Atypical imaging features of adrenal gland lesions in children - report of three cases and review of literature.

BACKGROUND: The differential diagnosis of adrenal pathology depends on the child's age and imaging findings. CASE REPORT: Three children without clinical symptoms of neoplasm, with an adrenal lesion discovered on diagnostic ultrasound imaging. Laboratory tests for neoplasm were negative. The final diagnosis was based on histopathological examinations after surgical resection. CONCLUSIONS: 1. The value of diagnostic imaging and laboratory tests in differential diagnosis of adrenal gland lesions is limited. 2. Malignant tumors of adrenal glands should be taken into account in children. 3. Surgical resection should be considered in diagnostic algorithm of adrenal gland masses. 4. The final diagnosis is always based on histopathological examination.

Ussefulnes of imaging techniques in the diagnostics of precocious puberty in boys.

BACKGROUND: Precocious puberty (PP) is defined as the appearance of symptoms of puberty in girls before 8 years of age and in boys under 9. Statistically, it occurs much more frequently in girls, while it is a rare pathology in boys. MATERIAL/METHODS: Over the period of 10 years, between 1999 and 2009, 39 girls and 17 boys aged 18 months - 9 years were diagnosed with precocious puberty,, and treated at the Endocrinology Clinic. THE FOLLOWING TESTS WERE PERFORMED IN ALL CHILDREN: physical and anthropometric examinations, abdominal ultrasound scan (US) with evaluation of adrenal glands, examination of testes in boys or breasts and pelvic organs in girls, evaluation of skeletal age and, in selected cases, CT scans of the abdomen, MRI of the CNS, and hormonal laboratory tests. RESULTS: In the group of 17 boys the findings included: gonadotropin -dependent central puberty in 6 boys: idiopathic in 5 cases, and 1 case of a brain tumor - astrocytoma. Gonadotropin-independent precocious pseudopuberty was diagnosed in 11 boys: congenital adrenal hyperplasia in 5; in 1case - hyperandrogenism caused by overactivity of 5-α reductase; in 2 subjects - adrenal adenoma; in 2 boys adrenocortical carcinoma was diagnosed and Leydig cell tumor of testis in 1. CONCLUSIONS: 1. Precocious puberty occurs less often in boys, but in our population it was found in 17 boys of 56 treated children, which constituted as much as 30%. 2. Precocious pseudopuberty was found in 64% of the boys with PP. 3. Adrenal and testicular tumors were the causes of precocious puberty in the youngest group of boys aged 18 months - 6 years.

Abscess mimicking lung metastasis in a 10-year-old boy - case report.

BACKGROUND: Malignant pulmonary tumours in children are very rare; the majority are metastases. Nonspecific radiographic findings of these abnormalities are challenging and may delay the final diagnosis and treatment. CASE REPORT: A 10-year-old boy was admitted to our hospital because of the clinical and radiographic symptoms and signs of pneumonia with abscess formation in the left lower lobe. After initial improvement on antibiotic therapy, a significant deterioration of the patient's condition was observed, together with progression in radiographic examinations. The patient was treated surgically and transferred to the Haematology and Oncology Department with a final diagnosis of pulmonary metastasis of clear cell sarcoma. CONCLUSIONS: Radiographic findings of metastatic diseases may mimic non-neoplastic pulmonary conditions. A lack of specific clinical symptoms and a confusing radiographic pattern in our patient with clear cell sarcoma lung metastasis caused serious diagnostic difficulties.

[Can children with Hodgkin's disease be treated with chemotherapy only?]. / Czy dzieci z choroba Hodgkina moga byc leczone wylacznie chemioterapia?

Currently over 90% of children and adolescents with Hodgkin's disease (HD) can be cured thanks to use of multidrug chemotherapy (CT) combined with involved-field radiotherapy (IF-RT). However, the intensive treatment may increase the risk of late complications which may impair the patients' quality of life. In order to decrease the incidence of late complications the protocol with limited use of IF-RT was introduced in centers of Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG). This study presents the treatment results of patients treated with CT only in comparison with the therapy results of children treated with CT and IF-RT. From 1997 to 2006, 634 children (age: 2-22,5 years) with HD were treated in 14 oncological centers of PPLLSG. Majority of patients received CT (3-8 cycles of MVPP/B-DOPA) combined with IF-RT. In 45 patients with IA-IIA stages presenting favorable risk factors (small mediastinal tumor, peripheral nodular mass of a maximum diameter < 6 cm, involvement of less than three nodular regions, ESR < 50 mm after 1 h, histologic type other than lymphocyte depletion and very good treatment response assessed after 3 CT cycles) IF-RT was omitted. Among 634 children first complete remission (RC) was not achieved in 2.4% of patients. Relapses occurred in 24 children (3.9%). The rates of 5-year overall survival (OS), relapse-free survival (RFS) and event-free survival (EFS) were 97%, 96% i 92%, respectively. All patients treated with CT only remain in first CR. All serious late complications (including 7 second neoplasms) occurred in patients treated with CT combined with RT. Seven children died because of severe complications, among them two in first CR (aplastic anemia, sepsis). Our results show that the use of CT only in precisely selected group of patients with HD do not impair the treatment results and may decrease the risk of late life threatening complications. Treatment response assessment with the use of PET may in future increase the number of patients treated without RT and limit the need of the use of invasive diagnostic methods in patients with residual mass.

