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Malign periferik sinir kılıfı tümörü: Olgu Sunumu

Year 2014, Volume: 22 Issue: 3, 114 - 116, 01.12.2014

Abstract

Giriş: Malign periferik sinir kılıfı tümörleri (MPSKT) terimi iğsi hücreli sarkom olup; periferik sinir tümörleri, nörofibromlar veya sinir kılıfından gelişir. En sık ekstremiteler ve gövdedeki büyük sinirlerini tutar. Agresif tümörlerdir ve yumuşak doku sarkomlarının yaklaşık %10’luk kısmını oluştururlar. Klinik olarak büyük boyutlu kitleler olarak karşımıza çıkar. Kitle bölgesinde ağrı ve sinir tutulumuna bağlı, sinir fonksiyon değişikliği eşlik edebilir. Olgu: Doksan bir yaşında bayan, sol ön kol anterolateralinde kitle nedeniyle kliniğimize başvurdu. Muayenede 8x4x5 cm boyutunda, mobil sert kitle mevcuttu. Klinik bir şikayeti yoktu ve muayenede sinir hassasiyeti ve defisiti yoktu. Kitle total eksize edildi. Bulgular: Makroskobik olarak 8x6x6 cm ölçülerindeydi. Mikroskobik olarak tümör dokusunda yaygın koagülasyon nekrozu mevcuttu. Kitle patolojik olarak malign periferik sinir kılıfı tümörü olarak raporlandı. Tartışma: Sporadik olarak gelişebilen, daha çok NF-1’li hastalarda oluşan malign periferik sinir kılıfı tümörleri ayrıca radyoterapi alımı sonrası da oluşabilmektedir. Agresif bir tümördür, lokal nüks oranı yaklaşık %40, metastaz oranı ise %50’dir. Hastalar sıklıkla bölgesel ağrı ve sinir fonksiyon azalması veya kaybı ile doktora başvururlar ancak bizim olgumuzda başvuru şikayeti kitlesel görünümdü ve bölgesel ağrı şikayeti mevcut değildi. Muayenede sinir fonksiyonları doğaldı. Sonuç: İleri yaşta, özellikle ekstremite ve gövdede dev kitle nedeni ile başvurulduğunda, malign periferik sinir kılıfı tümörü olabileceği akıldan çıkarılmamalıdır. Anahtar sözcükler: Malign periferik sinir kılıfı tümörü, nörofibromatozis, sarkom.

References

  • Canda M. Şerefettin. Periferik sinir kılıfı tümörleri. Türkiye Ekopa- 1.
  • toloji Dergisi 2004; 10 (1-2): 65-74.
  • Enzinger FM, Weiss SW. Malignant Tumors of the Peripheral 2.
  • Nevre. Soft Tissue Tumors. St louis: Mosby-Year Book, 1995; 889- 928.
  • Woodruff JM. Pathology of tumors of the peripheral nevre 3.
  • sheath in type 1 neurofibromatosis. Am J Med Genet 1999; 89: 23-30-18.
  • Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup 4.
  • DM. Malignant peripheral nerve sheath tumors. A clinicopatho
  • logic study of 120 cases. Cancer. 1986; 57:2006-2021.
  • long-term follow-up of von Recklinghausen neurofibromatosis. 5.
  • Survival and malignant neoplasms. Sİrensen SA, Mulvihill JJ,
  • Nielsen A. N Engl J Med 1986; 314:1010-5.
  • BS Ducatman, BW Scheithauer, DG Piepgras, HM Reiman. Ma- 6.
  • lignant Peripheral Nerve Sheath Tumors. A Clinicopathologic
  • Study of 120 Cases. Cancer, 1986, 57:2006-21.
  • Kim DH, Murovic JA, Tiel Rl, Moes G, Kline DG, A series of 397 7.
  • peripheral neural sheath tumors: 30-year experience at loui
  • siana State University Health Sciences Center. J Neurosurg. 2005;102(2):246.
  • lantos Pl, louis DN, Rosnblum MK, Kleihues P. Tumors of the 8.
  • Nervous System. in: Graham DI, lantos Pl, eds. Greenfield’s Neu
  • ropathology. london: Arnold, 2002; 906
  • Baehring JM, Betensky RA, Batchelor TT. Malignant peripheral 9.
  • nerve sheath tumor: the clinical spectrum and outcome of treat
  • ment. Neurology. 2003;61(5):696. 10. Kchouk M, Rabet AM, Ghedas K, et al. Extensive malignant
  • schwannoma of the sciatic nerve. Contribution of imaging tech
  • niques [in French]. J Radiol 1993;74:641–4. 11. Kargi E, Aslan G, Senen D, Erdogan B, Albayrak l. Primary malig
  • nant triton tumor of the foot. Eur J Plast Surg (2002) 24: 397–9. 12. Carli M, Ferrari A, Mattke A, et al. Pediatric malignant peripheral
  • nerve sheath tumor: The Italian and German soft tissue sarcoma
  • cooperative group. J Clin Oncol 2005;23:8422-30. 13. lamichhane B, Yogi N, Paudel GR, Panth R, Devkota UP, Gongal
  • DN. large Malignant Peripheral Nerve Sheath Tumor of lum
  • bar spine: A Case Report. Nepal Journal of Medical Sciences
  • ;2(2):190-3. 14. RH Hruba, MH Shiu, RT Senie, JM Woodruff. Malignant periph
  • eral nerve sheath tumors of the buttock and lower extremity. A
  • study of 43 cases. Cancer, 1990;15:1253–65.
  • mörü olabileceği akıldan çıkarılmamalıdır.
  • Dr. Mehmet Haşim Güner
  • Zonguldak Üniversitesi Tıp Fakültesi,
  • Plastik, Rekonstrüktif ve Estetik Cerrahi AD, ZONGUlDAK
  • E-posta: dr_mhguner@yahoo.com

