J Korean Neurosurg Soc.  2016 Mar;59(2):158-160. 10.3340/jkns.2016.59.2.158.

A Rare Case of Concomitant Intramedullary Gangliocytoma at the Cervicomedullary Junction in Patient with Neuroendocrine Tumor of Lung

• Aydemir F
• Cekinmez M
• Kardes O
• Kayaselcuk F

• KMID: 2192047
• DOI: http://doi.org/10.3340/jkns.2016.59.2.158

Abstract

Ganglion cell tumors (GCT) are divided into two subtypes : gangliocytoma and ganglioglioma. Intramedullary gangliocytomas are extremely rare. A 20-year-old male patient with pain of neck, who also had a previously known neuroendocrine tumor of lung, was operated for mass found in the cervicomedullary junction with a presumptive diagnosis of metastases. Only partial resection could be performed. Pathological diagnosis had been reported as gangliocytoma. Only ten cases of intramedullary gangliocytoma have been reported in the literature. Although association with scoliosis and Von Recklinghausen's disease were previously reported in the literature, no gangliocytoma case concomitant with endocrine tumor of lung have been published. Pathological study is the most important diagnostic method for gangliocytomas. Surgical excision is the primary treatment, but difficulty in total surgical tumor resection is the most important problem.

Keyword

Gangliocytoma; Intramedullary; Tumor

MeSH Terms

Diagnosis
Ganglioglioma
Ganglion Cysts
Ganglioneuroma*
Humans
Lung*
Male
Neck
Neoplasm Metastasis
Neuroendocrine Tumors*
Neurofibromatosis 1
Scoliosis
Young Adult

Figure

• 

  Fig. 1 Diffuse cord edema extending from medulla oblongata to C6 level and cystic component are seen on sagittal T1 (A) and T2 (B) weighted MR images. Intense contrast-enhancing lesion approximately 44×14 mm in size extending from medulla oblongata to C2 level on post-contrast sagittal T1 (C) and axial T1 (D) weighted MR images.

• 

  Fig. 2 Expansion of the cord is observed at cervicomedullary junction following dural opening (A), tumor cavity is seen after partial resection (B).

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  Fig. 3 Neuroendocrine tumor of lung H&E ×100, inlet : closer view of tumor cells H&E ×400 (A), neoplastic development in ganglion cells H&E ×100, inlet : S-100 positive staining ganglion cells, original magnification H&E ×400 (B).

Reference

1. Azzarelli B, Luerssen TG, Wolfe TM. Intramedullary secretory gangliocytoma. Acta Neuropathol. 1991; 82:402–407. PMID: 1767634.

2. Baehring J, Ogle E, Sze G, Duncan C, Bannykh S. Ganglioneurocytoma of the spinal cord. J Neurooncol. 2005; 71:149. PMID: 15690130.

3. Choi YH, Kim IO, Cheon JE, Kim WS, Yeon KM, Wang KC, et al. Gangliocytoma of the spinal cord : a case report. Pediatr Radiol. 2001; 31:377–380. PMID: 11373931.

4. Coca S, Moreno M, Martos JA, Rodriguez J, Barcena A, Vaquero J. Neurocytoma of spinal cord. Acta Neuropathol. 1994; 87:537–540. PMID: 8059608.

5. Jacob JT, Cohen-Gadol AA, Scheithauer BW, Krauss WE. Intramedullary spinal cord gangliocytoma : case report and a review of the literature. Neurosurg Rev. 2005; 28:326–329. PMID: 15739067.

6. Kalyanaraman UP, Henderson JP. Intramedullary ganglioneuroma of spinal cord : a clinicopathologic study. Hum Pathol. 1982; 13:952–955. PMID: 7129414.

7. Komotar RJ, O'Toole JE, Mocco J, Khandji AG, Keller CE, Connolly ES Jr, et al. Gangliocytoma of the spinal cord. Neurosurgery. 2007; 60:895–900. discussion 895-900PMID: 17460525.

8. Kwon JW, Kim IO, Cheon JE, Kim WS, Chi JG, Wang KC, et al. Cerebellopontine angle ganglioglioma : MR findings. AJNR Am J Neuroradiol. 2001; 22:1377–1379. PMID: 11498430.

9. Lagares A, Gómez PA, Lobato RD, Ricoy JR, Ramos A, de la Lama A. Ganglioglioma of the brainstem : report of three cases and review of the literature. Surg Neurol. 2001; 56:315–322. discussion 322-324PMID: 11750003.

10. Milligan BD, Giannini C, Link MJ. Ganglioglioma in the cerebellopontine angle in a child. Case report and review of the literature. J Neurosurg. 2007; 107(4 Suppl):292–296. PMID: 17941493.

11. Ng TH, Fung CF, Goh W, Wong VC. Ganglioneuroma of the spinal cord. Surg Neurol. 1991; 35:147–151. PMID: 1990483.

12. Rosenblum MK. Central nervous system. In : Rosai J, editor. Rosai and Ackerman's Surgical Pathology. ed 9. Edinburgh: Mosby;2004. p. 2461–2622.

13. Russo CP, Katz DS, Corona RJ Jr, Winfield JA. Gangliocytoma of the cervicothoracic spinal cord. AJNR Am J Neuroradiol. 1995; 16(4 Suppl):889–891. PMID: 7611066.

14. Shin JJ, Oh SH, Yoon DH, Kim TS. Cervical ganglioglioma. J Korean Neurosurg Soc. 2001; 30:239–243.

15. Wakao N, Imagama S. Intramedullary gangliocytoma with calcification and multiple intramedullary cysts. Neuroradiology. 2012; 54:893–895. PMID: 22538840.

16. Yang C, Li G, Fang J, Wu L, Yang T, Deng X, et al. Intramedullary gangliogliomas : clinical features, surgical outcomes, and neuropathic scoliosis. J Neurooncol. 2014; 116:135–143. PMID: 24132615.