- Department of Pathology and Laboratory Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
- Department of Hematology and Oncology, American University of Beirut Medical Center, Beirut, Lebanon.
- Department of Neurosurgery, American University of Beirut Medical Center, Beirut, Lebanon.
- Department of Radiology, American University of Beirut Medical Center, Beirut, Lebanon.
Sara Sinno, Department of Pathology and Laboratory Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
DOI:10.25259/SNI_902_2022Copyright: © 2022 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Lama Farhat1, Mohammad Hassan Hodroj2, Sarah Kawtharani3, Charbel Moussalem3, Tamara El Annan4, Hazem Assi2, Houssein Darwish3, Sara Sinno1. Spinal intradural extramedullary granular cell tumor. 28-Oct-2022;13:496
How to cite this URL: Lama Farhat1, Mohammad Hassan Hodroj2, Sarah Kawtharani3, Charbel Moussalem3, Tamara El Annan4, Hazem Assi2, Houssein Darwish3, Sara Sinno1. Spinal intradural extramedullary granular cell tumor. 28-Oct-2022;13:496. Available from: https://surgicalneurologyint.com/surgicalint-articles/11958/
Background: Granular cell tumors (GCTs) are uncommon peripheral nerve sheath tumors of Schwann cell origin that may occur throughout the body. However, they rarely occur in the spinal canal.
Case Description: A 49-year-old male presented with burning sensation in the left knee. The MRI of the lumbar spine showed an L3-L4 intradural extramedullary tumor. Complete surgical resection was successfully performed and the L3 root burning improved. Histopathologically, the lesion proved to be a benign GCT.
Conclusion: Spinal GCTs are rare benign tumors that may be found in an intradural extramedullary location in the spine. The preferred treatment is complete surgical resection as subtotal/partial resection may result in recurrence warranting radiation therapy.
Keywords: Granular cell tumor, Intradural extramedullary, Intramedullary, Nerve sheath tumor, Spinal canal
Granular cell tumors (GCTs) are uncommon soft-tissue tumors that were initially described as granular cell myoblastoma by Abrinkossoff in 1926.[
A 49-year-old male presented with burning sensation and pain in the left knee for 2 years’ duration. The pain was severe and refractory to pain medications. The lumbar MRI showed a well-circumscribed soft-tissue lesion in the left L3-L4 neural foramen along the course of the left L3 nerve root. It was isointense to hypointense on T1W, hypointense on T2W images [
Under fluoroscopic guidance, a bilateral L3-L4 laminectomy was performed. The pedicles of L3 and L4 were exposed along with the transverse processes. The traversing L3 nerve root was identified and the tumor was completely dissected and resected off the root. Postoperatively, the patient sustained improvement in the burning sensation and exhibited no new motor deficit.
The histological examination was consistent with a benign GCT. It revealed polygonal tumor cells with abundant, eosinophilic, and granular cytoplasm arranged in sheets [
Spinal granular cell tumor. (a) The tumor is composed of sheets of polygonal tumor cells with abundant eosinophilic granular cytoplasm (Hematoxylin and Eosin, original magnification ×20). (b-d) The tumor cells are diffusely positive for S-100, SOX-10, and inhibin (original magnification ×20).
GCTs are peripheral nerve sheath tumors that show neuroectodermal differentiation.[
The majority of the reported cases of spinal GCTs have an intradural extramedullary location. The differential diagnosis for tumors in this location includes neurofibroma, schwannoma, meningioma, paraganglioma, epidermoid cyst, intradural dissemination, and, in the cauda equina region, myxopapillary ependymoma. Those tumors can be difficult to distinguish from GCTs on MRI.[
Three spinal GCTs were described as being intramedullary.[
The vast majority of GCTs behave in a benign fashion; however, they may recur if incompletely excised.[
GCTs are peripheral nerve sheath tumors that rarely occur in the spinal canal where they are usually intradural/ extramedullary in location. The optimal treatment is gross total surgical excision, as subtotal/partial removal may result in recurrence and the need for adjuvant radiation therapy.
Patient’s consent not required as patient’s identity is not disclosed or compromised.
There are no conflicts of interest.
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