Malignant peripheral nerve sheath tumor: A Case Report

Year 2014, Volume: 22 Issue: 3, 114 - 116, 01.12.2014

Abstract

Introduction: Malignant peripheral nerve sheath tumors (MPNSTs’) are spindle cell sarcomas and they arise from the peripheral nerve tumors, neurofibromas and nerve sheaths differentiation. They are commonly seen large nerves on extremities and trunk. MPNSTs’ are highly aggressive tumors which appear approximately 10% of all soft tissue sarcomas. They present as an enlarging mass and often associated with pain and nerve deficit. In accordance with the literature, the mass location is left front arm to our case. Case report: Ninety one year-old woman, referred to our clinic with mass which had been localized on the left antero-lateral forearm. Upon examination, 8x4x5 cm sized, mobile and solid mass evaluated .The patient had not any compliant about pain and nerve defiency. The mass and skin excision was performed and primer closure was applied for defect. Findings: The mass was 8x6x6 cm sized macroscopically. Microscopically, wide spread coagulation necrosis was determined and pathologic diagnosis was reported as malignant peripheral nerve sheath tumor. Discussion: MPNSTs’ were usually developed sporadically also seen related to radiotherapy or neurofibromatosis type 1. Furthermore, these aggressive tumors have %40 ratio of local recurrence and %50 ratio of metastasis. The patients, who have MPNST, usually presented with complaints about local pain and deficit of specific nerve function. Conversely, our case has not any compliant about pain and nerve dysfunction. Conclusion: Definitive diagnosis of extremity and trunk mass in elder patients, MPNSTs’ may be kept in mind even without pain and nerve deficit. Keywords: Malignant peripheral nerve sheath tumor, neurofibromatosis, sarcoma.

