İntradural ekstramedüller melanotik schwannom: Olgu sunumu

Melanotik schwannom nöroektodermal orjinli olup, schwann hücre stoplazmasının melanotik pigmentasyonu ile karakterizedir. Genellikle benign, yavaş büyüyen, kapsüllü ve nadir lezyonlardır. Bu çalışmada, iki aylık bel ve bacak ağrısı yakınması ile başvuran, nörolojik muayenesinde bilateral ayak dorsofl eksiyonu +4/5 motor kuvvetinde ve sol L5 dermatomunda hipoestezi olup lomber manyetik rezonans incelemesinde L4-L5 intradural ekstramedüller yerleşim gösteren kitle lezyonu saptanan 41 yaşında erkek hasta sunulmuştur. Embiryolojik gelişim esnasında nöral krestten göç eden hücreler melanositlere ve schwann hücrelerine dönüştüğü bilinmektedir. Tümoral dönüşüm gösteren schwann hücrelerinin melanin sentezleme özelliği kazandığına inanılmaktadır. Nadir görülen bu olgu ve manyetik rezonans görüntülemenin kesin ve erken tanıdaki önemi literatürler ışığında tartışılmıştır. Subtotal eksizyon yapılan olgulara radyoterapinin de kombine edilmesi önerilmektedir.

Intradural extramedullary melanotic schwannoma: Case report

Melanotic schwannomas are tumors of neural crest cells origin, characterized by cytoplasmic deposition of melanin. These tumors are rare and generally considered benign, capsulated. A 41- year old man presented with low-back and leg pain for two months. On neurological examination; his foot dorsofl exion muscle strenght was +4/5 (5/5) bilaterally and he had hemihypoesthesia on left L5 level .His magnetic resonance imaging scan revealed an intradural extramedullary lesion at the level of L4-L5. It is generally believed that, both schwann cells and melanocytes are derived from migrating neural crest cells. Perhaps schwann cells were changed to tumor have ability for synthesizing melanin. The rarity of this entity and the relevance of magnetic resonance imaging in the accurate and early diagnosis is discussed under the view of the literature. Radiotherapy may be necessary for subtotal resected tumor.

___

  • 1. Er U,Kazanci A,Eyriparmak T,Yigitkanli K,Senveli E:Melanotic schwannoma.J Clin Neurosci 2007;14:676-678
  • 2. Santaguida C, Sabbagh AJ, Guidot MC, Del Maestro RF:Agressive intramedullary melanotic shcwannoma. Neurosurgery 2004;55(6): 1430-1434.
  • 3. Bird CC, Willıs RA: The histogenesis of pigmented neurofibromas.J Pathol 1969; 97: 631-637.
  • 4. Carney JA, Stratakis CA:Epitheloid blue nevus and psammomataus melanotic schwannoma. Semin Diag.Pathol 1998;15(3): 216-24.
  • 5. Tawk RG, Fenstermaker RA:Melanotic schwannoma with drop metasteses to the caudal spine and high expression of CD 117(c-kit). J Neurooncol 2005;71(2): 151-156.
  • 6. Carney JA: Psammomatous melanotic schwannoma.A distinctive,heritable tumor with special associations,including cardiac myxoma and the cushing syndrome. Am J Surg Pathol 1990;14: 206-222.
  • 7. Goasgen O, Boucher E, Pouit B, Soulard R, Pernot P:Melanotic schwannoma, a tumor with a unpredictable prognosis: Case report and review of the literatüre. Neurochirurgie 2003; 49: 31-38.
  • 8. Miller WG:Malignant melanotic tumor of ganglion cells arising from the thoracic sympathetic ganglion. J Pathol 1932; 35: 351-357.
  • 9. Abbott AE, Murray GF:Melanotic schwannoma of sympathetic ganglia.Pathologic and clinical characteristics. Ann Thoracic Surg 1990; 49(6):1006-8.
  • 10. Carney JA:The Carney complex myxomas, spotty pigmentation, endocrine overactivity, and schwannomas. Dermatol Clin 1995;13: 19-26.
  • 11. Peltier J, Page C, Toussaint P, Bruniau A, Desenclos C, Le Gars D: Melanocytic schwannomas: Report of three cases. Neurochirurgie 2005 ;51:183-189.
  • 12. Wetson JA:The migration and differentiation of neural crest cells. Adv Morphogenesis 1970;8:41-42.
  • 13. Zhang Hang-Yong, Yang Guang Hua, Zhnag Yuan-Hnag:Clinicopathological,im munohistochemical, and ultrastructural study of 13 case with melanotic schwannoma. Chin Med J 2005 118:1451-1461.
  • 14. Killen RM, Davy CL, Bauserman SC: Melanocytic schwannoma. Cancer 1998;62: 174-183.
  • 15. Martin Reay D.G:Psammomatous melanotic shcwannoma Am J Clin Pathol 1991;95(4): 484-489.
  • 16. McGavran WL, Sypert GW, Ballinger WE:Melanotic Schwannoma. Neurosurgery 1978;2(1): 47-51.
  • 17. Utiger CA:Psammomatous melanotic schwannoma: A new cutaneous marker for Carney’s complex. Arch Dermatol 1993;129(2): 202-204.
  • 18. Mennemeyer RP, Bockus D. Melanotic schwannoma: Clinical and ultrastructural of studies three cases with evidece of intracellular melanin synthesis. Am J Surg Pathol 1979;3(1):3-10.
  • 19. Marchese MJ, Mc Donald JV:Intramedüllery melanotic schwannoma of the cervical spinal cord: Report of a case. Surg Neurol 1990;33: 353-355.
  • 20. Liessi G, Barbazza R, Sartori F, Sarbadin P, Scapenillo A: CT and MR imaging of melanocytic schwannomas: Report of three cases. Eur J Radiol 1990;11: 138-142.
  • 21. Lecam-savin C, Gontier MF, Martin A, Sevestre H, Cordonnier C, Samadja A:Spinal melanocytic schwannomas with maligant course:Microscopic and ultrasuctural study of a case. Arch Anat Cytol Pathol 1995; 43: 160-163.