- Department of Neurosurgery, Ehime University School of Medicine, Toon, Ehime, Japan
- Division of Diagnostic Pathology, Ehime University School of Medicine, Toon, Ehime, Japan
Correspondence Address:
Akihiro Inoue, Department of Neurosurgery, Ehime University School of Medicine, Toon, Ehime, Japan.
DOI:10.25259/SNI_993_2024
Copyright: © 2025 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: Ryo Inagaki1, Daisuke Kohno1, Seiji Shigekawa1, Akihiro Inoue1, Riko Kitazawa2, Takeharu Kunieda1. Radiological and pathological findings of spinal intramedullary granular cell tumor. 31-Jan-2025;16:28
How to cite this URL: Ryo Inagaki1, Daisuke Kohno1, Seiji Shigekawa1, Akihiro Inoue1, Riko Kitazawa2, Takeharu Kunieda1. Radiological and pathological findings of spinal intramedullary granular cell tumor. 31-Jan-2025;16:28. Available from: https://surgicalneurologyint.com/?post_type=surgicalint_articles&p=13357
Abstract
Background: Granular cell tumors (GCTs) are rare, usually benign, tumors with classic histomorphology. This tumor can occur throughout the body, but the spine is a distinctly rare location. Here, we report a very rare case of intramedullary GCT arising in the thoracic spinal cord.
Case Description: A 36-year-old woman presented to our hospital with an approximately 1-year history of gradually worsening numbness in the left toe and weakness in both lower limbs. Neuroimaging showed a tumor mass in the upper spine at the level of thoracic vertebrae 7-8, appearing hypointense on T2-weighted imaging (WI) and showing uniform gadolinium enhancement on T1-WI. Complete surgical resection was successfully performed. Histopathological examination revealed round or polygonal cells with abundant granular eosinophilic cytoplasm strongly staining for S-100 and SOX10, and benign intramedullary GCT in the thoracic spinal cord was diagnosed. Postoperative magnetic resonance imaging (MRI) showed no residual tumor, and the patient recovered well from this intervention, showing no sequelae. Follow-up neuroimaging after 2 years showed no signs of recurrence.
Conclusion: This report describes an extremely rare case of GCT arising from the intramedullary thoracic spinal cord, which is difficult to diagnose by routine neuroimaging. Therefore, accurate diagnosis requires careful identification of clinical signs, MRI including hypointensity on T2-WI, and analysis of combined morphologic and immunohistochemical studies.
Keywords: Granular cell tumor, Intramedullary spinal tumor, Pathological findings, Radiological findings, Thoracic spinal cord
INTRODUCTION
Granular cell tumor (GCT) is a rare neoplasm characterized by nests of polyhedral cells with abundant granular eosinophilic cytoplasm and was originally described as granular cell myoblastoma by Abrinkossoff in 1926.[
CASE DESCRIPTION
A 36-year-old woman presented to our department with an approximately 1-year history of gradually worsening numbness in the left toe and weakness in both lower limbs. She had no obvious history of trauma, congenital scoliosis, systemic diseases, or metabolic disorders. Neurological examination on admission revealed dysesthesia in the thoracic (Th)7-8 region and hyperreflexia in both lower limbs. Hemiparesis and marked numbness were present in both lower limbs, particularly on the left side. Decreased superficial sensation and paresthesia were evident in both lower limbs below the Th7 dermatome, and cysto-rectal dysfunction was also observed. Magnetic resonance imaging (MRI) of the spine revealed a well-defined intradural intramedullary lesion measuring 14 mm × 10 mm × 25 mm in the Th7-8 region that appeared ISO-to hypointense on T1-weighted imaging (WI) and hypointense on T2-WI, with uniform uptake on gadolinium (Gd)-enhanced T1-WI [
Figure 1:
(a-c) Preoperative magnetic resonance imaging (MRI) on initial admission shows a well-defined intradural intramedullary lesion in the region of thoracic vertebrae (Th)7-8. Lesions are hypointense on (a-1: sagittal view, a-2: axial view) T1-weighted imaging (WI) and hypointense on (b-1: sagittal view, b-2: axial view) T2-WI. The lesion appears enhanced following administration of (c-1: sagittal view, c-2: axial view) gadolinium (Gd). (d-1, d-2) Intraoperative findings reveal a soft, yellow lesion below the incision site. No adhesions are evident between the tumor and dura mater. (d-3) The tumor margin appears free of the spinal cord and is carefully dissected and removed. T, tumor.
Figure 2:
(a-1, a-2) a paraffin-embedded specimen from surgical biopsy shows round and polygonal cells with abundant granular eosinophilic cytoplasm and perivascular lymphocytic aggregates. Most nuclei are round to oval in appearance without evidence of cellular atypia and mitotic figures (hematoxylin and eosin staining). (b) This tumor shows slight positive staining for Ki-67 monoclonal antibody (MIB-1) (MIB-1 labeling index: 1.0%). Most tumor cells are immunoreactive for (c) S-100 and (d) SOX10 protein, but negative for (e) glial fibrillary acidic protein (GFAP) (3,3’diaminobenzidine staining). a-1, c, d, e: Magnification, x200; scale bar, 50 µm. a-2, b: Magnification, x400; scale bar, 100 µm.
