Childhood rhabdomyosarcoma metastatic to bone marrow presenting with disseminated intravascular coagulation and acute tumour lysis syndrome: review of the literature apropos of two cases.

The paper presents diagnostic and therapeutic difficulties in two adolescents with widespread rhabdomyosarcoma (RMS) presenting with severe haemorrhages resulting from disseminated intravascular coagulation (DIC) and with laboratory features of acute tumour lysis syndrome (ATLS). Other published cases of childhood RMS with DIC at admission have been listed and reviewed. It has been concluded that the clinical picture of a widespread RMS in children may resemble acute hematologic malignancy and pose a big diagnostic problem. That is why the presence of small blue round cells morphologically similar to lymphoblasts and/or myeloblasts in bone marrow (BM), lacking hematopoietic makers, should prompt the pathologist to consider possible diagnosis of RMS. Inclusion of desmin, MyoD1 and myogenin Myf4 to the immunohistochemical panel is obligatory in such cases. When the representative histopathological tumour specimens are difficult to obtain, the flow cytometric immunophenotyping of BM metastases could help the standard morphological/immunohistological diagnostic procedures and advance the diagnosis. Recently, the flow cytometric CD45- CD56+ immunophenotype together with Myf4 transcript has been assigned to RMS cells infiltrating BM. In children with disseminated RMS complicated with DIC rapid polychemotherapy aimed at diminishing the malignancy-triggered procoagulant activity should be initiated. However, in cases with concomitant ATLS the initial doses of chemotherapy should be reduced and the metabolic disorders and renal function monitored. The prognosis in children with RMS metastatic to BM with signs of DIC or ATLS at admission depends on the response to chemotherapy, however generally it is highly disappointing.

[Therapeutic difficulties in soft tissue sarcoma occurring in children with neurofibromatosis type 1 - own observations]. / Trudnosci terapeutyczne w miesakach tkanek miekkich u dzieci z nerwiakowlókniakowatoscia typu i- obserwacje wlasne.

UNLABELLED: Neurofibromatosis type I (NF1) is one of the most common genetic disorders in man, predisposing to benign and malignant tumours. The most common malignancies comprise nervous system tumours, less frequently soft tissue sarcomas (STS) and leukaemia - myelodysplasia syndrome. Herein we report five cases of STS diagnosed in children affected with NF1 (3 girls and 2 boys, age: 8 months -17 years). Neurogenic tumours were diagnosed in three children (malignant peripheral nerve-sheath tumour in two and malignant triton tumour in one), while soft tissue sarcomas of rhabdomyosarcoma origin were found in two patients. In four cases the primary tumours were highly locally advanced, unresectable and located in pelvis minor. All patients received protocols for soft STS: CWS-91, 96 and 2002. The paper presents the clinical symptomatology, the course and outcome in children with NF1 affected with STS. CONCLUSION: basing on our own observations STS in NF1 seems to have poor prognosis in spite of combined therapy. Since early diagnosis is essential, children with NF1 should remain under the care of the oncologist.

[Analysis of factors influencing treatment results in children with soft tissue head and neck sarcomas - in the material of the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk]. / Analiza czynników wplywajacych na wyniki leczenia miesakow tkanek miekkich okolicy glowy i szyi u dzieci w materiale kliniki pediatrii, hematologii, onkologii i endokrynologii akademii medycznej w gdansku.

UNLABELLED: The aim of the study was to analyze the prognostic factors for the outcome in childhood head/neck soft tissue sarcomas (STS) treated in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk between 1992 and 2007. MATERIAL AND METHODS: Among 23 children with STS in ten it was located in non-parameningeal region, in eight it was in the parameningeal region, and in five in the orbit. Patients were qualified to particular disease stages according to the current diagnostic and therapeutic protocols (Cooperative Weichtel-sarkom Studie-CWS). The qualifications were based on the results of radiologic examinations (ultrasonography, computed tomography and magnetic resonance of the head and neck region, computed tomography of the chest and abdomen and bone scintigraphy) and also on myelogram smears and cerebrospinal fluid investigation. RESULTS: The longest history concerned the parameningeal (8 months), the shortest the orbital soft tissue sarcomas. Six out of ten patients with non-parameningeal STS were referred to the oncologist with a delay of 6.5 months. This was due to the initially false histopathological diagnosis which excluded the neoplastic process. 82.6% of tumours were diagnosed in advanced, inoperable stages. In 16 children rhabdomyosarcomas (including embryonal subtype - RME in 10, alveolar - RMA in five and undifferentiated in one); in seven non-rhabdomyosarcomas (non-RMS) were found. Good response to chemo- and radiotherapy was observed in 60% of children, mainly with RME. Nine children (mainly with non-parameningeal STS) relapsed. 15 patients are alive (including all with orbital, 6/10 with non-parameningeal and 4/8 with parameningeal STS). Eight children died of disease progression. CONCLUSIONS: 1. Poor outcome in our patients with non-parameningeal head/neck STS results from false initial diagnosis cansing a delay in referring them to the oncologist. It is essential to give more training to the histopathologists about neoplasms in children. 2. Because complete tumour resection in parameningeal STS is rarely feasible, the prognosis in this group is uncertain despite intense chemo- and radiotherapy. 3. The prognosis in orbital STS is usually good; however, they need mutilating surgery in selected cases not responding to therapy.