[Treatment results of children with neuroblastoma: report of Polish Pediatric Solid Tumor Group]. / Wyniki leczenia dzieci ze zwojakiem zarodkowym wspólczulnym: wstepny raport Polskiej Pediatrycznej Grupy Guzów Litych.

Approximately 60 children aged 0-18 years are diagnosed of NBL each year in Poland. About 60% of all patients suffering from NBL have a chance for durable cure. Unfortunately the prognosis for patients within the high-risk group accounting for more than 50% of all NBL patients remains poor despite the introduction of more intensive chemotherapy regimens with radical surgery procedures and megachemotherapy with subsequent stem cell transplantation. Only one third of patients in this group can be cured. To improve the treatment results of the high-risk patient group and to decrease the rate of therapy related side effects current European treatment protocols have been introduced systematically in Poland. In February 2009 information about 389 patients (age 0.1-16.5 years) diagnosed between 2001 and 2008 were obtained. Results of therapy of 319 patients who started treatment from 2001 to 2007 were analyzed. Between 104 infants and 215 children over 1 year of age, stage 4 of disease was found in 25% and 54.5%, respectively. In this period additionally to European treatment protocols, two another protocols were used. Satisfactory treatment results were obtained in 104 infants (5-year event free survival /EFS/=82.6%), irrespective of the type of treatment protocol. Over 5-year EFS for children over 1 year of age in 1, 2 and 3 stage of disease was: 100%, 86.3% and 64.5%, respectively. On the contrary, 107 patients with 4 stage of disease achieved the 5-year EFS of 27% only. Treatment results obtained in patients treated according to the European HR-NBL-1/ESIOP protocol were better than for patients treated according to other treatment protocols (5-year EFS: 31.1% and 16.4%, respectively), but difference between these groups was not significant. Between 2001 and 2007 data reporting increased to 81% from 19% noted earlier. Unfortunately, results of treatment for children over 1 year of age remain still unsatisfactory. That is why there is a need of improvement of modern, unified treatment realization as well as better data reporting. For realization of these aims adequate financial support is essential.

[Does the residual mediastinal mass have prognostic significance in children with Hodgkin's disease (HD)?]. / Czy obecnosc przetrwalej zmiany w sródpiersiu ma znaczenie prognostyczne w chorobie Hodgkina (HD) u dzieci?

Prognostic significance of residual mediastinal tumor mass in children treated for HD as well as the choice of the optimal management of these cases still remains unknown. In years 1994-2001 in 10 PPLLSG participating centers 480 children (age 2-19.7 years) were treated for HD (stages I-IV). In 338 cases initial mediastinal/lung hilus involvement was present. All patients with initial mediastinal/lung hilus involvement were treated with multidrug chemotherapy combined with involved field radiotherapy. In five cases remission was not achieved. Complete remission (CR) was achieved in 226 patients and uncertain complete remission (UCR) in 107 patients, in whom after completion of planned treatment residual changes in mediastinum/lung hilus were identified in radiological examinations. Twenty four children with persistent mediastinal tumor underwent thoracoscopy or thoracotomy. In only one case histopathological examination revealed the presence of neoplastic cells in mediastinal mass tissue, in 2 another cases cystic changes in mediastinum were present, in one case thymic tissue was identified, necrotic tissue was present in 20 cases. Among 107 children with residual mediastinal tumor and 226 patients with CR achieved, relapses occurred in 6 and 18 patients respectively. Over 5-year relapse-free survival was 92.4% and 91.3% respectively. Patients with the presence of mediastinal/lung hilus tumor after the completion of the treatment do not have an increased risk of relapse, but before the completion of therapy they require careful, clear-sighted and repeated examinations including computed tomography (CT), magnetic resonance imaging (MRI) and especially positron emission tomography (PET) to evaluate the nature of persistent lesions. Only in clinically and radiologically doubtful cases tumor biopsy with subsequent histopatological examination should be performed.