References

  • Canda M. Şerefettin. Periferik sinir kılıfı tümörleri. Türkiye Ekopa- 1.
  • toloji Dergisi 2004; 10 (1-2): 65-74.
  • Enzinger FM, Weiss SW. Malignant Tumors of the Peripheral 2.
  • Nevre. Soft Tissue Tumors. St louis: Mosby-Year Book, 1995; 889- 928.
  • Woodruff JM. Pathology of tumors of the peripheral nevre 3.
  • sheath in type 1 neurofibromatosis. Am J Med Genet 1999; 89: 23-30-18.
  • Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup 4.
  • DM. Malignant peripheral nerve sheath tumors. A clinicopatho
  • logic study of 120 cases. Cancer. 1986; 57:2006-2021.
  • long-term follow-up of von Recklinghausen neurofibromatosis. 5.
  • Survival and malignant neoplasms. Sİrensen SA, Mulvihill JJ,
  • Nielsen A. N Engl J Med 1986; 314:1010-5.
  • BS Ducatman, BW Scheithauer, DG Piepgras, HM Reiman. Ma- 6.
  • lignant Peripheral Nerve Sheath Tumors. A Clinicopathologic
  • Study of 120 Cases. Cancer, 1986, 57:2006-21.
  • Kim DH, Murovic JA, Tiel Rl, Moes G, Kline DG, A series of 397 7.
  • peripheral neural sheath tumors: 30-year experience at loui
  • siana State University Health Sciences Center. J Neurosurg. 2005;102(2):246.
  • lantos Pl, louis DN, Rosnblum MK, Kleihues P. Tumors of the 8.
  • Nervous System. in: Graham DI, lantos Pl, eds. Greenfield’s Neu
  • ropathology. london: Arnold, 2002; 906
  • Baehring JM, Betensky RA, Batchelor TT. Malignant peripheral 9.
  • nerve sheath tumor: the clinical spectrum and outcome of treat
  • ment. Neurology. 2003;61(5):696. 10. Kchouk M, Rabet AM, Ghedas K, et al. Extensive malignant
  • schwannoma of the sciatic nerve. Contribution of imaging tech
  • niques [in French]. J Radiol 1993;74:641–4. 11. Kargi E, Aslan G, Senen D, Erdogan B, Albayrak l. Primary malig
  • nant triton tumor of the foot. Eur J Plast Surg (2002) 24: 397–9. 12. Carli M, Ferrari A, Mattke A, et al. Pediatric malignant peripheral
  • nerve sheath tumor: The Italian and German soft tissue sarcoma
  • cooperative group. J Clin Oncol 2005;23:8422-30. 13. lamichhane B, Yogi N, Paudel GR, Panth R, Devkota UP, Gongal
  • DN. large Malignant Peripheral Nerve Sheath Tumor of lum
  • bar spine: A Case Report. Nepal Journal of Medical Sciences
  • ;2(2):190-3. 14. RH Hruba, MH Shiu, RT Senie, JM Woodruff. Malignant periph
  • eral nerve sheath tumors of the buttock and lower extremity. A
  • study of 43 cases. Cancer, 1990;15:1253–65.
  • mörü olabileceği akıldan çıkarılmamalıdır.
  • Dr. Mehmet Haşim Güner
  • Zonguldak Üniversitesi Tıp Fakültesi,
  • Plastik, Rekonstrüktif ve Estetik Cerrahi AD, ZONGUlDAK
  • E-posta: dr_mhguner@yahoo.com
There are 39 citations in total.

Details

Other ID JA59CS37TM
Journal Section Articles
Authors

Mehmet Haşim Güner This is me

Tahsin Görgülü This is me

Abdülkerim Olğun This is me

Ayhan Buz This is me

Publication Date December 1, 2014
Published in Issue Year 2014 Volume: 22 Issue: 3

Cite

APA Güner, M. H., Görgülü, T., Olğun, A., Buz, A. (2014). Malign periferik sinir kılıfı tümörü: Olgu Sunumu. Türk Plastik Rekonstrüktif Ve Estetik Cerrahi Dergisi, 22(3), 114-116.
AMA Güner MH, Görgülü T, Olğun A, Buz A. Malign periferik sinir kılıfı tümörü: Olgu Sunumu. turkplastsurg. December 2014;22(3):114-116.
Chicago Güner, Mehmet Haşim, Tahsin Görgülü, Abdülkerim Olğun, and Ayhan Buz. “Malign Periferik Sinir kılıfı tümörü: Olgu Sunumu”. Türk Plastik Rekonstrüktif Ve Estetik Cerrahi Dergisi 22, no. 3 (December 2014): 114-16.
EndNote Güner MH, Görgülü T, Olğun A, Buz A (December 1, 2014) Malign periferik sinir kılıfı tümörü: Olgu Sunumu. Türk Plastik Rekonstrüktif Ve Estetik Cerrahi Dergisi 22 3 114–116.
IEEE M. H. Güner, T. Görgülü, A. Olğun, and A. Buz, “Malign periferik sinir kılıfı tümörü: Olgu Sunumu”, turkplastsurg, vol. 22, no. 3, pp. 114–116, 2014.
ISNAD Güner, Mehmet Haşim et al. “Malign Periferik Sinir kılıfı tümörü: Olgu Sunumu”. Türk Plastik Rekonstrüktif Ve Estetik Cerrahi Dergisi 22/3 (December 2014), 114-116.
JAMA Güner MH, Görgülü T, Olğun A, Buz A. Malign periferik sinir kılıfı tümörü: Olgu Sunumu. turkplastsurg. 2014;22:114–116.
MLA Güner, Mehmet Haşim et al. “Malign Periferik Sinir kılıfı tümörü: Olgu Sunumu”. Türk Plastik Rekonstrüktif Ve Estetik Cerrahi Dergisi, vol. 22, no. 3, 2014, pp. 114-6.
Vancouver Güner MH, Görgülü T, Olğun A, Buz A. Malign periferik sinir kılıfı tümörü: Olgu Sunumu. turkplastsurg. 2014;22(3):114-6.