Publications by authors named "Anna Synakiewicz"

21 Publications

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Correction to: Plasma free amino acid profiling as metabolomic diagnostic and prognostic biomarker in paediatric cancer patients: a follow-up study.

Amino Acids 2021 Jan;53(1):139-141

Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, 7 Debinki Street, 80-211, Gdansk, Poland.

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http://dx.doi.org/10.1007/s00726-020-02924-2DOI Listing
January 2021

Plasma free amino acid profiling as metabolomic diagnostic and prognostic biomarker in paediatric cancer patients: a follow-up study.

Amino Acids 2021 Jan 11;53(1):133-138. Epub 2020 Nov 11.

Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, 7 Debinki Street, 80-211, Gdansk, Poland.

Amino acids (AAs) play a crucial role in cancer cell metabolism. Levels of 22 plasma AAs at the time of diagnosis and after treatment were established among 39 pediatric cancer patients and 33 healthy children. Glutamic acid levels decreased and tryptophan levels increased during treatment. Cancer patients presented significantly lower levels of glutamine and leucine post-treatment while levels of 12 other AAs were higher comparing to controls. Results suggest that plasma free AA profile may serve as a prognostic biomarker.
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http://dx.doi.org/10.1007/s00726-020-02910-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7822799PMC
January 2021

Biomarkers for pediatric cancer detection: latest advances and future perspectives.

Biomark Med 2020 04 9;14(5):391-400. Epub 2020 Apr 9.

Department of Pediatrics, Hematology & Oncology, Medical University of Gdansk, Poland 7 Debinki Street, 80-952 Gdansk, Poland.

Cancer is one of the major health problems of the modern world. With the development of novel biochemistry and analytical instrumentation, precancer diagnosis has become a major focus of clinical and preclinical research. Finding appropriate biomarkers is crucial to make an early diagnosis, before the disease fully develops. With the improvement of precancer studies, cancer biomarkers prove their usefulness in providing important data on the cancer type and the status of patients' progression at a very early stage of the disease. Due to the constant evolution of pediatric cancer diagnosis, which includes highly advanced molecular techniques, the authors have decided to focus on selected groups of neoplastic disease and these include brain tumors, neuroblastoma, osteosarcoma and Hodgkin lymphoma.
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http://dx.doi.org/10.2217/bmm-2019-0613DOI Listing
April 2020

Efficacy of systemic sirolimus in the treatment of generalized lymphatic anomaly and Gorham-Stout disease.

Pediatr Blood Cancer 2019 05 22;66(5):e27614. Epub 2019 Jan 22.

Vascular Anomalies Center, Division of Hematology/Oncology, Cancer and Blood Disorders Center, Boston Children's Hospital, Boston, Massachusetts.

Background: Generalized lymphatic anomaly (GLA) and Gorham-Stout disease (GSD) are rare complicated lymphatic malformations that occur in multiple body sites and are associated with significant morbidity and mortality. Treatment options have been limited, and conventional medical therapies have been generally ineffective. Emerging data suggest a role for sirolimus as a treatment option for complex lymphatic anomalies.

Procedure: Disease response was evaluated by radiologic imaging, quality of life (QOL), and clinical status assessments in children and young adults with GLA and GSD from a multicenter systematic retrospective review of patients treated with oral sirolimus and the prospective phase 2 clinical trial assessing the efficacy and safety of sirolimus in complicated vascular anomalies (NCT00975819). Sirolimus dosing regimens and toxicities were also assessed.

Results: Eighteen children and young adults with GLA (n = 13) or GSD (n = 5) received oral sirolimus. Fifteen patients (83%) had improvement in one or more aspects of their disease (QOL 78%, clinical status 72%, imaging 28%). No patients with bone involvement had progression of bone disease, and the majority had symptom or functional improvement on sirolimus. Improvement of pleural and pericardial effusion(s) occurred in 72% and 50% of affected patients; no effusions worsened on treatment.