[The health state evaluation in persons after therapy of Hodgkin's disease in childhood: report of the Polish Pediatric Leukemia/Lymphoma Study Group]. / Ocena stanu zdrowia osób z zakonczonym w dziecinstwie leczeniem choroby Hodgkina: raport Polskiej Pediatrycznej Grupy ds. Leczenia Bialaczek i Chloniaków.

The introduction of modern methods of combined therapy: chemotherapy and radiotherapy, allows the cure more than 90% of children and adolescents with Hodgkin's disease. However, the intensive treatment may cause early and late complications. The late complications may include: damage of soft tissues and respiratory, cardiovascular, skeletal, and endocrine systems, and second cancers. Late complications may impair the patients' quality of life after cessation of therapy. This study presents evaluation of health status of persons who underwent therapy for pediatric Hodgkin's disease in centers of Polish Pediatric Leukemia/Lymphoma Study Group. The special questionnaire was established to collect data concerning hematopoietic system function, damage of soft tissues and skeletal system, thyroid and reproductive organs function, respiratory, cardiovascular, and nervous system function, liver and kidney function, status of dentition, occurrence of infections, immunologic system function, and psychological and social problems. The project included patients treated in 9 pediatric oncology centers. During the study the questionnaires of 288 patients [151 boys (52.4%) and 137 girls (47.6%)] in whom treatment of Hodgkins's disease started in 1994-1996 (44 questionnaires), and in 1997-2001 (244 questionnaires). In 42 patients no diagnostic procedures were performed. In this group 20 patients are currently under care of adult oncology clinics, 21 failed to come for a visit, and one patient is treated because of HD progression. The questionnaires of 246 patients treated in 1994-1996 (25 questionnaires) and 1997-2001 (221 questionnaires), were analyzed and the data on the late complications of these patients were obtained. General health status of examined patients was found satisfactory. However, 2.3% of patients had radiological evidence of pulmonary fibrosis, and 4.9% had various ECG abnormalities. Endocrine therapy is needed in 4.5% of patients because of thyroid function abnormalities. Second cancers were diagnosed in 1.7% of patients. The health status of children and adolescents cured from Hodgkin's disease and other childhood cancers should be regularly evaluated. Results of these evaluations will be the basis for introducing new treatment protocols aimed of decreasing the incidence of late complications while maintaining or improving cure rates.

[Genitourinary soft tissue sarcomas located outside bladder and prostate in children treated according to the CWS-96 protocol--report from the Polish Paediatric Solid Tumours Study Group]. / Przebieg i powiklania terapii u dzieci z miesakami ukladu moczowo-plciowego z wylaczeniem pekcherza moczowego i prostaty leczonych wedlug schematu CWS-96.

AIM: Analysis of therapy efficacy in non-bladder/prostate genitourinary sarcomas in children treated from I'1997 to VI'2003 with CWS-96 protocol in Poland. MATERIAL AND METHODS: 19 children (M/F: 15/4, age: 3m-17,5y; median 7,2y). Histopathology: RMS - 15pts (RME-13), non-RMS- 4. RESULTS: Primary site: testes - 9 patients, paratesticular region - 6, uterus - 2, vagina and ovary-1 of each. 63% presented with low stage neoplasm (I - 7, II - 5). Primary tumour exceeded 5cm and/or invaded surrounding tissues in 7 patients (37%). 3 patients had regional, 2 patients--distant lymph nodes metastases. Primary excision: complete in 7 patients, incomplete - 12 (microscopically - 5, macroscopically - 7). Six of 7 patients with macroscopic tumour residues responded to chemotherapy (CR-4, GR-2). One patient (stage III triton tumour of uterus) did not, respond but obtained complete remission after mutilating delayed surgery. No other patient required delayed tumour resection. Radiotherapy (23,5-54 Gy) was given to 8 patients. 3 children developed local relapse, 3 patients died (16%): 2 due to neoplasm progression, 1 of neutropenia-related sepsis. 16 patients are alive (84%) with mean follow-up 48 months. The only permanent complications result from mutilating surgery. CONCLUSIONS: 1) prognosis in children with non-bladder/prostate genitourinary sarcomas is favourable despite incomplete primary excision of the neoplasm. 2) chemotherapy and radiotherapy were accompanied by severe but transient myelosupression in the HR group.