[Second neoplasms in children with solid tumours in the years 1992-2007. Experiences of Gdansk medical academy]. / Drugie nowoteory u dzieci z guzami litymi w latach 1992-2007. Doswiadczenia osrodka gdanskiego

UNLABELLED: The occurrence of a second tumour is a severe complication of neoplastic disease and its treatment, and it reduces the patient's chances to survive. The aim of the study was to assess the frequency of a second neoplasm and its clinical course in children treated in Gdansk in the years 1992-2007. PATIENTS AND METHODS: There were 420 children and young adults included in the study. They were treated for malignant tumours in this period in the Department of Paediatrics, Haematology, Oncology and Endocrinology Medical Academy of Gdansk. The medical records of these patients were analysed. RESULTS: The second neoplasm was diagnosed in 9 patients, aged 9 to 23 years. They were treated for nephroblastoma - 3 cases, soft tissue sarcoma - 2, Ewing's sarcoma - 1, medulloblastoma - 1, retinoblastoma - 1 and neuroblastoma - 1 case. The second neoplasms were: acute non lymphoblastic leukaemia - 2, soft tissue sarcoma - 2, osteosarcoma - 2, chondrosarcoma - 1, renal cell carcinoma - 1 and glioblastoma multiforme - 1 case. Time between the first and second diagnosis was from 3 and 11/12 to 19 years. Treatment failed in 5 out of 9 children treated for osteosarcoma (2/2), chondrosarcoma (1/1), soft tissue sarcoma (1/2) and acute non lymphoblastic leukaemia (1/2). These patients died of progression of neoplastic disease during 2 to 20 months after the diagnosis of the second tumour. CONCLUSIONS: The diagnosis of the second tumour worsens the prognosis. It is difficult to define the factors that predispose to the second neoplasm. In 5 cases the second neoplasm occurred in the region which was previously irradiated.

Sarcomas in children with neurofibromatosis type 1-poor prognosis despite aggressive combined therapy in four patients treated in a single oncological institution.

OBJECTS: Patients with neurofibromatosis type 1 (NF1) are predisposed to developing soft tissue sarcomas (STS). MATERIALS AND METHODS: We report on four cases of STS diagnosed in locally advanced, unresectable stages in children with NF1 (three girls, one boy; age = 8 months-14 years). All patients received protocols for STS: Cooperative Weichteilsarkomstudie 91, 96 and 2002. One patient with limb rhabdomyosarcoma entered complete remission but developed late metastatic relapse and died of progression despite complete excision and autologous bone marrow transplantation. The other patient with bladder rhabdomyosarcoma died of neutropenia-related sepsis without remission. Patients with malignant peripheral nerve sheet tumour and malignant triton tumour located in the pelvis did not respond to therapy. One of them died of disease progression, while the other is disease-free 6 years post-therapy after mutilating tumour resection. CONCLUSION: STS in NF1 seem to display poor prognosis in spite of combined therapy; thus, children with NF1 should remain under detailed control of the oncologist.

[Neurofibromatosis type 1 in children. Experiences of the Gdansk Paediatric Oncohaematology Centre. Preliminary results]. / Nerwiakowlókniakowatosc typu 1 u dzieci. Obserwacje osrodka gdanskiego. Doniesienia wstepne.

