| 1. |
- Ljungman, Gustaf, et al.
(författare)
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Incidence and survival analyses in children with solid tumours diagnosed in Sweden between 1983 and 2007
- 2011
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Ingår i: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 100:5, s. 750-757
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Tidskriftsartikel (refereegranskat)abstract
- Aim: Solid tumours constitute 40% of childhood malignancies. The Swedish Childhood Cancer Registry is population based and includes all children with cancer reported from the six paediatric oncology centres in Sweden. The aim was to investigate incidence and survival. Methods: We used the new WHO ICCC-3 for reclassification of the patients. Incidence and survival analyses were performed in the study population. Results: Two thousand four hundred and eighty-seven children (< 15 years) were diagnosed with solid tumours in Sweden between 1983 and 2007. The distribution of diagnoses was similar to that reported in other studies. The annual incidence was 65.3 per million children. The survival rates at 10 years of follow-up have improved significantly when comparing the two time periods, 1983-1995 and 1995-2007 (76 vs. 82%; p < 0.01). Conclusions: The mean annual incidence of solid tumours in children was 65.3/million and has been stable during the study period. Survival rates for solid tumours at 5, 10 and 20 years follow-up were 80, 79 and 76%, respectively.
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| 2. |
- Andersson, Carola, et al.
(författare)
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Primary spinal intradural mesenchymal chondrosarcoma with detection of fusion gene HEY1-NCOA2 : a paediatric case report and review of the literature
- 2014
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Ingår i: Oncology Letters. - : Spandidos Publications. - 1792-1074 .- 1792-1082. ; 8:4, s. 1608-1612
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Forskningsöversikt (refereegranskat)abstract
- Mesenchymal chondrosarcoma is an extremely rare malignant tumour that most commonly originates in the bone, but is also present in extraskeletal sites. The tumour is morphologically characterized by a biphasic pattern of small round cells and islands of cartilage. Spinal mesenchymal chondrosarcomas are even rarer and, therefore, few investigations exist regarding the biological behaviour of the tumours. In the present study, we report a case of a 10-year-old female presenting with 9 months of back pain and radiographic findings of an intradural lesion measuring 1.5 cm at the level of Th4. The tumour was completely excised and subjected to pathological analyses. Following detection of the HEY1-NCOA2 fusion gene, the tumour was morphologically and immunohistochemically defined as an intradural mesenchymal chondrosarcoma attached to the dura mater. In this study, we validate the recent identification of the fusion gene HEY1-NCOA2 in paediatric extraskeletal mesenchymal chondrosarcomas: The relevant literature is reviewed and further discussed in relation to our findings.
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| 3. |
- Fadeel, Bengt, et al.
(författare)
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Langerhans´ cellhistiocytos: nytt ljus över patogenesen. 75 år sedan Sture Siwes klassiska arbete om »systemisk retikuloendotelios«
- 2008
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Ingår i: Läkartidningen. - 0023-7205. ; 105:51-52, s. 3737-3742
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Tidskriftsartikel (refereegranskat)abstract
- Langerhans´ cellhistiocytos (LCH) benämndes tidigare histiocytosis X, vilket i sin tur var ett samlingsnamn för diagnoserna eosinofilt granulom, Hand–Schüller–Christians sjukdom och Letterer–Siwes sjukdom. Sjukdomen kännetecknas av granulom av langerhanska dendritiska celler och andra immunceller i skelett och/ eller hud/hörselgång, lungor, lever/mjälte och lymfkörtlar samt även andra organ såsom centrala nervsystemet. LCH förekommer dels i en lindrig och ibland rent av självläkande form som begränsas till enstaka organ, dels som en svårare multiorgansjukdom med i vissa fall dödlig utgång. Den bakomliggande patogenesen har länge varit oklar, men senare tids forskning stödjer tanken att LCH orsakas av en obalans i kroppens immunförsvar, inklusive ökad produktion av interleukin-17a, och att denna obalans avspeglar en reaktiv (infektiös) sjukdom snarare än en malign process.
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| 4. |
- Gavhed, Desiree, et al.