DISCUSSION
Abrinkossoff first described GCT as granular cell myoblastoma and has been reported in a variety of anatomic sites, but very few cases in the spinal cord have been reported.[
Clinically, the median age of patients with intraspinal GCT is 23 years, with a typical age range of 13–49 years, and the vast majority of cases have been reported in females.[
GCTs are rare neoplasms derived from Schwann cells with characteristic pathological findings.[
Regarding the treatment of GCT in the spinal region, surgical resection has the most important role. The treatment advocated for GCT at more usual sites is excision with wide margins. While complete resection should be attempted, the recurrence rate is high after partial resection. Local recurrence is observed in more than 20% of cases with positive resection margins, even if the disease is benign. In general, radio- and chemotherapy are not recommended. [
CONCLUSION
This case is one of the few reported adult cases of spinal intramedullary GCT. Given the rarity of this pathology, a multidisciplinary approach is essential. At present, surgical resection of the tumor remains the logical approach to tumor management to maximize long-term survival. In this case, surgery was performed as early as possible, yielding favorable clinical outcomes. To the best of our knowledge, as of the time of writing, no such cases have been reported previously in which complete removal of a spinal intramedullary GCT was possible. Therefore, this report should prove invaluable in elucidating the importance of the surgical procedure for spinal intramedullary GCT. Further research and accumulation of cases are needed to understand the behavior of these tumors better, identify optimal treatment plans, and standardize immunohistochemical and imaging analyses for diagnosis.
Ethical approval
The Institutional Review Board approval is not required.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
Use of artificial intelligence (AI)-assisted technology for manuscript preparation
The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.
Disclaimer
The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.
References
1. Abrikossoff A. Uber Myome ausgehend von der quergestreiften Willkürlichen muskulatur. Virchows Arch Pathol Anat. 1926. 260: 215-33
2. Burton BJ, Kumar VG, Bradford R. Granular cell tumour of the spinal cord in patient with Rubenstein-Taybi syndrome: Case report. Br J Neurosurg. 1997. 11: 257-9
3. Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: Diagnostic criteria and clinic pathologic correlation. Am J Surg Pathol. 1998. 22: 779-94
4. Farhat L, Hodroj MH, Kawtharani S, Moussalem C, Annan TE, Assi H. Spinal intradural extramedullary granular cell tumor. Surg Neurol Int. 2022. 13: 496
5. Han F, Gao L, Wang Y, Jin Y, Lv Y, Yao Z. Clinical and imaging features of granular cell tumor of the neurohypophysis: A retrospective analysis. Medicine (Baltimore). 2018. 97: e9745
6. Kusakawa A, Inoue A, Nakamura Y, Nishida N, Fukushima M, Senba H. Clinical features and endoscopic findings of granular cell tumor of the sellar region: A case report and review of the literature. Surg Neurol Int. 2020. 11: 101
7. Lack EE, Worsham GF, Callihan MD, Crawford BE, Klappenbach S, Rowden G. Granular cell tumor: A clinicopathologic study of 110 patients. J Surg Oncol. 1980. 13: 301-16
8. Le BH, Boyer PJ, Lewis JE, Kapadia SB. Granular cell tumor: Immunohistochemical assessment of inhibin-alpha, protein gene product 9.5, S100 protein, CD68, and Ki-67 proliferative index with clinical correlation.. Arch Pathol Lab Med. 2004. 128: 771-5
9. Liu HL, Huang BY, Zhang MS, Wang HR, Qu YM, Yu CJ. Sellar and suprasellar granular cell tumor of neurohypophysis. Chin Med J (Engl). 2017. 130: 741-3
10. Neelon D, Lannan F, Childs J, editors. Granular cell tumor. StatPearls. Treasure Island, FL: StatPearls Publishing; 2024. p.
11. Ordóñes NG, Mackay B. Granular cell tumors: A review of the pathology and histogenesis. Ultrastruc Pathol. 1999. 23: 207-22
12. Ordóñez NG. Granular cell tumor: A review and update. Adv Anat Pathol. 1999. 6: 186-203
13. Qu J, Ma J, Luo L, Ai L, Li S, Dai J. Subdural granular cell tumor in thoracic vertebral canal. Neurol India. 2009. 57: 679-80
14. Takayama Y, Hasuo K, Takahashi N, Nishimiya M, Nonoshita T, Takita Y. Granular cell tumor presenting as an intradural extramedullary tumor. Clin Imaging. 2004. 28: 271-3
15. Thomas de Montpréville V, Dulmet EM. Granular cell tumours of the lower respiratory tract. Histopathology. 1995. 27: 257-62
16. Vaghasiya VL, Nasit JG, Parikh PA, Trivedi PP. Intradural spinal granular cell tumor. Asian J Neurosurg. 2014. 9: 96-8
17. Weinstein BJ, Arora T, Thompson LD. Intradural, extramedullary spinal cord granular cell tumor: A case report and clinicopathologic review of the literature. Neuropathology. 2010. 30: 621-6