INTRODUCTION: Neurofibromatosis type 1 (NF1) is a frequent genetic disorder of autosomal-dominant pattern. The incidence is about 1 per 3000 live births. Patients with NF1 are predisposed to malignancies including soft tissue sarcomas and leukaemias. The aim of the study was assessment of the most frequent symptoms on the basis of long term observation of children with NF1 and presentation of implemented diagnostic and therapeutic procedures. MATERIAL AND METHODS: In our department there are 149 children (71 boys and 78 girls) aged from 7 months to 18 yrs with diagnosed or suspected NF1. Each child is carefully followed up every 6 months on outpatient basis. Paediatric, neurological and opthalmological examinations are performed during the first visit and in cases of any new symptoms. Number of Lisch nodules, vision field, audiogram, dermatological evaluation of skin abnormalities as well as orthopaedic examination are also investigated. In any case of NF1 without neurological symptoms, MRI of the brain and spine is carried out every 2 years. Moreover, each child is consulted in the Genetic Clinic. RESULTS: Cafe-au-lait spots were observed in all 149 children, freckling of the armpits in 40, peripheral neurofibromas in 30, Lisch nodules in 2 patients. Secondary symptoms and complications such as mental retardation (9 cases) and epilepsy (10 cases), cognitive disorders and learning disabilities (21), abnormalities in MRI examination (53), benign or malignant CNS tumours (9), scoliosis (99) were diagnosed. In 5 patients malignant neoplasms occurred (3.4%) including: RMS--2 cases, Triton tumour--1 case, MPNST--1 case. Two children died of disease progression, one of treatment complications (sepsis) and two children are alive. CONCLUSIONS: 1. Patients with NF1 need regular specialist medical care. 2. Continuous education of the families with this disease is necessary. 3. Diagnostic and therapeutic procedures recommended for patients with NF1 need to be implemented at different levels of health care.

[Soft tissue sarcomas of the parameningeal region in children--own observations]. / Miesaki tkanek miekkich okolicy okolooponowej u dzieci--obserwacje wlasne.

UNLABELLED: Soft tissue sarcomas (STS) non-Hodgkin's lymphomas and less frequently nasopharyngeal carcinomas are the most common malignancies located in the parameningeal region in children. AIM: To assess diagnostic and therapeutic problems in children with parameningeal STS treated in the Departments of Paediatric Oncology in Gdansk and Lublin between 1992 and 2006. MATERIAL AND METHODS: The study includes 17 patients with parameningeal STS; mean age of patients was 5.6 years. In one boy an undifferentiated STS was diagnosed 7 years after treatment of retinoblastoma. RESULTS: Initial symptoms lasted from 2 weeks to 24 months, mean 5.5 months. Symptoms associated with parameningeal location of the tumour (snoring, breathing through the mouth, epistaxis, chronic purulent rhinitis, dysphagia and earache) predominated and were treated initially as upper respiratory tract infections. All analysed patients presented with highly advanced stages of STS. Oncological treatment was conducted according to the schemes approved by the Polish Paediatric Solid Tumours Study Group. Good response to therapy was stated only in 24% children with STS. These patients (all with embryonal subtype) entered complete remission after standard I line therapy. 13 children required more aggressive II line treatment because of poor response to therapy (NR - 5 children) or relapse (8 children). Seven of the analysed patients (41%) are in lasting complete remission, from 32 months to 13 years 2 months (mean 5 years) after therapy discontinuation. In four children (23%) persistent complications of oncological treatment occurred, including postradiation defect of the orbital bulb, postsurgical facial nerve palsy and cranio-nasal fistula complicated with pneumocephaly. A patient with STS of maxillary sinus developed a second neoplasm 2 years after first therapy. This was a glioblastoma multiforme located in the left parietal lobe (outside the radiation field). At present, the boy is in complete remission nearly 4.5 years after treatment for the second tumour. Ten patients died, all in the phase of disease progression. In two of them myelosupressive, gastrotoxic and infectious complications of antitumour therapy were the direct cause of death. CONCLUSIONS: 1. Non-specific initial symptoms of soft tissue sarcomas located in parameningeal region in children suggesting inflammatory process result in diagnostic dilemmas and proper diagnosis delay. 2. Because complete resection of the parameningeal STS is unfeasible, the prognosis is poor in spite of aggressive chemo- and radiotherapy. 3. Complex therapy carries a risk of severe complications, thus it should be conducted in highly specialized oncological centres.

[Initial results of the treatment of six patients with high risk neuroblastoma in the years 2002--2006]. / Wstepne wyniki leczenia szesciu chorych z rozpoznaniem zwojaka zarodkowego wspólczulnego z grupy wysokiego ryzyka w latach 2002--2006.