[Influence of age on treatment results in children and adolescents with Hodgkin's disease]. / Wplyw wieku na wyniki leczenia dzieci i mlodziezy z choroba Hodgkina.

Over the last few years, treatment failures (progression, relapse) in Hodgkin's disease (HD) occurred mainly in elder children treated in the Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG) participating centers. That is why analysis of the influence of age on the treatment outcome in children and adolescents treated with the protocol introduced in 1997 was performed. In years 1997-2001, in 10 centers of PPLLSG, 280 patients (age 2.3-18.8 years) were treated for HD. In all patients MVPP and B-DOPA chemotherapy with or without radiotherapy was introduced. Among 280 treated children the first remission was achieved in 275 patients (98.2%). Relapses occurred in 11 patients (4%). The 5-year probability of overall survival, relapse-free survival (RFS) and event-free survival (EFS) was 99%, 93% and 90%, respectively. All children with relapse were over 10 years old at the time of diagnosis (range: 10.6-17.1, median 14.6 years); mediastinal tumor mass was present in all of them. The logistic regression analysis did not reveal the border value for increasing the probability of relapse or event, thus age of 10 years (age of the youngest child with relapse) was identified as the border value. The probability of 5-year EFS and RFS for children over and under the 10th year of age was 98%, 92% and 100%, 92%, respectively. The differences were not statistically significant. Among children over the 10th year of age some features of the disease occurred more frequently: female sex, shorter history of the disease, presence of mediastinal tumor, greater stage of the disease, NS histopathological type of the disease, presence of general signs and ESR over 50 mm, greater tumor burden and higher number of involved lymphatic regions. Among the patients over the 10th year of age, the presence of the general signs and mediastinal tumor influenced the occurrence of relapses substantially. The aim of the further treatment modifications ought to comprise the need of better treatment outcome, especially in patients over the 10th years of age in which unfavorable prognostic factors are identified. child with relapse) was identified as the border value. The probability of 5-year EFS and RFS for children over and under the 10th year of age was 98%, 92% and 100%, 92%, respectively. The differences were not statistically significant. Among children over the 10th year of age some features of the disease occurred more frequently: female sex, shorter history of the disease, presence of mediastinal tumor, greater stage of the disease, NS histopathological type of the disease, presence of general signs and ESR over 50 mm, greater tumor burden and higher number of involved lymphatic regions. Among the patients over the 10th year of age, the presence of the general signs and mediastinal tumor influenced the occurrence of relapses substantially. The aim of the further treatment modifications ought to comprise the need of better treatment outcome, especially in patients over the 10th years of age in which unfavorable prognostic factors are identified.

[Progress in the treatment of non-Hodgkin's lymphoma (NHL) in children. The report of Polish Pediatric Leukaemia/lymphoma Study Group (PPLLSG)]. / Postep w leczeniu nieziarniczych chloniaków zlosliwych (NHL) u dzieci. Raport Polskiej Pediatrycznej Grupy ds. Leczenia Bialaczek i Chloniaków u Dzieci (PPGLBC).

Treatment results of non-Hodgkin lymphoma (NHL) in children has been shown in this study. From 1979 to 2003 children were registered with the diagnosis of NHL in oncology centers of Polish Pediatric Leukaemia/Lymphoma Study Group, a group of 397 patients with NHL B, 222 pts with NHL T and 54 pts with anaplastic large cell lymphoma (ALCL). The pts with NHL T have been treated according to BFM-90 protocol. The predominant primary site of disease was mediastinum (59.3%). Complete remission (CR) was achieved by 87%. EFS for all NHL T pts was 65% and 56% for pts with extensive tumours and 73% for pts with tumours < 10 cm. Patients with NHL B were treated according to the adopted LMB-89 protocol. The majority were Burkitts type and presented abdominal location (50%). 80% with disseminated disease. CR was achieved by 89% patients, but 94% with LDH < 500 IU/L and 73% with LDH > 500 IU. The median time of follow up was 53 months. EFS was 73% for all patients. The patients with ALCL were treated according to several protocols. Peripheral nodes were the most often primary location (40%), than mediastinum (24%) and abdomen (21%). EFS for all pts was 63%. Despite great progress in the therapy of NHL in children during 20 years of observation, the results are not satisfactory in disseminated stages. It is necessary to look for new prognostic markers which make it possible to improve classification of patients. Major surgery in advanced stages is not recommended since it delays chemotherapy and fails to improve overall survival. Early detection of neoplasm is one of the most important efforts to improve therapy success.

