Extrarenal nephroblastoma.

INTRODUCTION: Nephroblastoma is one of the most common solid tumours in children. The occurrence of extrarenal nephroblastoma is exceedingly rare. What can be defined as extrarenal Wilms' tumor must satisfy the following criteria: histologically confirmed nephroblastoma and extrarenal location. MATERIAL AND METHODS: Current data on extrarenal nephroblastoma based on a selective review of the literature. RESULTS: The retroperineal location is reported to be typical in males, whereas the inguinal region is believed to predominate in females. There are no characteristic manifestations of extrarenal nephroblastoma. The symptoms depend on the location and stage upon diagnosis. US, CT and MRI are used to detect tumours in the retroperiteneal space, inguinal, sacro-coccal and scrotal area. However, extrarenal nephroblastoma does not show characteristic radiological features. CONCLUSIONS: In absence of typical clinical presentation or conclusive imaging tests, the diagnosis is based on histology after the resection of the tumor. The recommended management of the extrarenal nephroblastoma is similar to the treatment of intrarenal nephroblastoma.

[Mediastinal tumors in children]. / Guzy sródpiersia u dzieci.

Mediastinal tumours are not very common among various tumours in children. The aim of our study was to evaluate the clinical features, diagnostic approaches and surgical management in 125 patients treated in our Department for 30 years. There were 39 lesions in the anterior, 21 in the medial and 49 in the posterior mediastinum. Solid tumours were diagnosed in 78% of patients. The range of age was between 4 days and 18 years of life. Patients below 5 years of life accounted for 68%. The most common were neurogenic tumours--35%, less frequently tumours of lymphatic origin were seen. Complete excision was possible in 66% of patients, in 26% open biopsy examinations were performed. Enlarged thymus was under control in our out-patient clinic. Combined treatment (radio-, chemotherapy and surgical treatment) was introduced to deal with neurogenic tumours in children. Complete remission lasting 3 years was achieved in 71% of patients in this group. Comparing two periods of time (1970-85 v 1986-2000) no significant differences were found in the prevalence of any kind of mediastinal tumours.

[Assessment of function of the only kidney in children after nephrectomy due to Wilms tumor]. / Ocena czynnosci jedynej nerki u dzieci po nefrektomii z powodu guza Wilmsa.

UNLABELLED: The aim of the study was to evaluate kidney function in children after unilateral nephrectomy caused by Wilms' tumour. The study included 40 former patients of the Department of Paediatric Surgery, who underwent complex therapy of Wilms' tumour at the age from 6 months to 8 years (mean 3.42 +/- 2.5 years). In all patients unilateral nephrectomy was performed and chemotherapy was administered. Additionally 24 out of 40 patients underwent radiotherapy. The time of follow-up was 5-26 years (mean 12.2 +/- 5 years). The patients were divided into 2 groups: la--observation time over 10 years, lb--observation time less than 10 years. Another division was made depending on radiotherapy factor: group R+ (24 patients) and group R-(16 patients). The control group consisted of age-matched 24 persons. In all patients the following was evaluated: endogenous creatinine clearance (Ckr), TRP, RH2O, serum and urine concentrations of electrolytes (Na, K, Ca, P), serum concentration of beta 2-microglobulin (beta 2-M) and urine excretion of beta 2-M and NAG. RESULTS: In all patients Ckr was significantly higher than in the controls. The highest values of Ckr were found in group la, and the difference in relation to group lb was significant (p < 0.02). No difference of Ckr was seen between groups R+ and R-. The mean urinary concentration of beta 2-M was similar in all groups of patients and significantly higher than in the controls. Serum concentration of beta 2-M and urine activity of NAG in all patients were within the normal range. Urine excretion of Na was significantly higher in all patients comparing to controls. CONCLUSIONS: 1. In children, who underwent Wilms' tumour therapy, glomerular hyperfiltration may occur even many years after nephrectomy. 2. Higher urinary excretion of beta 2-M and Na along with normal NAG activity and normal excretion of other electrolytes are the evidence of persistent although slight renal function impairment. 3. Children after complex therapy for Wilms' tumour should be under nephrological care for a long time.

[Renal neoplasms in children]. / Nowotwory nerek u dzieci.