UNLABELLED: Between the years 2002 and 2006 in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk, six patients with neuroblastoma were qualified for the high risk group. Aim of the study was the evaluation of treatment results after using new methods of therapy. MATERIAL AND METHODS: Intensive multimodal therapy according to the new European protocol HR-NBL-1/ESIOP, was implemented in 6 children. In two cases the high risk group qualification was delayed. Mean time of observation was 2 years 4 months. RESULTS: After the end of therapy, three of six children remain in complete remission. The histopathology of the tumour was assessed according to Shimada classification. Number of NMYC copies was estimated by the FISH method. One patient died of infection after high dose chemotherapy. Relapses occurred in two patients. One of them died of progression and the other is alive, in the third relapse, with progression of disease. CONCLUSIONS: The preliminary analysis shows good results of the new protocol in half of the patients. Further additional cooperative studies with bigger groups of patients are required.

[Clinical characteristics and therapy outcome in children with stage IV Hodgkin's lymphoma--the experience of two oncological centres]. / Charakterystyka kliniczna i wyniki leczenia dzieci z choroba Hodgkina w IV stadium zaawansowania--doswiadczenia dwóch osrodków onkologicznych.

UNLABELLED: The cure rate in children with Hodgkin's disease (HD), at present time exceeds 90% but the prognosis in stage IV HD is much worse. THE AIM of the study was to analyze the initial symptoms, course and results of oncological therapy in children with stage IV of Hodgkin's disease. MATERIAL AND METHODS: The analyzed group comprised of 15 patients with IV stage HD (M/F: 11/4, mean age: 12 years), treated from January 1993 to March 2005, in two Polish centres of paediatric oncology in Gdansk and Lublin. The diagnosis and therapy were carried out according to the current protocols approved by the Polish Paediatric Leukaemia / Lymphoma Study Group (PPGBCh). RESULTS: Mean duration of initial symptoms was 4.5 months, with most children presenting general symptoms of HD. At diagnosis, the involvement of mediastinal and/or hilar lymph nodes was found in nine patients, lung infiltrations in six, involvement of the spleen, liver and bones in five, three and one patient, respectively. The nodular sclerosis histopathological type of HD predominated. Poor response to standard treatment was observed in five children. One patient received additional cycles of chemotherapy MVPP/B-DOPA, four children were administered the 2nd line chemotherapy Salvage 95. One boy with very poor response to the 1st and 2nd therapy lines additionally underwent megachemotherapy with peripheral blood stem cells transplantation. Radiotherapy was given to 13 children. 13 out of 15 children are alive and free of disease with mean follow-up duration of 6 years. In two of them late complications affecting hormonal status, cardio-pulmonary disorders and chronic B and C hepatitis were observed. Two children died including one admitted in a very severe condition, after long-lasting medical history who died of neutropenia-related sepsis. The second boy died 12 months after stem cell transplantation because of a second neoplasm--acute myeloblastic leukaemia. CONCLUSION: Chemo- and radiotherapy implemented according to protocols approved by the PPGBCh for children with stage IV HD, result in complete remission in most patients. Diagnosis made at earlier stages would result in giving less aggressive therapy, connected with a lower risk of durable late complications.

[Complications in children cured from Hodgkin's disease]. / Powiklania plucne u dzieci wyleczonych z choroby Hodgkina.

UNLABELLED: THE AIM of the study was to evaluate the incidence of pulmonary complications in children cured from Hodgkin's disease (HD). MATERIAL AND METHODS: 42 children with HD were treated in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk, between 1994 and 2004. Stages of HD: II--26 children, III--10, IV--6; general symptoms (group B) were present in 50% of patients. Mediastinal involvement was found in 33 children, lung parenchyma infiltration in seven and bronchi involvement in one. In 1/3 of these patients the localization of HD within the chest was massive and symptomatic with signs of the superior caval vein symptoms, cough, dyspnea and cardiac tamponade. The treatment was conducted according to the schemes of the Polish Paediatric Leukaemia /Lymphoma Study Group. Eleven patients required therapy modification including six, in whom the intense line II chemotherapy Salvage 95 was introduced. 29 patients received chest irradiation with doses between J 75 and 36.5 Gy. Pulmonary function was evaluated from the results of clinical examination, 1 chest radiography (CXR), computed tomography, spirometry and lung scintigraphy. RESULTS: Pulmonary complications occurring as fatigue and diminished physical effort tolerance was observed in only two children. Some of the remaining 40 patients demonstrated asymptomatic abnormalities in the analysed tests. Abnormalities in CXR (upper mediastinal fibrosis, postoperational changes within the diaphragm and pneumonitis) were found in six children, minor ventilation problems in spirometry--in 12 and decreased lung perfusion in five. The scintigraphic signs of lung embolisation were not observed in our material. Most of the pulmonary complications occurred in children with enlarged lymph notes located within the chest, especially these with bulky disease presenting with cardio-pulmonary symptoms. In this group of patients the chest irradiation was performed in all except four children, three patients were also administered aggressive salvage chemotherapy. CONCLUSION: The pulmonary complications in children after completed therapy of HD are not common and mainly asymptomatic and occur predominantly in patients with massive mediastinal and/or lung involvement at diagnosis. The issue needs further evaluation of a more numerous group of HD survivors and a longer follow-up.