Conclusions: Sirolimus appears effective at stabilizing or reducing signs/symptoms of disease in patients with GLA and GSD. Functional impairment and/or QOL improved in the majority of individuals with GLA and GSD with sirolimus treatment.
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http://dx.doi.org/10.1002/pbc.27614DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6428616PMC
May 2019

Bioanalysis of a panel of neurotransmitters and their metabolites in plasma samples obtained from pediatric patients with neuroblastoma and Wilms' tumor.

J Chromatogr B Analyt Technol Biomed Life Sci 2018 Feb 28;1074-1075:99-110. Epub 2017 Dec 28.

Department of Pharmaceutical Chemistry, Medical University of Gdańsk, Hallera 107 Str., 80-416 Gdańsk, Poland. Electronic address:

This paper details the quantitative analysis of neurotransmitters, including dopamine (DA), norepinephrine (NE), epinephrine (E), and serotonin (5-HT), along with their respective precursors and metabolites in children with solid tumors: Wilms' tumor (WT) and neuroblastoma (NB). A panel of neurotransmitters was determined with the use of dispersive liquid-liquid microextraction (DLLME) technique combined with liquid-chromatography mass spectrometry (LC-MS/MS) in plasma samples obtained from a group of pediatric subjects with solid tumors and a control group of healthy children. Next, statistical univariate analysis (t-test) and multivariate analysis (Principal Component Analysis) were performed using chromatographic data. The levels of tyrosine (Tyr) and tryptophan (Trp) (the precursors of analyzed neurotransmitters) as well as 3,4-dihydroxyphenylacetic acid (DOPAC) (a product of metabolism of DA) were significantly higher in the plasma samples obtained from pediatric patients with WT than in the samples taken from the control group. Moreover, statistically significant differences were observed between the levels of 5-HT and homovanillic acid (HVA) in the plasma samples from pediatric patients with solid tumors and the control group. However, elevated levels of these analytes did not facilitate a clear distinction between pediatric patients with WT and those with NB. Nonetheless, the application of advanced statistical tools allowed the healthy controls to be differentiated from the pediatric oncological patients. The identification and quantification of a panel of neurotransmitters as potential prognostic factors in selected childhood malignancies may provide clinically relevant information about ongoing metabolic alterations, and it could potentially serve as an adjunctive strategy in the effective diagnosis and treatment of solid tumors in children.
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http://dx.doi.org/10.1016/j.jchromb.2017.12.031DOI Listing
February 2018

Amino acid profiles as potential biomarkers for pediatric cancers: a preliminary communication.

Biomark Med 2017 Aug 3;11(8):619-627. Epub 2017 Aug 3.

Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, 80-211 Gdansk, Poland.

Aim: Childhood cancer remains one of the main cause of death in the pediatric population. Amino acids (AAs) level alterations in plasma are considered to play a role in carcinogenesis and further course of the disease.

Methods: Seventy-seven children with cancer, including 47 with hematological and 30 with solid tumors were enrolled in this study and compared with healthy children. Twenty-two plasma-free AAs were determined by HPLC with fluorometric detection.

Results: The results revealed significant decrease in glutamine levels for oncological patients and significant increase in aspartic acid, glutamic acid, asparagine, serine, citrulline, alanine, GABA, tryptophan, methionine, valine, phenylalanine and isoleucine levels in cancer children versus control.

Conclusion: Plasma-free AA profile as a biomarker, which combines metabolic and clinical data, as an innovative and interdisciplinary approach, may allow for faster detection of tumor occurrence, and in the future for monitoring patient during treatment, and possible prediction of cancer recurrence.
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http://dx.doi.org/10.2217/bmm-2017-0102DOI Listing
August 2017

Analytical approach to determining human biogenic amines and their metabolites using eVol microextraction in packed syringe coupled to liquid chromatography mass spectrometry method with hydrophilic interaction chromatography column.

Talanta 2016 Apr 21;150:331-9. Epub 2015 Dec 21.

