial plexi bilaterally (Fig. 4A and 4B, arrows). Because the right radial nerve pain limited his ability to do physical work using his dominant hand, he subsequently underwent surgical removal of this radial nerve mass. The histological feature of this mass was also consistent with schwannoma, and immunohistochemical staining for INI1 demonstrated diffuse nuclear positivity (Fig. 5). Taken together, the presence of multiple schwannomas in peripheral nerves and the diffuse INI1 immunohistochemical staining indicate that our subject has the sporadic form of schwannomatosis.


Polyradiculopathies from schwannomatosis.

Jia Y, Kraus JA, Reddy H, Groff M, Wong ET - Open Neuroimag J (2011)

Immunohistochemical staining for INI1 of the schwannoma removed from the patient’s right radial nerve. There is diffuse immunoperoxidase positivity in the nuclei. Original magnification X 200.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3106375&req=5

Figure 5: Immunohistochemical staining for INI1 of the schwannoma removed from the patient’s right radial nerve. There is diffuse immunoperoxidase positivity in the nuclei. Original magnification X 200.
Mentions: A 49-year-old previously healthy Chinese man presented with radicular pain and numbness in the S1 distribution of the left leg. There was no bowel or bladder problem, saddle anesthesia, or weakness. He underwent a gadolinium-enhanced lumbosacral MRI that showed a 1.7 cm mass compressing the conus of the spinal cord at the level of T12 and L1 (Fig. 1A and 1B, arrows). There was another smaller enhancing mass, measuring 0.7 cm, at L4 (Fig. 1A, arrow). In order to remove mass effect exerted on the conus, he underwent a posterior laminectomy for the surgical removal of the mass at T12-L1. The histological feature of the mass was consistent with schwannoma, World Health Organization grade 1. There were hypercellular Antoni A areas (*), hypocellular Antoni B areas (**), Verocay bodies (arrows), and hyalinized blood vessels (arrowhead) (Fig. 2). Postoperatively, his radicular pain resolved but the S1 numbness remained. He had no family history of schwannoma. But because his past history was notable for removal of another schwannoma from the right superficial paraspinal region, this prompted further staging evaluations to look for schwannomas at other body sites. A head MRI revealed a gadolinium-enhancing mass at the cerebellopontine angle that infiltrates into the left internal auditory canal, measuring 2.7 cm in diameter, and the neuroradiographic features of this mass was consistent with a vestibular schwannoma (Fig. 3, arrow). There was also a palpable nodule in his right inner arm and compression of this nodule resulted in shooting pain radiating in the radial nerve distribution. An MRI of the brachial plexi showed a 2.0 cm enhancing mass involving the right radial nerve and multiple smaller gadolinium-enhancing lesions measuring 0.8 to 1.3 cm causing nodular thickening of the brachial plexi bilaterally (Fig. 4A and 4B, arrows). Because the right radial nerve pain limited his ability to do physical work using his dominant hand, he subsequently underwent surgical removal of this radial nerve mass. The histological feature of this mass was also consistent with schwannoma, and immunohistochemical staining for INI1 demonstrated diffuse nuclear positivity (Fig. 5). Taken together, the presence of multiple schwannomas in peripheral nerves and the diffuse INI1 immunohistochemical staining indicate that our subject has the sporadic form of schwannomatosis.

Bottom Line: There were multiple peripheral nerve tumors detected by magnetic resonance imaging (MRI) at the left vestibular nerve, cauda equina, right radial nerve, thoracic paraspinal nerve, and brachial plexi.Several resected tumors have features of schwannomas, including hypercellular Antoni A areas, hypocellular Antoni B areas, Verocay bodies, and hyalinized blood vessels.The specimens are also positive for immunohistochemical staining for INI1 with diffuse nuclear staining.

View Article: PubMed Central - PubMed

Affiliation: Division of Gerontology, Department of Medicine, Beth Israel Deaconess Medical Center.

ABSTRACT
We describe a case of schwannomatosis presenting as radicular pain and numbness in multiple radicular nerve distributions. There were multiple peripheral nerve tumors detected by magnetic resonance imaging (MRI) at the left vestibular nerve, cauda equina, right radial nerve, thoracic paraspinal nerve, and brachial plexi. Several resected tumors have features of schwannomas, including hypercellular Antoni A areas, hypocellular Antoni B areas, Verocay bodies, and hyalinized blood vessels. The specimens are also positive for immunohistochemical staining for INI1 with diffuse nuclear staining. The findings are consistent with sporadic form of schwannomatosis. This case highlights the importance of using MRI and INI1 immunohistochemistry to differentiate familial schwannomatosis, neurofibromatosis 2 (NF2)-associated schwannomatosis, and sporadic schwannomatosis.

No MeSH data available.


Related in: MedlinePlus