[Malignant neoplasms localised in the parameningeal region in children treated at two Polish oncological centres-diagnostic and therapeutic dilemmas]. / Nowotwory zlosliwe o lokalizacji okolooponowej u dzieci w materiale dwóch osrodków onkologicznych--trudnosci diagnostyczne i terapeutyczne.

UNLABELLED: Malignant neoplasms localized in the parameningeal region include mainly soft tissue sarcomas (MTM), non-Hodgkin s lymphomas (NHL-B) and, less frequently, nasopharyngeal carcinomas. The aim of the study was to analyze diagnostic and therapeutic problems in children with parameniingeal neoplasms treated in Departments of Paediatric Oncology in Gdansk and Lublin between 1992 and 2004. MATERIAL AND METHODS: The study includes 32 patients (M/F: 23/9), aged 2 to 17 years, mean 6,3 years. In 17 children MTM was diagnosed: in nine NHL-B-cell and in six--nasopharyngeal carcinoma (lymphoepithelioma). The diagnosis of NHL-B and undifferentiated MTM were made in two children treated previously for NHL-nonB and retinoblastoma. Two cases of NHL appeared in a girl with ataxia-teleangiectasia syndrome. RESULTS: Initial symptoms lasted from 2 weeks to 24 months, mean 4,5 months for the whole group. In NHL patients mean period ofsymptoms was 4,5 weeks, in MTM- 5,5 months and in lynmphoepithelioma--7 months. Symptoms associated with the tumours localisation (snoring, breathing through the mouth, epistaxis, chronic purulent rhinitis, dysphagia and earache) predominated and were treated initially as upper respiratory tract infections. Cervical lymph nodes enlargement was observed in 30% children with MTM and 83% with lymphoepithelioma. Most of patients presented with highly advanced stages of neoplasms. MTM and NHL-B treatment was conducted according to the protocol approved by the Polish Paediatric Solid Tumours and Leukaemia/Lymphoma Studies Group. In patients with lymphoepithelioma different treatment schemes were administered, including chemo- and radiotherapy. Good response to therapy was found in 13/32 patients (41%). The group included 24% children with MTM (all with embryonic subtype), 56% with NHL-B and 67% with lymphoepithelioma. All these patients attained complete remission after standard line I therapy. But 13 children with MTM, four with NHL-B and two with lymphoepithelioma required more aggressive line II treatment because ofpoor response to therapy (NR) or relapse. Finally, 20 of 32 followed-up patients (62,5%) are in durable complete remission from 10 months to 11 years 4 months (mean 4 years) after therapy discontinuation. This group consists of all nine patients with NHL-B, 67% children with lymphoepithelioma and 41% with MTM. In six children (30%) persistent complications of oncological treatment occurred, including: hypoacusia, postradiation defect of the eye ball, postsurgical facial nerve palsy and cranio-nasal fistula complicated with pneumocephaly. A patient with MTM of maxillary sinus developed a second neoplasm 2 years after first therapy. This was glioblastoma multiforme located in the left parietal lobe (outside the radiation field). At present, the boy is in complete remission 2,5 years after treatment for the second tumour Among 32 children with parameningeal neoplasms 11 patients died (nine with MTM and two with lymphoepithelioma), all in the phase of disease progression (five NR and six after relapse). In two of them (with MTM) the direct cause of death was myelosupressive, gastrotoxic and infectious complications of antitumour therapy. One child still undergoes treatment for MTM relapse.

[Influence of surgery and radiotherapy on growth and pubertal development in children treated for brain tumour]. / Wplyw zabiegu operacyjnego i radioterapii na tempo wzrastania i rozwoju dzieci z guzami osrodkowego ukladu nerwowego.