Department of Pharmaceutical Chemistry, Medical University of Gdańsk, Gen. J. Hallera 107, Gdańsk, Poland. Electronic address:

Analysis of biogenic amines (BAs) in different human samples provides insight into the mechanisms of various biological processes, including pathological conditions, and thus may be very important in diagnosing and monitoring several neurological disorders and cancerous tumors. In this work, we developed a simple and fast procedure using a digitally controlled microextraction in packed syringe (MEPS) coupled to liquid chromatography mass spectrometry (LC-MS) method for simultaneous determination of biogenic amines, their precursors and metabolites in human plasma and urine samples. The separation of 12 low molecular weight and hydrophilic molecules with a wide range of polarities was achieved with hydrophilic interaction chromatography (HILIC) column without derivatization step in 12 min. MEPS was implemented using the APS sorbent in semi-automated analytical syringe (eVol(®)) and small volume of urine and plasma samples, 5 0µL and 100 μL, respectively. We evaluated important parameters influencing MEPS efficiency, including stationary phase selection, sample pH and volume, number of extraction cycles, and washing and elution volumes. In optimized MEPS conditions, the analytes were eluted by 3 × 50 μL of methanol with 0.1% formic acid. The chromatographic separation of analytes was performed on XBridge Amide™ BEH analytical column (3.0mm × 100 mm, 3.5 µm) using gradient elution with mobile phase consisting of phase A: 10mM ammonium formate buffer in water pH 3.0 and phase B: 10mM ammonium formate buffer in acetonitrile pH 3.0. The LC-HILIC-MS method was validated and, in optimum conditions, presented good linearity in concentration range within 10-2000 ng/mL for all the analytes with a determination coefficient (r(2)) higher than 0.999 for plasma and urine samples. Method recovery ranged within 87.6-104.3% for plasma samples and 84.2-98.6% for urine samples. The developed method utilizing polar APS sorbent along with polar HILIC column was applied for simultaneous bioanalysis of trace amounts of polar endogenous biogenic amines in real human urine and plasma samples.
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http://dx.doi.org/10.1016/j.talanta.2015.12.056DOI Listing
April 2016

Thymoma and thymic carcinoma in children and adolescents: a report from the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT).

Eur J Cancer 2015 Nov 7;51(16):2444-52. Epub 2015 Aug 7.

Division of Hematology-Oncology, Department of Woman's and Child's Health, University Hospital of Padova, Italy.

Background: Thymomas and thymic carcinomas belong to a group of thymic epithelial tumours arising from the anterior mediastinum and, are extremely rare in children in which no therapeutic guidelines have been established. The aim is to describe paediatric characteristics of these tumours and give some therapeutic indications.

Methods: Retrospective analysis of clinical data and therapeutic characteristics of paediatric patients less than 18years with thymic tumours treated between 2000 and 2012 registered in the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) database of the cooperating national rare paediatric tumour working groups from France, Italy, Germany and Poland.

Results: Sixteen children with thymoma, median age 11years and 20 patients with thymic carcinoma, median age 14years were enrolled into study. At diagnosis complete primary resection was possible in 11 patients with thymoma and one with thymic carcinoma; resection with microscopic residue was performed in three cases and incomplete resection with macroscopic residue in four patients. Chemotherapy with various regimens was administered to 22 children; 17 of them as neoadjuvant chemotherapy. Eight patients with thymic carcinoma received additional radiotherapy. Seventeen children died (15 thymic carcinoma, two thymoma). Five-year overall survival for patients with thymic carcinoma is 21.0±10.0%.

Conclusions: This study confirms the possibility to perform European retrospective analysis even in very rare paediatric tumours. Thymic carcinoma is associated with paediatric patients to give a very poor prognosis independently despite multimodal management. Multidisciplinary, multicenter approach and collaboration with adults' physician are necessary in order to propose homogenous guidelines.
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http://dx.doi.org/10.1016/j.ejca.2015.06.121DOI Listing
November 2015

The role of arginine and the modified arginine deiminase enzyme ADI-PEG 20 in cancer therapy with special emphasis on Phase I/II clinical trials.

Expert Opin Investig Drugs 2014 Nov 26;23(11):1517-29. Epub 2014 Jun 26.

