Primitive Neuroectodermal Tumor of Kidney: success of neoadjuvant chemotherapy in an unresectable tumor?

Abstract

Objective: Primitive neuroectodermal tumors PNET of the kidney are very rare tumors with aggressive behavior. They usually are associated with poor prognosis. Case: A 26-year old male patient presented to our clinic with right flank pain, microscopic hematuria and moderate-heavy nausea complaints. CT imaging studies showed a tumor with 17x13x18 cm dimensions. A mass excision was not possible caused by tumor spread to liver surface and duodenum and the tumor could not be separated from these surfaces. The operation was finished with an excisional biopsy. The patient received 7 cycles of alternating IE/VAC chemotherapy sessions. At 5th month of follow-up, PET-CT scans showed that the tumor was regressed to 8x6x7 cm dimensions. Following this outcome, radical nephrectomy was performed without risking any other organs. Conclusion: Neoadjuvant chemotherapy can be employed prior to radical nephrectomy in such cases in order to radically/efficiently/ drastically reduce the tumor mass

Keywords

• neuroectodermal tumor,kidney,neoadjuvant chemotherapy

Böbreğin Primitif Nöroektodermal Tümörü: Rezektabl Olmayan Bir Tümörde Neoadjuvan Kemoterapinin Başarısı

Abstract

Amaç: Böbreğin primitif nöroektodermal tümörü oldukça seyrek ve agressif davranışlı ve kötü prognozludur. Olgu: 26 yaşında erkek hasta, hafif sağ yan ağrısı, mikroskopik hematüri ve hafif kusma yakınmaları ile kliniğimize refere edildi. BT de; 17X13X18 cm boyutlarında, sağ böbreği tümüyle saran ve sağ hipokondriumu dolduran bir tümör saptandı. Tümör, karaciğer yüzeyinden ve duodenumdan disseke edilemediği için eksize edilemedi, eksizyonel biyopsi yapılarak operasyon sonlandırıldı. IE/VAC alterne kemoterapi 7 siklus boyunca uygulandı. Tedavinin 5. ayında çekilen PET-CT de tümörün 8X6X7 cm boyutlarına küçüldüğü gözlendi. Herhangi bir komşu organa zarar verilmeden radikal nefrektomi yapıldı. Sonuç: Bu tip tümörlerde neoadjuvan kemoterapi, radikal nefrektomi öncesinde kitle küçültmek amaçlı olarak kullanılabilir.

Keywords

• nöroektodermal tümör,böbrek,neoadjuvan kemoterapi

References

1. Gupta RK, Soni SM, Joshi VV, Cartun R, Kumar A, Jain M.: Primary PNET of the kidney: report of two cases and review of literature. Indian J Cancer 2000;37: 184-9.
2. Palash KM, Supti M, Sravasti R, Nirmal KB.: PNET of kid- ney: Report of four cases. Indian J Med Paediatr Oncol 2012;33: 130-133 .
3. Kourda N, El Atat R, Derouiche A, Zeddini A, Chebil M, Baltagi S, Zermani R.: Primitive neuroectodermal tumor of the kidney with vena caval tumor thrombus: diagnosis and management. Pathologica 2007;99: 57-60.
4. Parham DM, Roloson GJ, Feely M, Green DM, Bridge JA, Beckwith JB.: Primary malignant neuroepithelial tumors of the kidney: a clinicopathologic analysis of 146 adult and pe- diatric cases from the National Wilms’ Tumor Study Group pathology Center. Am J Surg Pathol 2001;25: 133-46.
5. Gonsulen G, Ergin M, Paydaş S, et al.: Primitive neuro- ectodermal of the kidney: a rare entity. Int Urol Nephrol 2001;33: 449-451.
6. Thomas JC, Sebek BA, Krishnamurthi V.: Primitive neuro- ectodermal tumor of the kidney with inferior cava and atrial tumor thrombus. J Urol 2002;168: 1486-1487.