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Retroperitoneal neuroblastomas - a single institution experience in 90 patients
ESPU Meeting 2007 an initial screening procedure, bladderrenal ultrasonography showed a bladder lesion. Cystoscopy was performed under general anesthesia.
RESULTS A single papillary lesion was observed near the ureteral orefice in all cases. Size ranged from 1.5 to 2 cm. All patients underwent transurethral resection, and superficial biopsies were taken . Pathological grading, using the WHO/ISUP 2004 Classification, revealed
S43 4 urothelial papillomas and 3 papillary urothelial neoplasms of low malignant potential (PUNLMPs). All were superficial to the lamina propria. At follow-up urinalysis and urine cytology were checked monthly, bladder ultrasonography every three months and cystoscopy at six and twelve months for the first year after surgery. Subsequently urinalysis and urine cytology were done every three months, bladder ultrasound every six months and cystoscopy every year. At an average follow-up of 35
months (range 6 to 84 months) no recurrence was detected.
CONCLUSIONS Transitional cell carcinoma of the bladder is rare in children, and its long term prognosis is still unknown. Routine follow-up with urinary cytology and bladder ultrasound is adeguate. Cystoscopy can be reserved for patients with high grade or potentially highly malignant lesions. Multicenter protocols are required.
# S07-7 (O)
RETROPERITONEAL NEUROBLASTOMAS - A SINGLE INSTITUTION EXPERIENCE IN 90 PATIENTS ¨ DER, Ilka WIEMER*, Gerald TRABOLD*, Raimund STEIN, Annette SCHRO ¨ Joachim W. THUROFF and Peter GUTJAHR* Johannes Gutenberg University, Department of Urology, Mainz, GERMANY - * Johannes Gutenberg University, Department of Pediatrics, Mainz, GERMANY
PURPOSE Nowadays neuroblastomas are detected incidentally by ultrasound in early stages. Primary surgery, chemotherapy and/or radiotherapy are treatment options. However, the approach in babies is controversial. A surveillance strategy is propagated by some groups. In this retrospective study, we evaluated age and stage at diagnosis, therapy and outcome of patients treated at our institutions since 1970.
MATERIAL AND METHODS 90 patients with retroperitoneal neuroblastomas were treated. They were divided into 2 groups according to age at time of diagnosis: < 1 year, >1 year.
RESULTS 40 patients were <1 year and 50 > 1 year (1- 18 years). Detection of an asymptomatic tumour: < 1 year 30%, >1 year 10%.
Tumor stage at diagnosis
< 1 year > 1 year
Stage I Stage II Stage III Stage IV
4 4 17 14
3 2 10 35
One patient in each group could not be classified adequately. In 5/40 patients < 1 year, chemotherapy was started immediately because of life threatening symptoms from large tumour size.
Therapy
< 1 year > 1 year
Resection alone 13 Resection + 11 Chemo Resection + 5 Radio-chemo Chemo alone 8 Radio-chemo 3 alone
4 14 12 10 10
<1 year: 30/40 patients (75%) had no evidence of disease after therapy, in 5/40 patients tumour recurrence was successfully treated by chemotherapy. 2/40 children (5%) < 1 year died of disease, 2 have residual tumor and 1 is lost to follow-up. > 1 year: 15/50 patients (30%) have no evidence of disease, 33/50 patients (66%) died, 8 of which > 2 years after diagnosis and 2 patients (4%) have residual tumour.
CONCLUSIONS In patients with a neuroblastoma, early detection by ultrasound at a younger age and lower stage of disease compared to detection by clinical symptoms is associated with an improvement of prognosis. For babies the prognosis is good, even in advanced stages requiring aggressive therapy. Due to the large size of most high stage tumours, expectant management seems not to be justified. Patients older than 1 year with a stage IIIIV disease have a poor prognosis even with aggressive treatment.
RESULTS A single papillary lesion was observed near the ureteral orefice in all cases. Size ranged from 1.5 to 2 cm. All patients underwent transurethral resection, and superficial biopsies were taken . Pathological grading, using the WHO/ISUP 2004 Classification, revealed
S43 4 urothelial papillomas and 3 papillary urothelial neoplasms of low malignant potential (PUNLMPs). All were superficial to the lamina propria. At follow-up urinalysis and urine cytology were checked monthly, bladder ultrasonography every three months and cystoscopy at six and twelve months for the first year after surgery. Subsequently urinalysis and urine cytology were done every three months, bladder ultrasound every six months and cystoscopy every year. At an average follow-up of 35
months (range 6 to 84 months) no recurrence was detected.
CONCLUSIONS Transitional cell carcinoma of the bladder is rare in children, and its long term prognosis is still unknown. Routine follow-up with urinary cytology and bladder ultrasound is adeguate. Cystoscopy can be reserved for patients with high grade or potentially highly malignant lesions. Multicenter protocols are required.
# S07-7 (O)
RETROPERITONEAL NEUROBLASTOMAS - A SINGLE INSTITUTION EXPERIENCE IN 90 PATIENTS ¨ DER, Ilka WIEMER*, Gerald TRABOLD*, Raimund STEIN, Annette SCHRO ¨ Joachim W. THUROFF and Peter GUTJAHR* Johannes Gutenberg University, Department of Urology, Mainz, GERMANY - * Johannes Gutenberg University, Department of Pediatrics, Mainz, GERMANY
PURPOSE Nowadays neuroblastomas are detected incidentally by ultrasound in early stages. Primary surgery, chemotherapy and/or radiotherapy are treatment options. However, the approach in babies is controversial. A surveillance strategy is propagated by some groups. In this retrospective study, we evaluated age and stage at diagnosis, therapy and outcome of patients treated at our institutions since 1970.
MATERIAL AND METHODS 90 patients with retroperitoneal neuroblastomas were treated. They were divided into 2 groups according to age at time of diagnosis: < 1 year, >1 year.
RESULTS 40 patients were <1 year and 50 > 1 year (1- 18 years). Detection of an asymptomatic tumour: < 1 year 30%, >1 year 10%.
Tumor stage at diagnosis
< 1 year > 1 year
Stage I Stage II Stage III Stage IV
4 4 17 14
3 2 10 35
One patient in each group could not be classified adequately. In 5/40 patients < 1 year, chemotherapy was started immediately because of life threatening symptoms from large tumour size.
Therapy
< 1 year > 1 year
Resection alone 13 Resection + 11 Chemo Resection + 5 Radio-chemo Chemo alone 8 Radio-chemo 3 alone
4 14 12 10 10
<1 year: 30/40 patients (75%) had no evidence of disease after therapy, in 5/40 patients tumour recurrence was successfully treated by chemotherapy. 2/40 children (5%) < 1 year died of disease, 2 have residual tumor and 1 is lost to follow-up. > 1 year: 15/50 patients (30%) have no evidence of disease, 33/50 patients (66%) died, 8 of which > 2 years after diagnosis and 2 patients (4%) have residual tumour.
CONCLUSIONS In patients with a neuroblastoma, early detection by ultrasound at a younger age and lower stage of disease compared to detection by clinical symptoms is associated with an improvement of prognosis. For babies the prognosis is good, even in advanced stages requiring aggressive therapy. Due to the large size of most high stage tumours, expectant management seems not to be justified. Patients older than 1 year with a stage IIIIV disease have a poor prognosis even with aggressive treatment.