Summary Introduction Rhabdomyosarcoma (RMS) accounts for 5% of all pediatric tumors; 15-20% of these tumors are located in the urogenital tract, mostly originating from the prostate or bladder. In ...the light of the steadily improving prognosis for patients with RMS through multimodal and interdisciplinary study protocols with 60–70% long-term survivors, non-oncological aspects such as quality of life and erectile function (EF) have become increasingly important. The aim of this study was to evaluate erectile function in patients having undergone multimodal treatment for RMS of the bladder and prostate. Design The medical records of 24 male patients having undergone surgical treatment for pelvic RMS in the present department between 1975 and 2014 were reviewed, and follow-up was obtained. Erectile function was determined using the Self-Estimation Index of Erectile Function-No Sexual Intercourse (SIEF-NS) and the Erection Hardness Scale (EHS). Potential prognostic factors were evaluated in respect to their impact on erectile function. Results Thirteen patients were included in the study (median age 20 years). Median follow-up period was 12.7 years (1.09–39.85). All patients completed the EHS; nine patients completed the SIEF-NS. All three patients with preserved erectile function (EHS = 4) showed a score indicating no or minimal impairment on sexual function on SIEF-NS (median 33). None of these patients had undergone external radiotherapy, and radical cystoprostatectomy had been performed before the third year of life in two out of three. The remaining patients had erectile dysfunction (EHS = 0). Three patients had an unsatisfying treatment attempt with sildenafil. Seven patients, including all with failures of oral PDE-5-inhibitors, were successfully treated with intracavernous injection of Alprostadil. Discussion This was the largest study, to date, evaluating erectile function in patients treated for RMS of the bladder or prostate. Erectile function was preserved in some patients, despite aggressive treatment modalities. Patients affected by erectile dysfunction (ED) after therapy showed limited response to PDE-5 inhibitors, but even after failure of the latter, an intracavernous injection of Alprostadil showed a significant improvement in EHS and SIEF-NS. Limitations of the study included the retrospective nature, small sample size, and heterogeneity of underlying disease, stage, and treatment modalities used. Conclusions The results suggested that in a subset of patients, EF was preserved after radical surgical treatment of RMS, especially in young boys. Intracavernous injection of Alprostadil was effective, even after failure of PDE-5-inhibitors, and should be offered to patients without spontaneous erections, whereas PDE-5-inhibitors appeared to be largely ineffective. External radiation therapy appeared to have a negative impact on EF.
Craniopharyngiomas are tumorous embryogenic malformations. As the survival rate after craniopharyngioma is high (92 %), prognosis and quality of life (QoL) in survivors mainly depend on adverse late ...effects such as obesity.
We analyzed 214 children and adolescents with craniopharyngioma. The records of 185 patients (86 %) were available for retrospective analysis of weight profiles and risk factors for obesity. Quality of life (QoL) was measured in 145 patients by the Fertigkeitenskala Münster/Heidelberg score (FMH) and in 77 patients by PEDQOL questionnaire.
Eighty-two of 185 patients (44 %) developed severe obesity (body mass index BMI > 3 SD). Obese patients were compared with 79 patients (43 %) who kept normal weight (BMI < 2 SD). No differences between obese and normal weight patients were found in terms of gender distribution, age at diagnosis and follow-up period. However, the BMI SDS at the time of diagnosis was higher (p < 0.0001) in patients who developed obesity than in those who did not. Furthermore, obese patients presented with bigger tumors (p < 0.05) and a higher rate of a hydrocephalus requiring a shunt (p < 0.05) and hypothalamic involvement (p < 0.05). The mothers of patients who developed severe obesity had a higher BMI (p < 0.001) at the time of diagnosis. Obese patients had a higher height-SDS at diagnosis (p < 0.05) and at the time of last follow-up (p < 0.05) when compared with normal weight patients. A prediction model for severe obesity after craniopharyngioma was calculated by logistic regression based on the risk factors: patient's BMI > 2 SD at diagnosis (p < 0.05; odds ratio: 16.4), hypothalamic involvement (p < 0.05; odds ratio: 3.4) and maternal BMI > 25 kg/m(2) (p < 0.05; odds ratio: 4.6). Significant increases in BMI (p < 0.001) occurred during the early post-operative period especially during the first three years after diagnosis. FMH percentiles correlated negatively with BMI SDS (Spearman r: - 0.37; p < 0.001). Children with craniopharyngioma rated their QoL more negative (p < 0.05) in regard to physical abilities, cognitive functioning and social functioning when compared with healthy children of the same age group. Severely obese patients with craniopharyngioma estimated their QoL lower (p < 0.05) for all domains except for autonomy, cognition and familial integration in comparison with non-obese patients.