UNLABELLED: Nephroblastoma is the most common kidney tumor in Polish children. OBJECTIVE: to present clinical material and outcome of 533 children with renal tumors. MATERIAL: 500 pts with nephroblastoma and 33 of non-Wilms: CMN, RCC,CSSK, RTK and others tumors were registered, mean age 4.5 years between 1993 till 2002. Stage: CS I--148, CS II--191, CS III--114, CS IV--51, CS V--29 pts. All pts with nephroblastoma were treated according to the first national PPGGL 01-92 protocol with pre-operative chemotherapy (ACT, VCR) for CS I-III and ACT, VCR, DOX in pts of stage IV, over the age of 6 months. Pre-operative chemotherapy was done to 93.8% pts. RESULTS: Radical nephrectomy post pre op chemotherapy was performed in 451 (98%) pts over 6 months and in 44 (8.2%) infants less than 6 months with nephroblastoma. Partial nephrectomy for unilateral tumor post preoperative chemotherapy was made in 6 (1.2%). In 26/29 (89.65%) of CS V nephroblastoma kidney sparing surgery was possible, and in 12 uni-lateral nephrectomy was performed. Surgical complications were mild and occurred only in 8.9% pts. RESULTS: 5-years overall survival of CS I pts (favorable and standard histology) is 93.48%, CS II--96.8%, CS III--84.4%, CS IV--67%, CS V--58%. The results of treatment of 33 pts with non-Wilms renal tumors have improved lately. 78.7% of our pts achieved 5-years overall survival. CONCLUSIONS: The use of systemic neoadjuvant chemotherapy in all pts over 6 months according to the recommendation of SIOP Nephroblastoma protocol (01-92) produced tumor shrinkage, facilitated complete surgical nephrectomy, and was very advantageous in the treatment of renal tumors in children. The results of treatment of non-Wilms tumor have also improved thanks to introduction of new and more aggressive regimens of chemotherapy.

A comparison of renal function outcomes after nephron-sparing surgery and radical nephrectomy for nonsyndromic unilateral Wilms tumor.

OBJECTIVE: To better understand the impact of nephron-sparing surgery (NSS) on renal function in patients with nonsyndromic unilateral Wilms tumor (uWT), a group of such patients treated with NSS were compared with a stage-matched cohort managed with radical nephrectomy (RN). The recommended management of nonsyndromic uWT is RN. However, NSS may decrease the risk of long-term renal disease and associated comorbidities. MATERIALS AND METHODS: An international, multi-institutional review was conducted of nonsyndromic uWT cases managed with NSS and compared with a stage-matched RN cohort. Data were collected on demographics, oncologic characteristics and outcomes, serum creatinine, and estimated glomerular filtration rate (eGFR) calculated via Schwartz formula. RESULTS: Fifteen patients who underwent NSS (6 females and 9 males) met study criteria and were diagnosed at a median age of 2.5 years (range, 0.2-8.2 years) and followed for a median of 8.4 years (range, 0.5-31.8 years). The stage-matched RN cohort consisted of 15 patients (8 females and 7 males) diagnosed at a median age of 3.7 years (0.3-7.4) and followed for a median of 2.1 years (0.6-10.5 years). The median preoperative eGFR was 91.7 (39.4-237.7) and 149.9 (93.8-215.9) for NSS and RN, respectively, P=.026. The median eGFR at last follow-up was 135.3 (57.5-185.8) and 131.0 (98.6-161.2) for NSS and RN, respectively, P=.95. The median change in eGFR during the study period was a gain of 28.6 (-51.9 to 83.0) for the NSS cohort vs a loss of 19.1 (-54.7 to 25.2) for the RN cohort, P=.007. CONCLUSION: In a highly selected patient population with nonsyndromic uWT, NSS provides excellent renal function preservation when compared with RN. These data require validation via prospective investigation on a larger scale.

Clinical factors in relapses of Wilms' tumor--results for the Polish Pediatric Solid Tumors Study Group.

BACKGROUND: The risk factors responsible for recurrences of Wilms' tumor (nephroblastoma) are still under discussion. The aim of the study was to analyze the relationship between relapses of Wilms' tumor and the patients' clinical history. MATERIAL AND METHODS: Clinical data from children registered in the Polish Pediatric Solid Tumors Study Group were analyzed. The clinical stages (CS), pathology variants (high risk: HR, intermediate risk: INT, and low risk: LOW) and chemotherapy regimens were correlated with the outcomes. RESULTS: Recurrences developed in 34 out of 288 (11.8%) patients with Wilms' tumor treated in accordance with International Society for Pediatric Oncology 2001 (SIOP 2001) protocols. Of these 34 patients, 11 initially had CS I, seven were at CS II, four were at CS III, 11 were at CS IV and one had CS V. There were eight patients with second recurrences; of these, seven were in the INT risk group and one in the high histological risk group. There was no correlation between age (p=0.256) or gender (p=0.538) and the risk of tumor recurrence. In the study group, seven out of 10 patients with local recurrences are alive; as are 13 out of 22 patients with distant recurrences (p=0.703). Those who died due to disease progression comprised six out of 26 patients with a first recurrence (four HR, two INT), and seven out of eight with a second recurrence (one HR, six INT). CONCLUSIONS: The prognosis after relapse in initially metastatic patients did not differ from that in patients who had primarily localized disease. The pathology variants probably had more significance.

