Ces Urol 2008, 12(1):44-48 | DOI: 10.48095/cccu2008007

Primitive Neuroectodermal Tumour of the Kidney

Tomáš Urge¹, Milan Hora¹, Ondřej Hes², Marie Votavová³, Jan Bulka⁴, Jiří Ferda⁴, Eva Ferdová⁵, Tomáš Skalický⁶

¹ Urologická klinika LF a FN, Plzeň

² Šiklův patologickoanatomický ústav LF a FN, Plzeň

³ Onkologické a radioterapeutické oddělení LF a FN, Plzeň

⁴ Radiodiagnostická klinika LF UK a FN, Plzeň

⁵ Oddělení nukleární medicíny LF UK a FN, Plzeň

⁶ Chirurgická klinika LF UK a FN, Plzeň

Introduction:
Primitive neuroectodermal tumour (PNET) occurs primaly as a tumour of gastro-intestinal tract. He rises from neuroectodermal and is classed into group of small-cells (round-cell) tumours. Primary PNET of kidney is very rare tumour, witch is occured rather in younger ill, average age 28 years (4-69 years). Tumour was represented either as extrasceletal Ewing sarcoma (ES), with which has coincident to many imunohistochemical and cytogenetic features. Therapy of PNET is different from renal carcinoma, therefore exact histopathologic differential diagnosis with ussing imunohistochemical methods is necessary. We present case report of 42 years old patient with PNET of kidney.

Case report:
Patient was investigated for macroscopic hemauria with renal colics on the right. On CT was described 75 mm tumour in pars intermedia of right kidney (density 80HU). Wes provided transperitoneal nephrectomy for PNET (category pTlbNOMO). Despite of adjuvantn chemotherapy (5 series aspect, 7 series VAC) gets to disorder progression. In December 2006 was performed upper lobectomy and partial pleurektomie for implantation of PNET. Patient give 3 series palliative chemotherapy (hycamtine, cyklofosfamide). Unfortunately in April 2007 got to progression disorder in mediastinum and in pleurae. Inmate died in September 2007, behind 45,2 months from nephrectomy.

Conclusion:
Primary PNET kidney is very rare tumour, which by the help of imaging methods is not possible distinguish from other renal tumours. Surgical treatment is the same as in other renal tumours. Used adjuvant chemotherapy scheme is at present coincident with therapy ES. Appearances to small number of patient is not reserved a question of optimal treatment. Despite of the best standard therapy discuss of aggressive disorder with wrong prognosis.

Keywords: neuroectodermal, PNET, tumour

Received: February 12, 2008; Accepted: March 18, 2008; Published: January 1, 2008