Hypothalamic tumor involvement and familial disposition for obesity are risk factors for the development of severe obesity in patients with craniopharyngioma. As weight gain starts early after diagnosis and severe obesity causes a reduction in QoL, early therapeutic efforts should be considered in patients at risk. To confirm our results the prospective multicenter study Kraniopharyngeom 2000 on children and adolescents with craniopharyngioma was initiated (www.kraniopharyngeom.com).
Rhabdomyosarcoma of pelvic organs is not common enough for many people to develop large series. However, the authors from Mainz retrospectively analysed 107 children with this condition, and suggest ...that primary chemotherapy followed by radical surgery yields excellent cure rates.
OBJECTIVE
To retrospectively analyse the outcome of children with rhabdomyosarcoma (RMS) of the bladder, prostate or vagina who were treated with chemotherapy, with or without radical surgery or additional radiotherapy, at our institution since 1968.
PATIENTS AND METHODS
From a total of 107 children with RMS seen between 1968 and December 2001, 22 (mean age 5.9, range 0.5–18) had RMS of bladder/prostate or vagina. Twenty of the patients received primary polychemotherapy (vincristine, actinomycin D, cyclophosphamide, adriamycin, and more recently including etoposide and ifosfamide), two had primary surgery and seven had additional radiotherapy. Fourteen patients had radical cystoprostatectomy, with continent cutaneous urinary diversion with an ileocaecal pouch in seven, in one each a transverse colonic pouch, orthotopic ileocaecal bladder substitution, a rectal reservoir and rectosigmoid pouch and a colonic conduit diversion in two patients.
RESULTS
After a mean (range) follow‐up of 8.6 (1.0–26) years, 17 patients had no evidence of disease. Five patients presenting initially with advanced tumour stages died from progressive RMS. Two patients with a continent urinary diversion required ureteric reimplantation for stenosis. In two patients severe bladder contraction after radiotherapy required bladder augmentation.
CONCLUSION
Primary chemotherapy followed by radical surgery of RMS of the prostate and/or bladder allows complete tumour resection in most cases, and yields excellent cure rates.
The treatment of Wilms Tumor is integrated into clinical trials since the 1970's. In contrast to the National Wilms Tumor Study Group (NWTSG) the SIOP trials and studies largely focus on the issue of ...preoperative therapy to facilitate surgery of a shrunken tumor and to treat metastasis as early as possible.
In the SIOP 93-01/GPOH trial and study 1 020 patients with a newly diagnosed renal tumor were registered. 847 of them had a histological proven Wilms Tumor, of whom 637 were unilateral localized, and 173 tumors had an other histology 40 congenital mesoblastic nephroma (CMN), 51 clear cell sarcoma (CCSK), 24 rhabdoid tumor (RTK) and 58 other tumors. Preoperative chemotherapy in benign tumors was given to 1.3 % of the patients. The main objective of the trial was the randomized question, if the postoperative two drug chemotherapy for stage I in intermediate risk or anaplasia can be reduced from conventional 3 courses to an experimental 1 course without loss of efficacy.
519 patients with unilateral nonmetastatic Wilms did receive preoperative chemotherapy. The histology in this group of patients was of intermediate risk in 469 (90 %) patients, 14 (3 %) tumors were low risk and 36 (7 %) high risk. The stage distribution of the tumors was stage I in 315 (61 %), stage II N- in 126 (24 %), stage II N+ in 25 (5 %) and stage III in 36 (7 %) patients. In 17 (3 %) patients the tumor stage remained unclear. Tumor volume was measured in 487 patients before and in 402 after preoperative chemotherapy. The median tumor volume did shrink from 353 to 126 ml. The amount of volume reduction depends on the histological subtype. The event free survival (EFS) after 5 years was 91 % for all patients with unilateral Wilms tumor without distant metastasis. Randomisation was done in 43.7 % for stage I patients and there was no difference in EFS for both treatment arms (90 versus 91 %). The EFS is identical for patients with stage I and II N- (0.92), as well as for stage II N+ and III (0.82). The tumor volume after chemotherapy is a prognostic factor for intermediate risk tumors with the exception of epithelial and stromal predominant tumors. These two subtypes often present as large tumors, they do not shrink during preoperative chemotherapy but they still have an excellent prognosis. On the other hand the prognosis of patients with blastemal predominant subtype after preoperative chemotherapy is worse than in any other patient group of intermediate risk tumors. There are less blastemal predominant tumors compared to primary surgery, but they are chemotherapeutic resistant selected by the preoperative chemotherapy.
Patients with unilateral Wilms tumor without metastasis have an excellent prognosis. The post-operative chemotherapy in stage I can be reduced to 4 weeks without worsening treatment outcome. The reduction of the tumor volume could be identified as a helpful marker for stratification of post-operative treatment. Post-chemotherapy blastemal predominant subtype of Wilms tumor has to be classified as high risk tumor. Focal anaplasia has a better prognosis than diffuse anaplasia and will be classified as intermediate risk tumor.
