Publications by authors named "Vicente Santa-Maria Lopez"

Purpose: Infant and young childhood medulloblastoma (iMB) is usually treated without craniospinal irradiation (CSI) to avoid neurocognitive late effects. Unfortunately, many children relapse. The purpose of this study was to assess salvage strategies and prognostic features of patients with iMB who relapse after CSI-sparing therapy.

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Malignant rhabdoid tumors (MRT) predominantly affect infants and young children. Patients below six months of age represent a particularly therapeutically challenging group. Toxicity to developing organ sites limits intensity of treatment.

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Purpose: Pediatric low-grade gliomas are the most frequent brain tumors in children. The standard approach for symptomatic unresectable tumors is chemotherapy. Recently, key molecular alterations/pathways have been identified and targeted drugs developed and tested in clinical trials.

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Article Synopsis
  • Histiocytoses are blood disorders linked to mutations in certain kinases, particularly BRAF and MEK1/MEK2.
  • The causes of histiocytosis in patients are still unclear, and alternative treatment options beyond BRAF and MEK remain undefined.
  • This study found that mutations in CSF1R and changes in RET and ALK lead to significant responses to specific drugs, selpercatinib and crizotinib, in patients with histiocytosis.
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  • Diffuse intrinsic pontine glioma (DIPG) is a rare brainstem cancer with a poor survival rate of around 11 months, prompting a need for standardized treatment guidelines in Europe.
  • A survey involving 74 healthcare professionals revealed that most preferred radiotherapy, but treatment strategies varied widely, especially at disease progression, with many opting for no treatment after the second progression.
  • The findings highlight the inconsistency in treatment approaches and the necessity for international consensus guidelines to improve DIPG management through collaborative clinical trials.
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Choroid plexus tumors (CPT) can present in the baseline magnetic resonance imaging (MRI) with lesions compatible with leptomeningeal dissemination. Therapeutic strategy in this condition is controversial. We present a case of an infant with CPP and significant diffuse leptomeningeal contrast enhancement at diagnosis, which spontaneously resolved after removal of the primary tumor.

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Article Synopsis
  • Langerhans cell histiocytosis (LCH) is a rare disease where some immune cells called histiocytes go a bit crazy, leading to problems, especially when affecting many parts of the body.
  • Some patients with LCH can develop a serious condition called HLH, which causes too much inflammation in the body, but not much is known about it.
  • A study showed that about 9.3% of children and young adults with LCH had HLH, and those with it had a lower chance of surviving long-term compared to those who didn’t.
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OBJECTIVEDiffuse intrinsic pontine glioma (DIPG) is a highly aggressive and lethal brainstem tumor in children. In the 1980s, routine biopsy at presentation was abandoned since it was claimed "unnecessary" for diagnosis. In the last decade, however, several groups have reincorporated this procedure as standard of care or in the context of clinical trials.

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Background And Objective: Diffuse intrinsic pontine glioma (DIPG) is a lethal brainstem tumor in children. Dendritic cells (DCs) have T-cell stimulatory capacity and, therefore, potential antitumor activity for disease control. DCs vaccines have been shown to reactivate tumor-specific T cells in both clinical and preclinical settings.

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