Medical University of Gdansk, Department of Pediatrics, Hematology and Oncology , 7 Debinki Street, 80-211 Gdansk , Poland +48 58 3492880 ; +48 58 349 2847 ;

Introduction: The metabolic differences between normal, healthy cells and neoplastic cells have been exploited by anticancer therapies targeting metabolic pathways. Various studies of malignant processes have demonstrated disturbances in both arginine synthesis and metabolism that enhance or inhibit tumor cell growth. Consequently, there has been an increased interest in the arginine-depleting enzyme arginine deiminase (ADI) as a potential antineoplastic therapy.

Areas Covered: This review summarizes the literature on the potential anti-cancer therapeutics arginine and ADI, an arginine-catabolizing enzyme. The authors searched the MEDLINE database PubMed using the key words: 'arginine, 'ADI', 'arginine in cancer' and 'ADI and cancer'. The authors evaluate prospective randomized studies on cancer patients between 2004 and 2013 as well as ongoing research found through the US National Institutes of Health trial database.

Expert Opinion: The results of current studies are promising but do not give unequivocal answers and so it is impossible to recommend arginine or its enzyme ADI as a therapeutic. In the opinion of the authors, further identification of arginine-dependent malignant tumors and their metabolism should be investigated. Furthermore, the use of these chemicals, in combination with other chemotherapeutics drugs, should be investigated and indeed may improve the success of arginine-depleting enzymes such as pegylated ADI (ADI-PEG20).
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http://dx.doi.org/10.1517/13543784.2014.934808DOI Listing
November 2014

Systemic mastocytosis in children - therapeutic problems.

Med Wieku Rozwoj 2013 Apr-Jun;17(2):126-9

Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdańsk, Poland.

Systemic mastocytosis is a myeloproliferative disorder characterized by growth and accumulation of abnormal mast cells in one or more organs. The symptoms of the disease are due both to the mast cells infiltrating the organs and to the action of its degranulation products. Over 85% of adult patients exhibit point mutations of KIT at position 816 (D816V). Systemic mastocytosis is rare in both adults and children, so treatment is highly individualized; therapy and further treatment is adjusted to each patient's needs. The aim of this study was to present the case of a 14-year old female with systemic mastocytosis and the problems with her treatment. Multidisciplinary management is recommended in systemic mastocytosis.
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October 2013

Glutamine as a supplemental treatment in pediatric and adult oncology patients.

Expert Opin Investig Drugs 2012 Dec 23;21(12):1861-71. Epub 2012 Aug 23.

Medical University of Gdansk, Department of Pediatrics, Hematology, Oncology and Endocrinology, 7 Debinki Street, 80-211 Gdansk, Poland.

Introduction: This review summarizes the achievement and role of supplemental therapy in prevention of severe complications following anticancer treatment.

Areas Covered: A promising supplemental therapy agent in the field is glutamine. Glutamine (Gln) is an amino acid that is produced in physiological conditions in human cells. However, in pathological states, glutamine production is often insufficient. In the clinical setting, glutamine has been shown to decrease metabolic side effects resulting from cancer treatment and to improve patient outcome. In clinical trials, the administration of glutamine was shown to benefit specific groups of patients, including oncology patients. The MEDLINE database PubMed search in English using the key words 'glutamine supplementation', 'parenteral and oral glutamine', 'glutamine in cancer' was performed. Only prospective randomized studies on cancer patients between 2005 and 2011 and ongoing researches from the US National Institute of Health trial database and EU Clinical Trials Register were taken into consideration.

Expert Opinion: In the opinion of authors, the optimal dose and route of administration of glutamine needs to be determined in future studies. This review provides an overview of the use of glutamine as supplemental therapy in patients with cancer, including its use in pediatric patients.
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http://dx.doi.org/10.1517/13543784.2012.717929DOI Listing
December 2012

Association between intestinal and antioxidant barriers in children with cancer.

Acta Biochim Pol 2012 11;59(2):237-42. Epub 2012 May 11.