Isolated extramedullary relapse, especially ovarian recurrence, of acute leukaemia is rare. Local therapy such as irradiation or extensive surgical resection of the mass is ineffective and ...unnecessary.
Over a 20-year period we observed two girls with ovarian relapse of acute lymphoblastic leukaemia (ALL) in over 300 treated children for ALL. Pre-B ALL was diagnosed in a girl at the age of three. Treatment was initiated according to the CoALL 82-protocol. At the age of 11, the girl presented with a huge abdominal mass. Chemotherapy and low-dose radiotherapy succeeded in shrinking the tumour mass, making it operable. A salpingo-oophorectomy was performed. In the second case, a 14-year-old girl in whom pre-B ALL was diagnosed was treated according to the protocol CoALL 06-97. After having achieved complete haematological remission in the bone marrow, she stayed in remission for 18 months. Subsequently, she developed a painless abdominal tumour. Laparoscopic lymph node staging was performed and biopsies were taken. Chemotherapy was initiated according to the BFM protocol for ALL recurrence. Extensive surgical resection of the leukaemic mass, as well as additional radiation was avoided.
Because we experienced favourable results with laparoscopic biopsy in our patients, we are of the opinion that laparoscopy-assisted biopsies are well suited for the management of intra-abdominal tumours in systemic malignant disease.
We study the one‐loop correction to the energy of free string states in light‐cone string field theory. In particular, we construct the supermultiplet of states with two stringy excitations and ...explicitly determine the complete perturbative contribution to their energy shift from the impurity‐conserving channel. The result does not reproduce the gauge theory prediction and features half‐integer powers of the effective gauge coupling I'. We show that the above truncation is incompatible with supersymmetry and thus not suited for conclusive tests of the BMN duality.
A study of fertility was conducted in postpubertal male patients who had been treated for acute lymphoblastic leukemia (ALL) during childhood or adolescence between 1970 and 1980. Thirteen men (age ...18 to 35 years) participated on a volunteer basis. Their age at diagnosis was between 2 and 15 years. Therapy followed the protocol ''Memphis VII (Pinkel)." Interview, physical examination, andrological studies (ejaculate), and hormone status (luteinizing hormone, follicle-stimulating hormone, and testosterone) were performed at least 5 years after completion of therapy. No normozoospermia was achieved; 10 patients were identified with asthenozoospermia and 3 patients with azoospermia. With respect to these data, patients treated for ALL between 1970 and 1980 have more significantly impaired spermatogenesis than expected.
A relationship exists between tumours and malformations both generally and in particular combinations. This is also valid for minor errors of morphogenesis suggesting that embryonic tumours are an ...expression of aberrant intra-uterine morphogenesis. We speculated that these minor aberrations might also manifest in other morphological defects, especially in minor anomalies and malformations of the ribs. We reviewed chest roentgenographs of 1000 children with malignancies for rib anomalies and compared them to 200 patients with mainly infectious diseases. We found 242 rib anomalies in 218 children with tumours (21.8%) and 11 (5.5%) in children without malignancy. This difference was statistically highly significant (P less than 0.001). A high incidence of cervical ribs was found in neuroblastoma (33%), brain tumour (27.4%), leukaemia (26.8%), soft tissue sarcoma (24.5%), Wilms tumour (23.5%) and Ewing sarcoma (17.1%). Only neuroblastoma showed a high incidence of rib bifurcation (4.5%). The increased incidence of these mesenchymal defects in children with malignancies may be another clue for an altered morphogenesis in tumour origin. In neuroblastoma the rib anomaly may be another expression of neurocristopathy as proposed for the association of congenital heart disease and neuroblastoma.
Present health status, complications, and development of long-term survivors of childhood cancer followed for more than 20 years in a single institution were reviewed. The departmental database was ...searched to identify patients diagnosed with childhood cancer and consequently treated between 1965 and 1978. A total of 124 (77%) long-term survivors participated on a voluntary basis in the study. A semi-standardized interview consisted of measures evaluating the present health condition, sequelae of treatment, second malignancies, intellectual development and presence of offspring of the former patients. The majority of patients were treated with chemotherapy (82%), 67% received radiotherapy and 67% underwent surgery. A relapse of the primary tumor was diagnosed in four patients as well as a second malignancy in four other patients. In 33% of the long-term survivors one or more serious therapy-related health problems were noted. Adequate mental and intellectual development was achieved in 65%. Children treated in the early years of pediatric oncology seem to have a satisfactory outcome as viewed over the long term. Consequent ongoing follow-up is still necessary to detect health problems and enhance quality of life for subsequent generations of children with cancer.
PDF
