[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.

[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.

Megachemotherapy followed by autologous stem cell transplantation in children with Ewing's sarcoma.

Twenty-one children with high-risk Ewing's tumor received high-dose chemotherapy with a PBSCT. Aim of the study was evaluation of efficiency and safety of this procedure. All but three patients have meta-static disease at presentation. There were 11 females and the median age at diagnosis was 12 yr (range 4.5-18 yr). Megachemotherapy consisted of melphalan 140 mg/m2/busulfan 16 mg/kg in 12 patients, melphalan 140 mg2/treosulfan 10.0 g/m2 in two patients and melphalan with other drugs in seven patients. Eight of 11 patients transplanted in CR survived with a median follow-up 24 month (range 14-60) and probability of 2-year OS is 0.68 and DFS is 0.63. There was no severe regimen-related toxicity in this group. Children transplanted without remission died: Two of them due to transplant related causes and eight had progression of disease in a median time 7 month after PBSCT. Megachemotherapy with PBSCT is a safe procedure in children with Ewing's sarcoma in remission. Autologos transplantation in children with metastatic Ewing's sarcoma seems to improve their outcome. Patients with Ewing's sarcoma, resistant to conventional therapy and with recurrent disease did not benefit from megachemotherapy. New approaches such as anti-tumor vaccination or using of imatinib are reasonable to introduce in patients with relapsed or resistant to therapy Ewing's tumor.

[Solid neoplasms localised within the parameningeal region and CNS infiltration]. / Nowotwory lite o lokalizacji okolooponowej a zmiany nowotworowe w oun.

INTRODUCTION: Solid neoplasms primarily localised within parameningeal region constitute a significant diagnostic and therapeutic problem. Solid tumours developing in the primary site are soft tissue sarcomas (STS) and lymphoepithelioma. AIM OF STUDY: Evaluation of the incidence of CNS infiltration, histological type of tumour and results of treatment. MATERIAL AND METHOD: The study enrolled 14 patients being treated for solid tumours primarily localised within the perimeningeal region, in the Department of Paediatric Haematology and Oncology of the Medical University of Lublin between 1992 and 2004. The age of patients ranged from 2 up to 17 years (mean 8.5): 4 girls and 10 boys. RESULTS: Soft tissue sarcoma was diagnosed in 10/14 children: rhabdomyosarcoma (RMS) embryonale in 8 patients, RMS alveolare in 1, leiomyosarcoma in 1, and lymphoepithelioma in four children. The stage of disease was III degree in all children (without CNS infiltration). Secondary infiltration of CNS was diagnosed in 2/14 children with STS. CONCLUSION: Secondary CNS infiltration was diagnosed in 2/14 patients. Despite of combined treatment, results of treatment are still poor due to local recurrance with CNS infiltration.

[Non-Hodgkin lymphomas of the nasopharynx in children--diagnostic and therapeutic difficulties]. / Chloniaki nieziarnicze nosogardla u dzieci--trudnosci diagnostyczne i terapeutyczne.

UNLABELLED: About 7% of all childhood cancers comprise non-Hodgkin's lymphoma (NHL). NHL are heterogenous group of neoplasms deriving from lymphatic system (cell B and T). B-cell NHL characterize by high malignancy, but coincidentally good reaction for treatment. In about 20% primary tumour is localised within head and neck, and nasopharynx lymphomas comprise 10%. This location maintains the biggest diagnostic and therapeutic difficulties because of tumours of this site proliferate in the region of frequent infections postponing a proper diagnosis and the local control after complete treatment is difficult. AIM OF THE STUDY: The authors analyse clinical symptoms before diagnosis, the incidence of nasopharynx lymphomas, histopathological type of neoplasm, clinical stage, results of treatment. MATERIAL AND METHODS: The study includes 97 patients who were treated because of lymphomas between 1993-2002. The character of clinical symptoms and their duration, histopathological type of lymphomas, results of treatment were analysed. RESULTS: The primary nasopharynx location was assessed in 9 patients (9.3%). Sex: 7 boys, 2 girls, age: 2-17 years. The duration which elapsed from initial clinical symptoms to diagnosis was 2-10 weeks. The histopathological assessment in 6 children was Burkitt lymphoma and in 3 children--Burkitt-like lymphoma. Metastases: CNS--1 patient, bone marrow--1 patient, abdomen--1 patient. Treatment was performed according to LMB-89 protocol. RESULTS: First complete remission--7 patients; second complete remission--2 patients. CONCLUSIONS: Lymphomas of nasopharynx cause diagnostic problems because of their early stage pseudo-inflammatory manifestation. Special attention should be paid to perform imaging studies (MRI/CT), which are useful in the reaching the proper diagnosis. The radiologic evaluation of primary lesion is still difficult. In the doubtful cases, the surgery and histopathological examination are necessary.

Characteristics and outcome of children with primary soft tissue sarcomas of extremities.