Department of Paediatrics, Haematology, Oncology and Endocrinology, University of Gdansk, Gdańsk, Poland.

Objective: Reactive oxygen species (ROS) play a role in cancerogenesis processing and damage tissues. Furthermore, oncological treatment may impair proper function of the gut barrier. The aim of this study was to measure intestinal permeability in children in clinical remission for solid tumours and to search for a possible relationship between free radicals and the intestinal barrier. No such investigation in children has been reported so far.

Research Methods And Procedures: The prospective study consisted of 19 paediatric patients with cancer after completion of chemotherapy. 32 healthy children from the outpatients clinics were recruited for measurement of intestinal permeability and antioxidant barrier as a control group. Intestinal permeability was assessed by measurement of urinary lactulose and mannitol after oral challenge. Antioxidant enzymes: superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px) in erythrocytes were assessed. Ischemia modified albumin (IMA) concentration was measured in serum.

Results: Cancer patients excreted less mannitol and more lactulose versus controls. The ratio of lactulose to mannitol was significantly higher in oncological children vs control (mean 0.188 and 0.0453, respectively, p=0.0006,). Significantly higher IMA level in the oncological group vs control was noted (mean 123.8 and 87.3 U/ml, respectively, p=0.0037). No correlation between intestinal permeability and oxidative stress barrier was found.

Conclusions: Our data shows that intestinal barrier is damaged in paediatric cancer patients after chemotherapy. IMA is believed to play a protective role in the defence against tissue damage. No correlation was found between these two barriers.
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October 2012

Parameters of antioxidant barrier in different histopathologic types of pediatric cancers.

Contemp Oncol (Pozn) 2012 29;16(2):165-9. Epub 2012 May 29.

Department of Pediatrics, Hematology, Oncology and Endocrinology, Medical University of Gdansk, Poland.

Aim Of The Study: The goal of this study was to evaluate the activities of erythrocyte superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px), and the levels of glutathione (GSH) and ischemia-modified albumin (IMA), as potential markers in different histopathologic types of pediatric neoplasms. No studies on this subject have been reported to date.

Material And Methods: SOD, GSH-Px, GSH, and IMA were measured before oncologic treatment in 129 children with neuroblastoma (NB), soft tissue sarcomas (STS), brain tumors, Hodgkin's disease (HD), and acute leukemias, and in 30 healthy controls.

Results: The statistical significance of SOD was observed in patients with brain tumors (median 1840.2 U/g Hb, p = 0.0500). The level of GSH was significantly higher in patients with NB (median 6.38 U/g Hb, p = 0.0031) and leukemias (5.16 U/g Hb, p = 0.0200). IMA was statistically significant in cases of STS, NB, and leukemias compared to healthy children (p = 0.0244, p = 0.0069, and p = 0.0000, respectively). The activity of GSH-Px was not statistically significant.

Conclusions: The antioxidant barrier in all types of pediatric cancers is disturbed. None of the measured parameters was specific enough to represent a reliable marker for any particular histopathologic type of children's neoplasm.
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http://dx.doi.org/10.5114/wo.2012.28797DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3687392PMC
June 2013

Ischemia-modified albumin as a biochemical marker in children with neuroblastoma and soft tissue sarcomas.

J Clin Lab Anal 2011 ;25(4):255-8

Department of Pediatrics, Medical University of Gdansk, Gdansk, Poland.

In this study, the levels of ischemia-modified albumin (IMA) in pediatric oncology patients with soft tissue sarcomas (STSs) and neuroblastoma (NB) were analyzed. To date, there have been no studies concerning IMA in these groups of patients. Ninety-nine children with STSs and NB were analyzed from 2006 to 2009, and 30 healthy children were also enrolled in the study. IMA levels were measured throughout treatment in all patients. The levels of IMA in all cancer patients (mean 116.8±39.3 U/ml), in patients with STSs (mean 119.8±27.5 U/ml), and in patients with NB (mean 114.6±36.6 U/ml) were significantly higher than in the control patients (mean 87.3±38.3 U/ml; P=0.0013, 0.0066, and 0.0164, respectively). IMA levels increased before and during the treatment compared with levels in the controls. The determination of IMA levels in pediatric oncology patients with poor prognoses from STSs and NB may play an important role in predicting response to therapy and overall outcome.
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http://dx.doi.org/10.1002/jcla.20469DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6647736PMC
January 2012

The antioxidant status and response to therapy in children with soft tissue sarcomas and neuroblastoma.

