[Severe bilateral hydronephrosis in a 12 years old boy with neurofibromatosis type-1].
The large neurofibromatic tumours developing in head, neck and abdominal cavity in children with neurofibromatosis type 1 make a serious clinical problem. In this report the case of 12 years old boy with benign abdominal tumor leading from nerve roots (S1-S2) is presented. He has been treated for 6 years. The other malignant neoplasms, additional congenital defects or neurological dysfunctions were not confirmed. The primary resection was not radical and adjuvant therapy (chemotherapy, hormonotherapy) was ineffective. Due to the progression and not coming to hospital in next 2 years severe obstructive uropathy developed leading to complete destruction of the left kidney. Bilateral ureterocutaneostomies were performed. Despite of slow tumor grow the patient is stable now. However he needs regular calibrations of the right ureterocutaneostomy due to the progressive contraction. Further treatment of this case remains open question.
Sitkiewicz
Anna
A
Klinika Chirurgii i Onkologii Dzieciecej Instytutu Pediatrii Akademii Medycznej w Łodzi.
Przewratil
Przemysław
P
Andrzejewska
Ewa
E
D000008:Abdominal Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D002648:Child; D006801:Humans; D006869:Hydronephrosis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000628:therapy; D014519:Ureterostomy