[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.

[Acquired Fanconi-de Toni-Debre syndrome due to therapy for Ewing's sarcoma in 5-years old boy]. / Nabyty zespól Fanconi-de Toni-Debre w przebiegu terapii miesaka Ewinga u piecioletniego chlopca.

We present a 5-years old boy with acquired Fanconi-de Toni-Debre syndrome being a effect of therapy for Ewing's sarcoma. At the age of 3 years, this boy was diagnosed as suffering from Ewing sarcoma of his right femur. The boy received a course of 8-month pre-surgery (6 VIDE--Vincristine, Ifosfamide, Doxorubicin, Etoposide cycles and 2 VAI--Vincristine, Actinomycin, Ifosfamide cycles) and 6-month post-surgery (6 VAI--Vincristine, Actinomycin, Ifosfamide cycles) cytostatic therapies according to EWING, EURO 99 protocol. In forth month of post-surgery cytostatic therapy, progressive malaise, polyuria, polydypsia, and recurrent vomiting occurred. The association between those symptoms and malignancy was excluded. Laboratory studies revealed hypokaliemia, hypophosphatemia, proximal tubular acidosis, proteinuria, glucosuria, aminoaciduria, hyperkaliuria and hyperphosphaturia. Acquired Fanconi-de Toni-Debre syndrome due to toxic effect of cytostatic therapy on renal proximal tubules was diagnosed. At present, two years after the time the diagnosis was made, despite constant substitution of potassium, phosphates and bicarbonates, deficit of body mass and height, and bone mineral density abnormalities are observed.

[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.

[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.

[Soft tissue sarcomas of bladder and prostate in children treated according to the CWS-96 protocol--report of the Polish Paediatric Solid Tumours Study Group]. / Przebieg i powiklania terapii u dzieci z miesakami pecherza moczowego i prostaty leczonych wedlug Schematu CWS-96.

AIM: analysis of the course, outcome and therapy complications in bladder/prostate soft tissue sarcomas (STS) in children treated from I'1997 to II'2003 according to CWS-96 protocol in Poland. MATERIAL AND METHODS: 22 children (M/F: 17/5, age: 8 months - 17 years 2 months; median 5,3y). Histopathology: RMS-20 patients (RME-14), non-RMS-2 patients. RESULTS: 96% presented with advanced neoplasm (III - 14, IV - 7). In 18 patients (82%) primary tumour exceeded 5cm; in 17 (77%) extended beyond site of origin. Lymph nodes metastases were stated in six (27%), distant metastases in seven patients (32%). No patient underwent primary complete tumour excision. All received chemotherapy for high risk group. Response to chemotherapy after three cycles was favourable in eight and unfavourable in eight children. Delayed resections were performed in 11 patients (mutilating - 7) proving complete in only five. Radiotherapy (32-50,4Gy) was given to 16 patients, mainly after delayed incomplete surgery. Five patients developed local relapse, four--continual disease progression. None of these nine patients had ever complete tumour resection. Nine patients died: seven due to neoplasm progression, two of therapy complications (septic shock, ARDS). Thirteen patients are alive (59%) with mean follow-up of 42 months. Eight children had bladder removed with continent ileal pouch formed in four. CONCLUSIONS: 1) Advanced stages of bladder/prostate sarcomas in children suggest the necessity of earlier diagnosis. 2) Due to adjuvant chemo- and radiotherapy approximately 60% of patients with this diagnosis may be nowadays cured of bladder/prostate sarcomas without mutilating surgical procedures.

[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.

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).