(författare)
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Biomarkers in the Cerebrospinal Fluid and Neurodegeneration in Langerhans Cell Histiocytosis
- 2009
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Ingår i: Pediatric Blood & Cancer. - : Wiley. - 1545-5017 .- 1545-5009. ; 53:7, s. 1264-1270
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Tidskriftsartikel (refereegranskat)abstract
- Background. Progressive neurodegeneration may result in potentially severe cognitive and motor dysfunctions as a complication of Langerhans cell histiocytosis (LCH), a suggested IL-17A-associated inflammatory condition. To detect this complication (CNS-LCH) early and to evaluate the potential efficacy of therapeutic interventions, biomarkers detecting and measuring ongoing neurodegeneration Would be valuable. We evaluated cerebrospinal fluid (CSF) biomarkers of ongoing neurodegeneration in CNS-LCH patients. Procedure. Nine patients with endocrine, neuromotor, cognitive or/and behavioral abnormalities as well as neuroradiological evidence of CNS-LCH were evaluated 4-12 years after LCH diagnosis for CSF levels of neurofilament protein light chain (NF-L), glial fibrillary acid protein (GFAp), and total tau protein (TAU). Two patients were analyzed longitudinally. One hundred ten children with newly diagnosed acute lymphoblastic leukemia (ALL) served as controls. Results. NF-L, TAU, and GFAp levels were elevated in four, six, and eight of nine patients studied, respectively. NF-L (P < 0.001) and GFAp (P < 0.001) were higher in patients than in controls (TAU not analyzed in controls). The patient with most severe clinical and neuroradiological CNS-LCH displayed the highest levels of NF-L and GFAp whereas three patients without signs of systemic disease had low TAU levels and normal/slightly elevated NF-L. NF-L tended to be higher at radiological progression of neurodegeneration than at Status quo (P = 0.07). Notably, we experienced frequent lumbar puncture complications in these patients. Conclusions. CSF levels of NF-L, TAU, and GFAp appear to be elevated in CNS-LCH. It would be valuable if these markers were validated in order to serve as markers for early CNS-LCH, to monitor disease progression and to evaluate various treatment attempts for CNS-LCH. Pediatr Blood Cancer 2009;53:1264-1270. (C) 2009 Wiley-Liss, Inc.
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| 5. |
- Mattsson, Niklas, 1979, et al.
(författare)
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Converging molecular pathways in human neural development and degeneration.
- 2010
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Ingår i: Neuroscience research. - : Elsevier BV. - 1872-8111 .- 0168-0102. ; 66:3, s. 330-2
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Tidskriftsartikel (refereegranskat)abstract
- Animal studies suggest that phosphorylation of microtubule-associated protein tau is a physiological way of destabilizing axons in the developing brain, promoting synaptic plasticity, while in the adult human brain tau phosphorylation is a specific sign of Alzheimer's disease. We here show, for the first time, that newborn human infants have extremely high levels of phosphorylated tau in their cerebrospinal fluid, and that these levels decrease during the first years of life. Tau phosphorylation in Alzheimer's disease may be a physiological response to Alzheimer-associated synaptotoxicity.
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| 6. |
- Smeland, Sigbjørn, et al.
(författare)
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Results of the Scandinavian Sarcoma Group XIV protocol for classical osteosarcoma: 63 patients with a minimum follow-up of 4 years.
- 2011
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Ingår i: Acta orthopaedica. - : Medical Journals Sweden AB. - 1745-3682 .- 1745-3674. ; 82:2, s. 211-6
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Tidskriftsartikel (refereegranskat)abstract
- The Scandinavian Sarcoma Group (SSG) XIV protocol is based on experience from previous SSG trials and other osteosarcoma intergroup trials, and has been considered the best standard of care for patients with extremity localized, non-metastatic osteosarcoma. We analyzed the outcome in 63 consecutive patients. Patients and methods From 2001 through 2005, 63 patients recruited from centers in Sweden, Norway, and Finland were included. They received preoperative chemotherapy consisting of 2 cycles of paired methotrexate (12 g/m²), cisplatin (90 mg/m²), and doxorubicin (75 mg/m²). 3 cycles were administered postoperatively, and poor histological responders were given 3 additional cycles of ifosfamide (10-12 g/m²) as a salvage strategy.
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| 7. |
- Virgone, C., et al.