INTRODUCTION: The increasing number of childhood cancer survivors has resulted in a growing interest in the late effects, which depend on type of treatment. Frequently, a brain tumour and its therapy in children are endocrinologically devastating. AIM OF STUDY: The aim of study was to compare growth and pubertal development in children after brain tumour therapy treated or not treated with recombinant growth hormone (rGH). MATERIAL AND METHODS: 18 children were included in this study. Group I - (12/18) not treated with rGH, after total resection of brain tumour: craniopharyngeoma (8/12), astrocytoma (2/12) ependymoma (1/12), germinoma (1/12). Mean time of remission was 5,0yrs (+/- 0,9). Group II - (6/12) treated with rGH, after subtotal resection of craniopharyngeoma (4/6), ependymoma (1/6), medulloblastoma (1/6) and cranial irradiation with mean total doses 46,5 Gy (+/- 5,65). Children were qualified for rGH replacement according to deceleration of growth and lower growth hormone secretion (< 10 ng/ml) in stimulating tests. Mean time of remission was 6,5 yrs (+/- 2,41). Growth, height in centimeters converted to standard deviation score--SDS, body mass index (BMI), pubertal status and hormonal tests, were also evaluated. RESULTS: All patients were treated with surgery with no cranial irradiation in prepubertal age. 100% children of group I needed substitution because of secondary hypothyreosis, 83% due to secondary adrenal insufficiency and 53% of diabetes insipidus. Mean height after brain tumour surgical treatment in group I was - 1,24 SDS (+/- 0,85) and did not significantly change in the time of observation. Two girls needed hormonal substitution for hypogonadotropic hypogonadism. Mean BMI after total resection of brain tumour was 18,09 (+/- 4,20) and significantly increased to 23,73 (+/- 2,82). In group II - all children presented multihormonal pituitary insufficiency. Mean deviation score of height before rGH treatment was - 3,84 SDS (+/- 2,87) and after mean time of rGH therapy of 1,5 yrs (+/- 1,2) decreased to 2,6 (+/- 1,06). Mean BMI before treatment with rGH 18, 06 (+/- 4,4) increased to 22,41 (+ 0,74) in the time of observation and decreased to 18,5 (+/- 2,87) after 1,5 years (+/- 1,2) of rGH treatment. CONCLUSIONS: 1. Children treated with surgery for brain tumour need substitution for secondary hypothyroidism, part of then need treatment for secondary adrenal and gonadal insufficiency and diabetes incipidus. 2. Children who were treated with surgery and/or cranial irradiation developed multihormonal pituitary insufficiency, growth failure and replacement rGh therapy was needed. 3. Total resection of brain tumour without chemo- and radiotherapy did not impair growth in first years after surgery.

[Neurologic symptoms in the course of neuroblastoma in children. Own observations]. / Objawy neurologiczne w przebiegu zwojaka zarodkowego wspólczulnego u dzieci. Doswiadczenia wlasne.

INTRODUCTION: Neurological origin and multiple localization of neuroblastoma (NB) in children predisposed to the occurrence of neurological deficits. They usually present as symptoms of spinal cord compression, peripheral nerve palsy or central nervous system metastases. The aim of the study was to analyze retrospectively the frequency and characteristics of neurological disorders in patients with NB, treated in the Department of Paediatrics, Oncology, Haematology and Endocrinology, Medical University of Gdansk, Poland, between 1992 and 2004. MATERIAL AND METHODS: 53 children with NB, aged 1 month to 10 years 10 months were included in the study. RESULTS: Neurological symptoms were present in 16 children (30%), all with advanced NB (stages III and IV). In 12 of them neurological deficits predominated in the medical history, contributing to the neoplasm's diagnosis. Most of the patients (10 children) demonstrated symptoms of the lower limbs paresis and bladder/bowel sphincter dysfunction. Four children suffered from severe back pain. Mentioned neurological disorders preceeded NB diagnosis for median period of 2,5 months. In three patients neurological complications of surgical tumour resection occurred (Horner syndrome in two and foot dorsiflexorparesis in one child). No neurological side effects of chemo- and radiotherapy were observed. Neurological symptoms recovered completely with oncological treatment in eight patients, while in three a considerable neurological improvement was observed. No recovery of neurological deficits was obtained in patients with post-surgical complications and in children suffering from disease progression. Three patients died of NB dissemination, 13 children are alive including two patients undergoing therapy. CONCLUSIONS: 1. Most of NB patients recovered completely or partly from neurological disorders while on therapy. Persistent symptoms of neurogenic bladder with accompanying urinary tract infections constitute a considerable clinical problem in some children. 2. NB survivors require intensive interdisciplinary medical care of paediatric oncologist, neurologist, and nephrologist.