OBJECTIVES: To determine the characteristics and outcome or patients with primary soft tissue sarcomas of extremities in children. MATERIAL AND METHODS: Thirty-six patients treated for soft tissues sarcomas were enrolled into the study. Features analysed: the incidence of soft tissues sarcoma of extremities, the time from first clinical symptoms to making the diagnosis, the primary site of tumour; histopathologic type of tumour, stage of disease, methods and results of the treatment. RESULTS: The time From first symptoms to making the diagnosis was 5.4 months (mean). The site of the tumour was the femur in 6 patients, arm in 3, knee in 1. Histopathologic types: synovial sarcoma in 4 patients, malignant haemangiopericytoma in 2, rhabdomyosarcoma in 2, sarcoma myogenes in 1, primitive neuroectodermal tumour in l. Stage of disease: III deg. -- 8 patients, IV deg. -- 2. Patients underwent treatment according to the soft tissue sarcoma protocols. Results of treatment: first complete remission was observed in 7 patients; second complete remission in 1, one patient is on postoperative treatment. One patient died. CONCLUSIONS: 1. Combined treatment achieves full remission in the majority of patients with soft tissues sarcomas localized within the limbs. 2. In patients with large tumours (>5 cm) the treatment should to be started with inductive chemotherapy, and the surgery should be postponed. 3. Early excision of the tumour should be considered in cases of small tumours (< 5 cm), when resection with wide margin of healthy tissues is possible, without deteriorating the function of the limb or cosmetic damage.

[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.

[Central nervous system metastases in children with solid tumours]. / Przerzuty do osrodkowego ukladu nerwowego w guzach litych u dzieci.

BACKGROUND: Central nervous system (CNS) metastases occur in 20-30% of adult patients with systemic cancers. but they rarely occur in children with solid tumours. AIM: clinical and prognostic characteristics of CNS recurrence in children treated for solid tumours were analysed. PATIENTS AND METHODS: The retrospective study enrolled 218 children treated for solid tumours in the Department of Paediatric Haematology and Oncology, Lublin Medical Academy, from January 1992 to December 2002. The diagnosis in this group was as follow: soft tissue sarcomas -- 51 patients, bone tumours -- 50. Wilms' tumour -- 48, neuroblastoma (NBL) -- 36, germ cell tumours -- 33: Children with primary CNS tumours, retinoblastoma, lymphoma and rare tumours were not analysed. RESULTS: CNS metastases were diagnosed in five children (2.3%) - (2 boys. 3 girls; aged 2,5 to 17 years). Two of them were treated due to Wilms' tumour, one -- NBL, one -- teratoma malignum, one -- leiomyosarcoma. None of the children with bone tumours had CNS metastases. Diagnosis of CNS metastases was confirmed by imaging studies (CT, MRI). The median time from initial diagnosis to the detection of CNS metastases was 14 months. Two children underwent surgical resection of solitary metastases. One of them was also irradiated and received chemotherapy and only this child is alive and achieved complete remission. Other children died, with median period of 32 days. CONCLUSIONS: CNS metastases may occur in children with the recurrence of primary neoplastic disease. The prognosis is grave.

[Results of treatment for osteosarcoma in children and youth]. / Analiza wyników leczenia miesaka kosciopochodnego u dzieci i mlodiezy w materiale wlasnym.

Bone tumours comprise about 5% of childhood neoplasms. Osteosarcoma is the most common sub-type and constitutes 60 % of all bone cancers. Incidence is similar in girls and boys and is increasing steeply with age. The most common localization of this tumour is long bones of the legs. Treatment including chemotherapy and surgery has led to an increase of the percentage of non-mutilating recovery. We present treatment results of 14 children with osteosarcoma.

Incidence of childhood cancers in rural areas of the Lublin Region in 1988-2000.

UNLABELLED: The purpose of the work was the analysis of the number and structure of new neoplasm and morbidity in children living in rural areas of the Lublin Region of Poland. METHODS: the study included the population of children aged 0-17 in the years 1988-2000. The tumours were divided according to the International Classification of Childhood Cancers. The analysis determined the incidence of all tumours and individual types and the rates of percentage structure and incidence. These parameters were calculated for the whole population according to sex and age. RESULTS: 430 cases of childhood cancers were reported. Boys were 53.7%. The most frequent was leukemia (24.4%), neoplasm of CNS (19.5%) and lymphomas (16%). The mean morbidity rate was 100,8 per million (among boys--105.6, girls--95.8). CONCLUSIONS: I. The patient's place of residence is one of the factors affecting the structure of cancers. 2. Higher percentage of lymphomas and CNS tumours and lower percentage of leucaemias and tumours of the sympathetic nervous system and bones was observed in the Lublin Region compared to the values determined for the whole country. 3. The population examined showed lower malignant tumour incidence than that in the whole country as well as increased lymphoma incidence.