Adult Wilms tumour.

BACKGROUND: Wilms tumour is the most common renal tumour in children, accounting for above 90% of cases and comprising 8% of paediatric solid tumours. In adults, it is extremely rare and represents only 0.5% of all renal tumours. We reviewed 9 patients older than 16 years: three were treated in the Department of Paediatric Surgery and three in the Department of Urology; additionally, 3 patients were referred to us for consultation with regard to further treatment after having been operated on previously in other centres. Clinical presentation, stage distribution, histology, treatment, and prognosis of all the patients are presented. PATIENTS AND METHODS: The age of 9 patients ranged from 17 to 32 years: there were 8 men and 1 women. Tumour stages were defined according to SIOP classification: 4 were of local stage II, 3 of stage III, and 2 of stage IV with metastases to lungs and bones. Three of them were treated according to the paediatric protocol of the Society of Paediatric Oncology (SIOP 93-01 protocol)-all after a fine-needle biopsy which confirmed nephroblastoma, received preoperative chemotherapy of two drugs (VCR + ACT D) during 4 weeks. Histological studies revealed a low-risk cystic tumour in one case, intermediate-risk tumour in 2 patients, and high-risk tumour with blastemal predominance or with the presence of diffuse anaplasia in 5 patients. One tumour was defined as a mixed high-risk tumour: renal cell carcinoma with nephroblastoma of blastemal predominance. RESULTS: Complete remission was achieved in 3 patients. Three relapsed after complete remission of 6-30 months. A second-line therapy was undertaken in these patients as well as in those who had undergone the initial treatment elsewhere. But the second-line therapy proved ineffective. CONCLUSIONS: Adult patients with Wilms tumour have more aggressive histology and poorer prognosis than children.

[Congenital mesoblastic nephroma: a clinicopathologic study of five cases]. / Wrodzony nerczak mezoblastyczny: kliniczno-patomorfologiczny opis pieciu przypadków.

Congenital mesoblastic nephroma (CMN) accounts for approximately 5% of paediatric renal tumours with the highest peak of incidence during the first 3 postnatal months. CMN almost always has a favourable prognosis. Therefore, CMN needs to be correctly diagnosed and differentiated, principally histologically, from other pediatric neoplasms. We present a clinicopathologic study of 5 cases of CMN.

[Diagnosis and treatment of thyroid cancer in children in the multicenter analysis in Poland for PPGGL]. / Diagnostyka i leczenie raka tarczycy u dzieci w Polscew analizie wieloosrodkowej dla PPGGL.

INTRODUCTION: Differentiated thyroid carcinoma (DTC) in children presents different biological behavior in comparison to adults. Authors presents preliminary results of multicenter analysis concerning incidence, diagnostics and treatment of DTC in children. MATERIAL AND METHODS: The study is a retrospective analysis of 107 pediatric patients from 14 academic centers based on the data from 2000 to 2005 obtained by questionnaire in hospitals involved in the treatment of DTC in children. RESULTS: Papillary thyroid cancer was diagnosed in 83 children, follicular thyroid cancer in 10 children and medullary thyroid cancer in 14 children. Incidence of DTC in children was estimated between 18 and 23 cases per year. The biggest group of patients consisted of children between 11 and 15 years of age, with girls to boys ratio 3.3 : 1. Clinically DTC in children presented most often as solitary thyroid nodule. Cervical lymphadenopathy was observed in 42% of patients. Intraoperative verification indicated metastatic nodes in 50% of children. Low stage DTC predominated (T1 in 36% and T2 in 26% of children). One step surgery was performed in 65% of children with DTC, two step surgery in 25% of patients. I131 therapy was undertaken in 80% of children. Lung metastases were indicated in post therapeutic studies in 14% of children with DTC. Prophylactic thyroidectomies were performed in 79% of children in the group of patients with MTC and RET gene mutations. CONCLUSIONS: The necessity of introduction of unified therapeutic standard in children with DTC in Poland is underlined.

Results of p53 analysis in pediatric malignancies in Poland.

