Serhan KÜPELİ, Bilgehan YALÇIN, Münevver BÜYÜKPAMUKÇU

İleri Evre Renal Hücreli Karsinomlu Pediatrik Vakanın Başarılı Tedavisi

Renal hücreli karsinomun çocukluk çağındaki tedavisi esas olarak geniş cerrahi rezeksiyon ile yapılmaktadır. Çoğu evre I-II vaka için radikal nefrektomi ve lenf nodu diseksiyonu yeterli olurken ileri evrelerde immünoterapinin de dahil olduğu farklı tedavi yöntemleri tanımlanmıştır. Burada tanı anında opere edilemeyen, sonrasında interferon alfa ile başarılı bir şekilde tedavi edilen ileri evre renal hücreli karsinomu olan 9 yaşında bir kız sunulmuştur.

Anahtar Kelimeler:

renal hücreli karsinom, tedavi, interferon alfa, çocuklar

Successful Treatment of a Child with Advanced Stage Renal Cell Carcinoma

Treatment of renal cell carcinoma (RCC) in children is based on an extensive surgery. Radical nephrectomy with lymph node dissection is sufficient for most of the stage I-II tumors. However, in advanced disease some different management strategies have been defined including immunotherapy. We present a 9-year-old girl with advanced stage RCC, inoperable at the time of diagnosis and successfully treated with interferon-alpha after operation.

Keywords:

renal cell carcinoma, treatment, interferon alpha, children,

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Age (yr.s)/sex Stage Chemotherapy Radiotherapy Interferon/ (mo.s) Outcome 2 3/M 4 ACT-D No Yes/? NED, 14 years 2 14/F 4 5-FU No Yes/? Died, 0.3 years 2 10/F 4 No No Yes/? Died, 5 years 4 9/M 3 No No Yes/72 NED, 7 years 4 6/M 3 No No Yes/36 Lung met., 3 years 5 13/F 3 V+ ACT-D No Yes/6 Died, 2 years 5 9/F 3 V No Yes/12 NED, 5 years Present case 9/F 3 Cis+Dac No Yes/36 NED, 5 years V: Vincristin, Cis+Dac: Cisplatin+Dacarbazine, ACT-D: Actinomycin-D, 5-FU: 5-Fluorouracil, NED: No evidence of disease, met: metastasis Since the patient tolerated IFN α well with a dose of 3 million IU/m 2 five days in a week, we administered it for one year, followed by 3 million IU/m 2 two times/week for another one year and once a week for the last year. Although the complete surgical resection has utmost importance in treatment, based on our experience on the presented case, adjuvant use of IFN α treatment can be considered in patients with locally advanced RCC who are under the risk of recurrence. Being a rare disease, the role of adjuvant therapies in pediatric RCC, including IFN α needs to be investigated in multicentric randomised studies. and a review of literature. J Surg Oncol. 2000; 75:266-9.
Androulakakis PA, Polychronopoulou-Androulakaki S, Michael V, Stephanidis A, Yannakis C. Renal cell carcinoma in children under 14 years old: long term survival. BJU International. 1999; 83:654-7.
Silberstein J, Grabowski J, Saltzstein SL, Kane CJ. Renal cell carcinoma in the pediatric population: Results from the California Cancer Registry. Pediatr Blood Cancer. 2009; 52:237-41.
Robson CJ, Churchill BM, Anderson W. The results of radical nephrectomy for renal cell carcinoma. J Urol. 1969; 101:297–301.
Otgün I, Arda IS, Haberal N, Güney H, Hiçsönmez A. Renal cell carcinoma: a case report and literature review. J Ped Surg. 2005; 40:13-6.
Selle B, Furtwängler R, Graf N, Kaatsch P, Bruder E, Leuschner I. Population-based study of renal cell carcinoma in children in Germany, 1980-2005: more frequently localized tumors and underlying disorders