Symmetric dumbbell ganglioneuroma of bilateral C1 roots with intradural extension associated with von recklinghausen's disease: A case presentation Von recklink housen hastalig̈i'na eşlik eden i̇ntradural simetrik bilateral C1 köklerinden kaynaklanan ganglionöroma; vaka sunumu
Journal of Neurological Sciences, cilt.28, sa.1, ss.123-128, 2011 (SCI-Expanded, Scopus, TRDizin)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 28 Sayı: 1
- Basım Tarihi: 2011
- Dergi Adı: Journal of Neurological Sciences
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, TR DİZİN (ULAKBİM)
- Sayfa Sayıları: ss.123-128
- Anahtar Kelimeler: Cervical spine, Dumbbell tumor, Ganglioneuroma, Von recklinghausen's disease
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
Introduction: Ganglioneuromas are rare benign tumours arising most commonly from the sympathetic nervous system. They occasionally grow in a dumbbell fashion extending into the spinal canal extradurally. But, ganglioneuromas in association with von Recklinghausen's disease are rare. Case presentation: A 45 year old male, was admitted to emergency unit with complaints of pain in both of arms and neck, progressive difficulty of swallowing and respiratory distress. A lots of cafe-au-lait and small subcutaneous noduls were seen on whole body and two fregling in axillary region were detected on physical examination. A moderate quadriparesis and IX-X cranial nerve dysfunction was observed on neurological examination. Cranio-cervical region magnetic resonance imaging revealed a bilaterally and symmetric mass lesion located in C1 region, spreaded to intra and extradural space like as known as "dumbbell shape". The patient was undergone surgery and tumour was totally removed with microsurgical technique via posterior approach. Ganglioneuroma was histopathologically verified. Postoperative period was uneventful and the patient was discharged as neurologically intact. Magnetic resonance imaging performed three months after surgery proved the total resection of tumour. Discussion: Ganglioneuromas are a subgroup of neuroblastic tumors that have been defined as childhood embrional tumors of migrating neuroectodermal cells derived from the neural crest. Ganglioneuromas originating within spinal canal constitute less than 10 percent of all ganglioneuromas. Furthermore involvement of cervical spine is extremely rare. Total surgical removal of tumour is usually curative and satisfactory. Patient must be follow in neurointensive care unit because the threat of respiratory distress and swallowing difficulty in early postoperative period. Neurological symptoms usually disappear gradually.