BACKGROUND: Mutations of the p53 gene are thought to be causally associated with the development of various neoplasms. In tumors overexpressing the wild-form of p53, its functional inactivation has been suggested, and MDM2 seems to be important in this process. We analyzed p53 in childhood solid tumors, as data on pediatric malignancies are still limited. PROCEDURE: The p53 gene was screened for mutations by the PCR-S SCP method and sequencing. p53, p21, and MDM2 proteins were analyzed by Western blotting. RESULTS: Overall, p53 mutations were found at a low frequency, 7% (9/126); the frequency calculated for sarcomas was also low, 8.6%. Interestingly, three of the nine detected mutations were new ones. p53 protein was demonstrated in all tumor histotypes, overall, in 63% (43/68) of the tumors, with 18% showing marked overexpression. No p21 was found; and the 76 kDa MDM2 protein was demonstrated in 18% (6/33) of the sarcomas. CONCLUSIONS: In the series of pediatric malignancies studied, the frequency of p53 mutations was very low, whereas p53 protein was present in a high fraction of the tumors. Thus, in total, p53 abnormalities were frequent.

Primary neurilemmoma of the thyroid gland in a 12-year-old girl.

A case of a neurilemmoma of the thyroid gland in a 12-year-old girl is reported. The tumor was noted as a asymptomatic left lower neck swelling 9 months before admission. Ultrasound scan showed the solid lesion of predominantly hypoechoic structure measuring 17.2 x 12 x 26 mm within the left lobe. Pathologic examination of the excised lobe was consistent with neurilemmoma with both Antoni A and Antoni B histologic patterns. To date only 14 cases of this nonepithelial tumor of the thyroid gland have been described in the literature, and all occurred in adult patients. This is the first report of thyroid neurilemmoma in a child.

[Nephroblastoma in children aged less than 6 months at diagnosis]. / Nowotwory nerek u noworodków i niemowlat w pierwszych 6 miesiacach zycia.

UNLABELLED: We present the results of treatment of kidney tumours in newborns and infants aged less than 6 months, in the years 1993-2000, from the Nephroblastoma Committee of the Polish Paediatric Group of Solid Tumours (PPGGL). We have analysed the diagnostic and treatment results in the group of 31 children aged 0 to 6 months. For 19 children registered between 1993 and 1996, event-free survival (EFS) and overall survival (AS) were assessed. Among 450 children registered between 1993 and 2000 by PPGGL and treated for kidney tumours, there were 31 (7.1%) newborns and infants aged below 6 months. The accuracy of diagnosis based on imaging studies was 97%. Only in one child the initial diagnosis of kidney tumour was not confirmed; cystic degeneration of kidney was finally established. The tumours removed during surgery were small, with average size 213 cm3, and in half of the cases the size of the tumour did not exceed 165 cm3. Primary complete excision of the tumour was performed in 21 children (67.7%). In 10 cases histopathology confirmed mesoblastic nephroma, in 19 cases nephroblastoma and in 2 cases sarcoma clarocellulare. In 10 infants (32.2%) with nephroblastoma delayed surgery preceded by chemotherapy was performed. Indications for initial preoperative chemotherapy comprised: tumour in a single kidney, tumour in a horseshoe kidney, preoperative diagnostic biopsy of the tumour and large tumour in neonates older than 3 months. In almost 70% of the children the stage of advancement was low (stage I and IIN-). Histopathology of excised tumours confirmed in 42% of cases low risk, and in 51.6% intermediate risk. Intraoperative complications occurred in 5 infants (16%). The tolerance of reduced chemotherapy by the infants was good. AS was 100%. ESF for the 19 children registered for nephroblastoma between 1993 and 1996 for all stages of advancement and types of histology was 94.75%. CONCLUSIONS: 1) Mesoblastic nephroma and low risk nephroblastoma are the most common tumours in children within the first three years of life. 2) The results of treatment of nephroblastoma in the youngest children (below 6 months of age) are the most favourable and represent world standards.3) Surgical complications in children operated primarily for nephroblastoma indicate the need of performing such operations in academic centres, specialised in newborn surgery. 4) In infants with extensive kidney tumours older than 3 months, primarily considered as inoperative, individual induction chemotherapy should be taken into account.

[Bilateral Wilms' tumour. Results of treatment in 29 children]. / Nerczak obustronny: wyniki leczenia 29 dzieci.

Retrospective analysis of 29 children with bilateral Wilms' Tumour, 14 boys and 15 girls, aged 4m-5 years, from regional centres of paediatric oncology in Poland, treated according to the O1-92 PPGL Protocol of Wilms' tumour, was performed. All patients were treated with two (ACT + VCR) or three (ACT, VCR, DOX) cytostatic drugs preoperatively, and 26 were operated upon after cytoreductive pretreatment. In 12 children nephrectomy was performed. In 28, kidney sparing surgery was possible and made with success. 2 patients who presented disseminated disease and 1 with nephroblastomatosis were given chemotherapy. 19 out of 29 (65.5%) are alive including 1 patient with nephroblastoma. EFS calculated according to Kaplan-Meier analysis for this group, was 58% at 67 months.