[Diagnostic and therapeutic difficulties in soft tissue sarcomas localized in nonparameningeal head and neck region--own experiences]. / Trudnosci diagnostyczne i terapeutyczne w przypadkach miesaków tkanek mieskkich zlokalizowanych w obrebie glowy i szyi u dzieci--doswiadczenia wlasne.

INTRODUCTION: Soft tissue sarcomas (MTM) localized within the nonorbital and non-parameningeal head and neck region in children are associated with favourable prognosis. However in our material we have observed many therapeutic failures in this group of patients. The aim of the study was to analyze the reasons for disappointing results of oncological therapy in children with MTM treated between 1992 and 2004. MATERIAL AND METHODS: Nine children (M/F: 6/3; mean age 5,5 years). Five patients were diagnosed with rhabdomyosarcoma, four--with non-rhlabdomyosarcoma, including: angiosarcoma, malignant triton tumour, fibrosarcoma and leiomyosarcoma. RESULTS: mean duration of initial symptoms was 7 months. The first sign of the neoplastic disease in all children was tumour. In as many as five of nine patients initially a false histopathological diagnosis was made based on material obtained from aspiration biopsy of the tumour performed in non-oncological centres. This resulted in a significant delay of the proper diagnosis of malignant disease ranging from 2 to 15 months (mean 7 months). That is why all our patients presented with highly advanced stages of MTM or even in the phase of disease recurrence. Therapy was conducted according to the schemes: MMT-89, CWS-91, CWS-96 and CWS-2002. Durable complete remission after the first line therapy was obtained in one child only. Six patients developed MTM relapse and two--progression during chemotherapy. Finally five children remain disease-free after treatment termination with follow-up of 1 to 1,5 years. Four of then had microscopically complete delayed resection of the tumour or the relapse. Four patients died of neoplasm recurrence and progression. In three of them the proper diagnosis was delayed significantly and they were diagnosed in the first or even second relapse of the tumour. CONCLUSION: Unfavourable course and treatment results in MTM located in nonorbital and nonparameningeal head and neck region in our patients result from initial wrong histopathological diagnosis and delayed therapy institution.

[Relapses in children with nephroblastoma--a single centre experience]. / Wznowy w nerczaku plodowym u dzieci. Badania wlasne.

UNLABELLED: The aim of the study was to assess the incidence of relapse and outcome of treatment in children with nephroblastoma relapse. MATERIAL AND METHODS: In the years 1989-2002 at our centre 67 children were treated for nephroblastoma. Patients were divided into two subgroups. Group I comprised 25 children treated in accordance with the SIOP-9 programme, whilst group II consisted of 42 patients following the SIOP 93-01 protocol. RESULTS: In group I, five children had relapse diagnosed: local in 2, pulmonary in 2 and cerebral in one case. Two of the cases were diagnosed during the treatment, and the other 3 after 1 to 19 months from the completion of treatment. In group II relapse was diagnosed in 4 children: local in 2 and pulmonary in 2 cases. One case was diagnosed while on treatment, and the other 3 were diagnosed 1,2 and 4 months after completion of treatment. Among the children who followed the SIOP-9 programme three patients died, while among those following SIOP 93-01 two patients died. CONCLUSIONS: The prognosis in patients who relapsed remains uncertain. The histological type of tumour and the moment of relapse occurrence are significant prognostic factors.

[Cardio-vascular disorders at the time of diagnosis of malignant solid tumours in children--own experiences]. / Zaburzenia ukladu sercowo-naczyniowego w momencie rozpoznania zlosliwych nowotworów litych u dzieci--doswiadczenia wlasne.