(författare)
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Adrenocortical tumours in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations
- 2021
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Ingår i: Pediatric Blood & Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 68:S4
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Tidskriftsartikel (refereegranskat)abstract
- Adrenocortical tumours (ACTs) are rare during childhood. A complete surgical resection provides the best chance of cure, but the role and efficacy of the adjuvant therapy are still controversial. Various histologic criteria of malignancy for ACTs adopted in children do not facilitate comparative studies and are not completely shared. Therefore, a sharp demarcation between benign and malignant lesions has not been recognised, making it difficult to identify who potentially needs perioperative therapy. This manuscript presents the internationally harmonised recommendations for the diagnosis and treatment of ACTs in children and adolescents, established by the European Cooperative Study Group for Paediatric Rare Tumours (EXPeRT) group within the EU-funded project PARTNER (Paediatric Rare Tumours Network - European Registry).
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| 8. |
- Österlundh, Gustaf, 1956, et al.
(författare)
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Neurochemical markers of brain damage in cerebrospinal fluid during induction treatment of acute lymphoblastic leukemia in children.
- 2008
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Ingår i: Pediatric blood & cancer. - : Wiley. - 1545-5017 .- 1545-5009. ; 50:4, s. 793-8
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Central nervous system (CNS) irradiation has been replaced by systemic high-dose methotrexate (MTX) and intrathecal MTX in acute lymphoblastic leukemia treatment due to the risk of late effects. However, treatment without CNS irradiation might also cause brain damage. PROCEDURE: Cerebrospinal fluid (CSF) was analyzed in 121 patients in an attempt to detect CNS injury. Seventy-three samples were analyzed for neuron-specific enolase (NSE), 108 for glial fibrillary acidic protein (GFAp), 110 for neurofilament protein light chain (NFp), and 70 for ascorbyl radical (AsR). Samples were taken at day 0, 8, 15, and 29 during induction treatment, including intrathecal MTX. Levels at days 8, 15, and 29 were compared with the levels before treatment. RESULTS: NSE levels were 9.0 (+/-3.5) microg/L (mean (+/-SD)) at day 0, 15.0 (+/-5.3) at day 8 (P < 0.001), 13.6 (+/-4.7) at day 15 (P < 0.001) and 11.1 (+/-4.3) at day 29 (P < 0.001). GFAp were 177 (+/-98) ng/L at day 0, 206 (+/-101) at day 8 (P < 0.001), 200 (+/-106) at day 15 (n.s.) and 228 (+/-137) at day 29 (P < 0.001). NFp were below the detection limit 125 ng/L at day 0 in all 110 CSF samples analyzed, and increased significantly above the detection limit in 6/77 samples at day 8, in 11/84 at day 15 and in 22/91 at day 29. The AsR content did not change significantly. CONCLUSIONS: Levels of NSE, GFAp, and NFp increased in CSF, which can be interpreted as early signs of brain damage. AsR levels do not show any convincing signs of oxidative stress.
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| 9. |
- Österlundh, Gustaf, 1956, et al.
(författare)
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Regional cerebral blood flow and neuron-specific enolase in cerebrospinal fluid in children with acute lymphoblastic leukemia during induction treatment.
- 1999
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Ingår i: Journal of pediatric hematology/oncology : official journal of the American Society of Pediatric Hematology/Oncology. - 1077-4114. ; 21:5, s. 378-83
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Tidskriftsartikel (refereegranskat)abstract
- PURPOSE: To investigate possible side effects on the central nervous system from intrathecal methotrexate given during induction treatment for acute lymphoblastic leukemia in childhood. PATIENTS AND METHODS: Twenty-five children with acute lymphoblastic leukemia were examined by cerebral single photon emission computed tomography at the beginning of treatment (16 untreated, 9 during the first week) and after 4 weeks of treatment. Cerebrospinal fluid was sampled for analyses of neuron-specific enolase on four occasions in 54 patients. RESULTS: Regional cerebral blood flow became impaired during treatment in all patients. The single photon emission computed tomography score for nonhomogeneous perfusion increased from 6.4/50 to 16.6/50. Hypoperfusion was global without any clear preference for any lobe. The cerebellum was not affected. Neuron-specific enolase increased significantly during treatment, with a peak after 1 week, followed by a gradual decrease, but it was still significantly elevated after 4 weeks. CONCLUSIONS: Nonhomogeneous cerebral hypoperfusion was found in all patients during induction treatment, including repeated intrathecal administration of methotrexate, but before systemic high-dose methotrexate. Signs of neuronal injury, in the form of a moderate increase in neuron-specific enolase in the cerebrospinal fluid, were found early in the treatment. Follow-up is needed to evaluate the long-term impact of these findings.