[Rare tumours--are they really rare in the Polish children population?]. / Nowotwory rzadkie--czy rzeczywiscie rzadkie w populacji polskich dzieci?

Clear statement that pediatric neoplasms are really rare is not easy. Thus the incidence of rare tumours in children has not been defined so far. The paper efforts to assess the topic of rare tumours of childhood in the Polish population. Following two categories are proposed: tumours typical for adults, but possible in children (neoplasms of epithelial origin--mainly carcinomas, melanomas, carcinoids) and paediatric tumours consisting less than 10% of cases in corresponding clinical groups according to the ICCC classification. Data on 317 patients aged 0-18 years treated in centres associated in the Polish Paediatric Group for Solid Tumours (PPGST) were analysed. Classical adult malignancies were registered in 130 patients: carcinomas in 90 (mean age 12.6 +/- 4.5 years), melanomas in 25 (mean age 9.4 +/- 4.9) and carcinoids in 9 (mean age 14.5 +/- 1.2 years). Non epithelial neoplasms were registered in 187 patients (mean age 10.4 +/- 5.5). That group included rare tumours of soft tissue, CNS, bones and other organs. Treatments of certain groups were specified by separate therapeutic protocols within PPGST. Rare malignancies of adult-type among children under 18 years of age in Poland comprised 1.5% of all pediatric neoplasms. The incidence of adult-type neoplasms increased with age until 14 years. In patients over 15 years of age the number of registered cases decreased. It may suggest a first peak of incidence in early adolescence or an underestimation of number of patients with carcinoma aged over 15 years. In the analyzed group, the mean age of patients with carcinomas and other epithelial and unspecified tumours significantly exceeded the age of children with rare neoplasms of non-epithelial origin (12.1 +/- 4.7 vs 10.4 +/- 5.5 years; p<0.05). A very young age at diagnosis of malignant melanomas (mean 9.4 years) and numerous cases of carcinomas affecting the digestive tract (n=24; 27% of all carcinomas), especially those located in colorectal region (n=10), seem surprising. The preliminary analysis of the collected data on rare neoplasms in Poland encourage to undertake a prospective study, meant to link the epidemiology and characteristics of rare epithelial tumours in childhood with diagnostic and therapeutic suggestions for these types that are not coordinated within Polish Paediatric Group of Solid Tumours.

[Over 30-year experience of Polish Pediatric Leukemia/Lymphoma Study Group for treatment of Hodgkin's disease in children and adolescents: improvement curability and decrease of serious complications]. / Ponad 30-letnie doswiadczenie Polskiej Grupy ds. Leczenia Bialaczek i Chloniaków w leczeniu choroby Hodgkina u dzieci i mlodziezy: poprawa wyleczalnosci i zmniejszenie powaznych powiklan.

Currently over 90% of children with HD can be cured thanks to use of chemotherapy (CT) combined with involved field radiotherapy (IF-RT). From 1971 to 2001, 1062 children and adolescents with HD (stage I to IV) were treated in 10 oncological centers PPLLSG. Majority of patients were treated with CT combined with IF-RT. Year by year the intensity of therapy (CT and RT) was gradually adjusted to the risk-factor groups, and invasive methods of staging were also gradually limited. Supportive care was improved at the same time. Along with the modified therapy protocol, five consecutive periods of time (I: 1971-82; II: 1983-87; IIII: 1988-93; IV: 1994-96; V: 1997-2001) were analyzed. In the first period the basic CT was MVPP (mechlorethamine, vinblastine, procarbazine, prednisone), while later B-DOPA (bleomycin, dacarbazine, vincristine, prednisone, doxorubicin) was gradually introduced alone or alternately with MVPP. In order to decrease the incidence of late complications, the dose of IF-RT from 45 Gy to 15-30 Gy was reduced in the next periods. In V period in 21 children with stage IA and IIA with favorable prognostic factors, CT alone was used. The probability of over 5-year: overall survival, relapse free survival and event free survival were in the I period: 92%, 83% and 77%, in the II period: 97%, 92% and 89%, in the III period: 97%, 93% and 91%, in the IV period: 95%, 91% and 90%, in the V period: 95%, 95% and 93%. Decreased rate of serious complications was also observed. Intensity of therapy should be tailored to the stage of disease, and to other significant prognostic factors. The current strategy of diagnosing and treatment of HD is aimed at balancing between the highest possible cure rates and risk of late complications.