Over the last two decades, the surgical treatment of NPSCs has changed from a formerly customary total splenectomy to spleen-conserving procedures, such as total cystectomy with or without partial splenectomy or partial cystectomy. These therapeutic modalities can be performed laparoscopically, if technically possible. Fenestration or deroofing of the cyst resulted in a high recurrence rate (7/9).

Bladder augmentation or substitution significantly improved the health-related quality of life in children and young adolescents taking part in the study. The authors are planning a prospective long-term follow-up of the patients (longitudinal study) to validate the results.

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Periodic prospective biopsy evaluation of children who have undergone either colocystoplasty or gastrocystoplasty failed to reveal any histological evidence of malignancy after 10-year followup. However, histological evidence of a premalignant lesion 13 years after followup suggests that screening for premalignant lesions should be initiated no later than 6 to 10 years following enterocystoplasty.

To determine the mortality and survival rates, side effects of surgery and adjuvant chemo- and radiotherapy, somatic development, and fertility, the data of 142 patients under the age of 1 year operated upon for solid malignant tumors from 1975 through 1983 were analyzed. The follow-up period ranged from 16 to 25 years (mean 20); 79 patients survived. The male/female ratio of the survivors was 51/28. Investigations were based on the Hungarian Tumor Registry, personal interviews with the patients and their parents, and detailed questionnaires. Fifty-one patients died, 44 of them before the age of 3 years; 13 were lost to follow-up. Of the 79 survivors, 48 had abdominal and 31 extra-abdominal tumors (35 neuroblastomas, 21 renal tumors, 15 soft-tissue sarcomas, 5 gonadal tumors, 2 sacrococcygeal carcinomas, 1 hepatic tumor). Side effects of surgical intervention included partial urinary incontinence (2), partial fecal incontinence (1), intestinal obstruction (2), nerve injury (1), thorax deformity (4), and scar formation resulting in psychological problems (12). Chemotherapy alone (41 patients) resulted in side effects in 19 patients, radio- and chemotherapy in combination (23) caused side effects in 20. Fifteen patients did not receive adjuvant therapy. The most serious late side effects were 24 spinal deformities, one-half of them severe, breast underdevelopment, muscular deformity, and renal damage. In 19 patients more then one side effect was detected. Height and weight gain decreased ( P < 0.01 and <0.05, respectively) in the first 8-10 years of follow-up and accelerated significantly ( P < 0.05 and <0.05, respectively) in the second half of follow-up. The short follow-up time (16-25 years) permitted only limited analysis of infertility. Whenever possible, surgical excision should be the treatment of choice. No routine aggressive chemotherapy is indicated. Radiation therapy, which frequently results in long-term musculoskeletal morbidity, should be avoided. Catch-up somatic development occurred in the second part of the follow-up period.

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