Pediatr Blood Cancer 2011 Oct 4;57(4):561-8. Epub 2011 Feb 4.

Department of Pediatrics, Hematology, Oncology and Endocrinology, Medical University of Gdansk, Gdansk, Poland.

Background: Antioxidant systems in cells maintain the proper homeostasis of reactive oxygen species, which at high concentrations can induce carcinogenesis. The aim of this study was to evaluate the serum levels of ischemia-modified albumin (IMA), erythrocyte superoxide dismutase (SOD), and glutathione peroxidase (GSH-Px) as markers for prognosis in children with neuroblastoma (NB) and soft tissue sarcomas (STS), two cancer types for which reliable prognostic factors are needed.

Procedure: SOD, GSH-Px, and IMA were measured before and during responses to therapy assessment in 99 children with NB and STS and in 30 healthy controls.

Results: There were no statistically significant differences in the erythrocyte SOD and GSH-Px activities between the patients with cancer and healthy controls. The levels of IMA in patients with STS and NB were found to be significantly higher than in the controls (P = 0.0013; P = 0.0066, and 0.0164, respectively). Decreased activities of SOD and GSH-Px were found in all patients with poor-responding (PRS) cancers and decreased SOD activity was found in patients with PRS NB. An increase in GSH-Px was observed in patients with good-responding (GR) NB. All patients with GR cancers demonstrated higher SOD and GSH-Px activities than patients with PRS cancers.

Conclusions: While determining the levels of specific antioxidants as antioxidant-barrier parameters in children with cancer may be valuable in predicting therapeutic responses as well as outcomes, additional studies are required.
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http://dx.doi.org/10.1002/pbc.23014DOI Listing
October 2011

Malignant germ cell tumors associated with Swyer syndrome.

Pediatr Blood Cancer 2011 Mar 15;56(3):482-3. Epub 2010 Nov 15.

Department of Pediatrics, Hematology, Oncology and Endocrinology, Medical University, Gdansk, Poland.

Swyer syndrome is characterized by a higher risk of developing genital malignancies. In this disorder, the most common is gonadoblastoma and dysgerminoma but also, in rare cases, choriocarcinoma. The prognosis in choriocarcinoma is poor. The early diagnosis of dysgenetic gonads is necessary in view of the risk of malignancies. It can be difficult due to its different clinical masks. When the neoplasm precedes the diagnosis of gonadal dysgenesis, adequate oncological treatment should be introduced with parallel gonadectomy. We present a case of 14-year-old female with 46, XY karyotype with choriocarcinoma in one gonad and dysgerminoma in the second one.
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http://dx.doi.org/10.1002/pbc.22675DOI Listing
March 2011

Multiple primary cranio-spinal tumours in a 13-year-old female with neurofibromatosis type 2 management strategy.

Childs Nerv Syst 2011 Jan 27;27(1):175-8. Epub 2010 Jul 27.

Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk, 7 Debinki Street, Gdansk, Poland.

Introduction: Neurofibromatosis type 2 (NF2) is an inherited, rare autosomal dominant syndrome characterised by the development of multiple benign cranial and spinal tumours, peripheral neuropathy, ophthalmological and cutaneous lesions. Herein, we report one case of NF2 treated with multivariate chemotherapy.

Material And Methods: A 13-year-old female presented with multiple cranio-spinal tumours in MRI. First symptoms were progressive changes in vision, left-sided paresis, unilateral sensorineural hearing loss, and left hypoglossal nerve paresis. The patient underwent palliative, partial surgical resection of the tumour which was located in a posterior fossa. Histopathological examination showed a psammomatous meningioma located near the great foramen and schwannomas of VIII nerve in the cerebello-pontine angle. Clinical and radiological examination revealed a rapid progression of the disease. As such, multivariate chemotherapy was used. The patient died 4 years after diagnosis.