UNLABELLED: Cardio-vascular abnormalities in children with neoplastic disease are observed in some cases even during initial diagnostic examinations. The aim of the study was to evaluate the frequency and characteristics of cardiological and vascular disorders in the initial phase of malignant solid tumors in children. MATERIAL AND METHODS: The study included 236 paediatric patients diagnosed with malignant solid tumours, treated in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdank, between 1992 and 2004. Disorders of the myocardium and pericardium as well as abnormalities in vein blood flow were identified. Diagnostic examinations comprised. electrocardiography, echocardiography, chest X-ray, computed tomography and magnetic resonance imaging. Angiography was performed in selected cases. RESULTS: Abnormalities of the heart and vessels were detected in 35 children (15%). Most of them (83%) were associated, directly or indirectly, with neoplastic growth. High frequency of pericardial effusion was detected (16 patients). Cardiologic emergency requiring immediate cardiosurgical intervention,occurred in two children with neoplasms infiltrating myocardium and/or pericardium. Abnormal vein circulation presented as vena cava superior and vena cava inferior syndromes in ten children. Cardio-vascular disorders not associated with neoplastic disease (congenital heart and vessel defects, pancarditis) were observed as rare conditions (2,5%) but in most cases resulted in delay in starting chemotherapy and modification of the treatment scheme. Cardio-vascular abnormalities associated with neoplastic growth tended to disappear during active chemo- and radiotherapy. CONCLUSIONS: The results of the study confirm that cardio-vascular abnormalities in children with cancer before therapy institution, are not infrequent. They may be life-threatening and influence the course and outcome of therapy. These observations imply the necessity of cooperation between the paediatric oncologist, cardiologist and radiologist during the initial diagnostics of malignant solid tumours in children.

[Malignant pelvic neoplasms in children treated in two Polish oncology centres]. / Nowotwory zlosliwe miednicy mniejszej u dzieci w materiale dwóch osrodków onkologicznych w Polsce.

BACKGROUND: Extensive diagnostic and therapeutic dilemmas appear in children With primary malignant neoplasms located in the minor pelvis. THE AIM OF THE STUDY: To evaluate the clinical symptoms, disease course and the results of treatment in patients with malignant pelvic neoplasms. MATERIAL AND METHODS: The study included 31 children (13 boys and 18 girls; aged 2 months to 16 years; mean age -- 8 years) treated in the Departments of Paediatric Oncology and Haematology in Gdansk and Lublin during the period of 1992-2003. The group comprised 17 patients with soft tissue sarcomas (MTM) (55%), 12 with germinal tumours (TGM) (39%) and tow. with neuroblastoma (NBL) (6%). The great majority of children (90%) presented with highly advanced disease (stages III + IV -- in 28 out of 31 patients). RESULTS: with data analysis we were able to distinguish two categories of patients with different prognosis: with MTM and TGM. Most of he MTM tumours (11/17 - 65%) were localized in the urinary tract, the remaining six developed within pelvic muscles. Ten out of twelve TGM tumours (83%) were located in the ovaries. Radical tumour resection, especially primary resection, was shown to play the key role in both groups. Among TGM patients it was performed in 75% while in MTM patients -- in only 12%. All of these patients entered clinical remission and remain disease free. After adjuvant chemo- and/or radiotherapy secondary tumour resection was done in 17% of TGM and 41% of MTM patients. CONCLUSION: in patients, who were not able to undergo radical tumour resection (mainly MTM patients), the disease progressed and led to death.

[Solid tumours of the vertebral column and spinal cord in children. Reasons and consequences of diagnostic delay]. / Guzy lite kregoslupa i kanalu kregowego u dzieci. Przyczyny i konsekwencje opóznien rozpoznania.

INTRODUCTION: Primary or secondary tumours of spinal cord and vertebral column have various histological structure, various degree of malignancy and various growth dynamics. Such localisation of neoplastic disease creates problems, connected with the risk of irreversible damage of neurological functions. It is essential to make a proper diagnosis very early. AIM: An analysis of initial clinical symptoms and reasons for diagnostic delay in children with neoplasms of the vertebral column and spinal cord. This can facilitate and accelerate diagnostic and therapeutic process. MATERIAL AND METHODS: 20 children treated from 1994 to 2004 in Department of Paediatrics, Haematology, Oncology and Endocrinology in the Medical University of Gdansk, were investigated. Patients were divided into 2 groups. In group I, there were 11 children with neuroblastoma and peripheral primitive neuroectodermal tumour (PNET). The primary focus of the neoplasm was localised beyond the vertebral column with secondary infiltration of the spinal cord. Nine children were included in-group II, with primary focus in the spinal cord: glioma (2), ependymoma (1), meningioma (1) and localised in bones of the vertebra: osteosarcoma (2), chondrosarcoma (1), Ewing sarcoma (1) and those eosinophilic granuloma (1). Patients were admitted to hospital late. In group I, advanced stage of disease (III and IV) was found in 5, and stage IV in 6 children. In the early stage of the disease in-group II, pain and neurological symptoms were predominant. Duration of symptoms was longer than in-group I. It was from 6 weeks to 12 months. In both groups diagnostic delay was noted in 13 out of 20 patients. RESULTS: In spite of using obligatory treatment protocols diagnostic delay contributed to permanent neurological complications in 65% of patients.