The radiologic diagnosis of invasive fungal infections in neutropenic children.

Fungal infections have substantially increased in incidence over the past two decades, especially among patients with cancer who have received high doses of chemotherapy. Diagnosis is based on clinical observations, radiological and laboratory methods and molecular biological techniques. Early diagnosis is important and increases overall response rates, so high-risk patients should be treated empirically, based on the clinical picture and radiologic findings alone. The aim of the study was the analysis of the early clinical picture and radiologic findings of systemic fungal infections. The subjects of this study were patients with childhood cancers and fungal systemic infections. The early symptoms of fungal infection and the radiologic findings were analyzed. The systemic fungal infections were recognized in eight children, treated due to ALL--in four patients, AML--in two and NHL--in two. In all, manifestations of fungal infection were observed during bone marrow suppression secondary to chemotherapy treatment of neoplasm. In all, X-ray examination was normal. Radiological pulmonary changes were found in CT scans in seven patients, in one MRI showed marked abnormnalities of the brain. All patients received intensive antifungal drugs, six patients had a complete or partial response and they were able to continue chemotherapy for treatment of neoplasm. The regression of pulmonary nodules was observed-in the control CT scans. Progression of fungal infection was noticed in two patients, these children died, Conclusions: The early diagnosis of fungal infection in neutropenic patients is important and increases overall response rates. The pulmonary fungal infection should be considered in the differential diagnosis of solitary or multiple pulmonary nodules, particularly in immunocompromised patients.

Outcome of children with idiopathic thrombocytopenic purpura, based on original material.

Idiopathic or immune thrombocytopenia purpura (ITP) is a common hematologic disease in children. ITP is subdivided in an acute and chronic form based solely on the duration of thrombocytopenia. The aim of the study was to analyse the course of disease and outcome of children with acute and chronic ITP. The study enrolled 52 patients with ITP, who were treated in the Department of Pediatric Hematology and Oncology in Lublin from 1998 to 2002. Of the 52 patients with ITP there were 28 girls and 24 boys aged from 12 months to 17 years. Out of the 52 patients, 3 patients had spontaneous recovery, 33 were treated with steroids, one with immunoglobulin, others with combined therapy. Good response to treatment was found in 40 patients. Nine children developed chronic ITP. Out of 9 patients, 6 had splenectomy with good result and 3 are only observed. The benefits of splenectomy in markedly symptomatic chronic ITP far outweigh the risks. The majority of patients with acute ITP had good response after corticosteroids therapy.

Outcome of Ewing sarcoma in children and adolescents with Ewing sarcoma: a five-year survival from a single institution.

Ewing sarcoma, together with neuroblastoma and central nervous system tumors, has got the lowest percentage of complete cure among all malignant diseases in children. From 1993 to 2003, 26 patients with Ewing sarcoma were treated in Pediatric Hematology and Oncology Department, Medical University in Lublin, Poland. In this study the character and time of the clinical symptoms to making the diagnosis, the abnormalities of laboratory tests, and the primary site of tumor were analyzed. The prognosis and results of treatment in children and adolescents were evaluated.

Primary chest tumours in children.

Primary chest tumours in children are rare and appear in 3% of cases, 83% of which are malignant. Early diagnosis is very difficult because of delayed symptoms and highly advanced stage. Symptoms of chest tumours depend on the tumour mass, localization and time of progression. These tumours can cause cough, dyspnoea, Horner's syndrome and superior vena cava syndrome. The purpose of this study was to analyze the kind of chest tumours, clinical symptoms before diagnosis, their duration and results of treatment.

Solid tumours of perimeningeal region in children--diagnostic and therapeutic difficulties.

Thirteen patients, aged 2-17 years, were treated because of primary solid tumours of head and neck location at the Department of Children Hematology and Oncology in Lublin. The authors analyzed clinical symptoms before diagnosis and the duration of these symptoms as well as the kind of tumours. In all cases the tumour was diagnosed on histopathological examination: soft tissue sarcomas--9 children, lymphoepithelioma--4 ones. The prognosis and treatment were estimated.

[Incidence of childhood solid tumours in Lublin region from 1988 to 2000]. / Wystepowanie nowotworów litych wieku dzieciecego na Lubelszczyznie w latach 1988-2000.

The purpose of this study was the analysis of number and structure of new solid tumours and tumour morbidity among children 0-17 years old, in the Lublin Region of Poland, from 1988 to 2000. The analysis was based on sex, age and place of domicile (urban/rural region). During this time in the Lublin Region 344 cases of childhood solid tumours were reported; it was 36.4% of all childhood cancers diagnosed in this region. The structure of the disease was related to sex, age and the place of living. The mean morbidity rate was 42.2 per 1 mln (among boys - 42.2, girls - 42.4). The highest incidence rate was observed among children 0-4 years old (78.1 per 1 million). Higher morbidity was noted among patients living in urban regions (47.3 vs 37.6 / million).