Conclusion: NF2 patients with multiple tumours at diagnosis may not be treatable with surgery alone and, as a result, presentation with such a disease in childhood results in poor prognosis. The unification of management strategies in NF2 patients is highly desirable.
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http://dx.doi.org/10.1007/s00381-010-1238-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3015198PMC
January 2011

Letter to the editor.

J Paediatr Child Health 2010 Jul;46(7-8):449-51

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http://dx.doi.org/10.1111/j.1440-1754.2010.01810.xDOI Listing
July 2010

Thymic carcinoma in children: a report from the Polish Pediatric Rare Tumors Study.

Pediatr Blood Cancer 2010 Jul;54(7):916-20

Department of Pediatrics, Hematology, Oncology and Endocrinology, Medical University, Gdansk, Poland.

Background: Invasive thymomas and thymic carcinomas are rare tumors jointly accounting between 0.2% and 1.5% of malignancies in adults. They are usually at an advanced stage when diagnosed and have both high recurrence and poor survival rates. In this report, the aim is to explore our experience in the treatment of thymic carcinomas in Polish children.

Procedure: The clinical data of nine children with thymic carcinomas, treated between 1992 and 2008 in the Polish oncological and surgical centers was retrospectively analyzed.

Results: In five cases, presenting symptoms resulted from the compression of the respiratory ways by the mediastinal tumor. In two children paraneoplastic autoimmune syndromes were associated with thymic carcinoma. In accordance with the Masaoka classification, two patients had stage II, five had stage III, and two had stage IV of the disease. Diagnostic biopsy of mediastinal tumor was performed on eight patients and one underwent complete primary resection and subsequently received radiotherapy; he has passed 11 years since the conclusion of therapy. Six patients received multi-drug chemotherapy with or without steroids. Delayed surgery was performed in four children (R0-2, R1-1, and R2-1). After complete resection, one child received chemotherapy. In three patients, chemotherapy and radiotherapy was administered. Seven patients died, including six due to progression of the disease with the other as a result of complications following chemotherapy; only two patients classed at stage II remain alive.

Conclusions: Most thymic tumors in pediatric patients are inoperable at diagnosis, which results in poor prognosis. Improved chemotherapy approaches are needed.
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http://dx.doi.org/10.1002/pbc.22482DOI Listing
July 2010

Poor-risk high-grade gliomas in three survivors of childhood acute lymphoblastic leukaemia--an overview of causative factors and possible therapeutic options.

Childs Nerv Syst 2009 May 20;25(5):619-26. Epub 2009 Mar 20.

Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk, 7. Debinki Street, 80-211 Gdansk, Poland.

Purpose: Malignant high-grade gliomas are the most common secondary neoplasms in children cured of acute lymphoblastic leukaemia (ALL). Although many predisposing factors exist (including systemic or intrathecal chemotherapy, young age, brain infiltration and genetic predispositions), cranial irradiation appears to be the strongest one.

Methods: Three cases of secondary high-grade gliomas (two multiform glioblastomas, grade IV; one anaplastic astrocytoma, grade III) developed in ALL survivors (F-M, 1:2) 3 to 6.3 years after stopping ALL therapy according to BFM-90 trial.

Results: All tumours were supratentorial, contrast-enhancing, space-occupying, highly advanced and aggressive. Possible risk factors and current therapeutic options for paediatric ALL and malignant gliomas are reviewed and discussed.

Conclusions: Prognosis in secondary malignant gliomas in children is poor (overall survival of 5, 10 and 19 months) despite intense therapy. Thus, protocols for paediatric ALL reduce prophylactic cranial irradiation in favour of intrathecal and intravenous high-dose MTX. Nevertheless, ALL survivors must undergo systematic, long-term surveillance for early detection of intracranial neoplasms.
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http://dx.doi.org/10.1007/s00381-009-0838-2DOI Listing
May 2009