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| 10. |
- Österlundh, Gustaf, 1956
(författare)
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Studies of cerebral blood flow and cerebrospinal fluid in childhood acute lymphoblastic leukemia
- 2008
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy and more than 80% of the patients are cured today. Treatment might cause side effects and central nervous system (CNS) irradiation has been replaced by systemic high-dose methotrexate (MTX) and intrathecal (IT) MTX due to the risk of late effects. However, treatment without CNS irradiation is also neurotoxic and might cause brain damage. Three patients developed subacute neurotoxicity, one after IT MTX and two after HDMTX including IT MTX. All showed impaired regional cerebral blood flow (rCBF) when examined by single photon emission computed tomography (SPECT). The patients improved within a few days during treatment with the Ca2+-channel blocker nimodipine and all recovered completely. Another three patients, without neurological symptoms, were examined at different phases of ALL treatment and all had disturbances in rCBF. The heterogeneous cerebral hypoperfusion was however less pronounced than in the patients with symptoms. Twenty-five patients were examined during remission induction with prednisolone, doxorubicin, vincristine and IT MTX. Sixteen of these patients were first examined before start of treatment and nine during the first week. None had any neurologic symptoms but rCBF had deteriorated in all patients when re-examined after four weeks. The nine patients examined during the first week had heterogeneous cerebral hypoperfusion already at the first examination but to a lesser degree than at four weeks when the two groups showed similar results. Fourteen of the twenty-five patients were re-examined seven years later, i.e. five years after cessation of treatment. Eleven had normalized rCBF, one had improved, one was unchanged and the last one had sequelae after a stroke. Impact on CNS can also be studied by analyzing neurochemical markers of brain damage in cerebrospinal fluid (CSF). Samples were collected before start of treatment, at day 8, at day 15 and at day 29. The levels of three brain specific proteins increased during remission induction indicating damage to neurons and glia cells. Neuron-specific enolase (NSE), a marker of neurons, reached the highest level at day 8. Glia fibrillary acidic protein (GFAp), a marker of astrocytes, and the light subunit of neurofilament protein (NFp), a marker of axons, reached the highest level at day 29. Analyses of ascorbyl radical (AsR) as a marker of oxidative stress were not conclusive.
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| 11. |
- Österlundh, Gustaf, 1956, et al.
(författare)
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Studies of cerebral blood flow in children with acute lymphoblastic leukemia: case reports of six children treated with methotrexate examined by single photon emission computed tomography.
- 1997
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Ingår i: Journal of pediatric hematology/oncology : official journal of the American Society of Pediatric Hematology/Oncology. - 1077-4114. ; 19:1, s. 28-34
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Tidskriftsartikel (refereegranskat)abstract
- PURPOSE: Cranial irradiation has been widely used in order to prevent central nervous system (CNS) relapse of acute lymphoblastic leukemia (ALL) in childhood. Owing to the risk of late side effects, the Nordic Society for Pediatric Hematology and Oncology (NOPHO) replaced CNS irradiation with systemic high-dose methotrexate (HDMTX) in 1992. A prospective study of the effects of HDMTX and intrathecal MTX on CNS function is in progress at our center. PATIENTS AND METHODS: Six ALL patients underwent (99m)Tc-HMPAO single-photon emission computed tomography (SPECT) examination of regional cerebral blood flow (rCBF): three owing to neurological symptoms during treatment for ALL and the other three as part of the study. RESULTS: All the patients had various degrees of disturbed rCBF, which was more pronounced in the patients with neurological symptoms. One patient had severe symptoms and impaired rCBF after three intrathecal injections of MTX but before administration of HDMTX. CONCLUSIONS: Impaired cerebral perfusion was found in patients with and without neurological symptoms during treatment for ALL. The impact of these findings is still unknown, from both the long- and the short-term perspective. The possibility that intrathecal MTX alone or in combination with HDMTX may affect rCBF through vascular damage should be further investigated, in terms of both mechanisms and clinical significance.
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