Successful treatment of neuroblastoma in a newborn baby

Neuroblastoma is a malignant embryonic tumor of childhood originating from progenitor cells of the sympathetic nervous system. It accounts for 50% of all tumors in the first year of life with a high mortality rate. The development of the tumor process in this nosology is based on genetic disorders that may be associated with segmental breakdowns in chromosomes or a change in their number. The presence of aberrations of 1p and 11q loci serves as a criterion for determining the risk group, and amplification of the NMYC oncogene is an indicator of the aggressiveness of the disease. The main cause of death in neuroblastoma is hepatomegaly as a result of liver metastasis and the development of severe life-threatening complications. The presented case demonstrates a positive outcome of the disease in a newborn child with prognostically unfavorable factors, with early complex treatment, including polychemotherapy, surgical removal of the tumor and bone marrow transplantation.

Glioblastoma multiform with primitive neuronal component, radiological and histology features: a case report

Background Glioblastoma multiform with primitive neuronal component (GBM-PNC) has been recently defined as a rare variant of glioblastoma multiform (GBM), which shows characteristically pathological pattern of less differentiated areas with small blue cell morphology and neuroectodermic immunophenotype. New studies emphasize its characteristics and differences, which have become vitally important due to the changes in therapeutic management. Case presentation We present the case of 57-year-old male patient who onset symptoms were secondarily widespread partial seizures and expression aphasia. Brain magnetic resonance imaging (MRI) reported left enhanced temporal infiltrating lesion, requiring surgery twice throughout two years. At first surgery, pathological samples revealed embryonic tumor of the central nervous system (grade IV, WHO 2016), so PACKER protocol consisting of CSRT (craniospinal radiation) plus weekly vincristine followed by 8 cycles of cisplatin, lomustine and vincristine usually used for medulloblastomas or other primitive neuroectodermal tumors was started. However, due to reappearance of symptoms and progression in MRI, reoperation was performed with definitive diagnosis of GBM-PNC (Grade IV, WHO 2016) and switched to STUPP protocol. Conclusions It is important to take into account the chance of this entity when histological, radiological and intraoperative findings orient toward a primitive neural tumor since the presence of GBM could be overlooked leading to mistakes in diagnosis and the therapeutic orientation.

Concurrent Extramedullary Hematopoiesis and Pseudometastatic Hypervascular Lesions Masquerading as Metastases in Liver in a Case of Neuroblastoma

Neuroblastoma is a common embryonic tumor presenting in childhood. Improving treatment protocols which include a combination of chemotherapy, surgical resection, hematopoietic stem cell rescue, and radiation therapy have tremendously improved outcomes. Childhood survivors are at risk of developing lesions which may mimic metastases. It is essential to accurately diagnose these due to its prognostic implications.

WNT Signaling in Neuroblastoma

The term WNT (wingless-type MMTV integration site family) signaling comprises a complex molecular pathway consisting of ligands, receptors, coreceptors, signal transducers and transcriptional modulators with crucial functions during embryonic development, including all aspects of proliferation, morphogenesis and differentiation. Its involvement in cancer biology is well documented. Even though WNT signaling has been divided into mainly three distinct branches in the past, increasing evidence shows that some molecular hubs can act in various branches by exchanging interaction partners. Here we discuss developmental and clinical aspects of WNT signaling in neuroblastoma (NB), an embryonic tumor with an extremely broad clinical spectrum, ranging from spontaneous differentiation to fatal outcome. We discuss implications of WNT molecules in NB onset, progression, and relapse due to chemoresistance. In the light of the still too high number of NB deaths, new pathways must be considered.

ALK and neuroblastoma: from molecular genetics to clinics

Neuroblastoma (NB) is the most common extracranial embryonic tumor in children with a variety of molecular biological and clinical characteristics. There is no single molecular genetic mechanism involved in the pathogenesis of NB, which determines its heterogeneity. Pathogenetically important event in the development of NB are aberrations of ALK gene (Anaplastic lymphoma kinase), which are found in 70 % of patients with familial form of NB and in 7– 10 % of patients with sporadic cases. ALK oncogene encodes a receptor of the same name, expressed on the membrane of cells of the central and peripheral nervous system, which is in the activated state in NB. The negative effect of ALK gene anomalies on the prognosis in patients with different risk groups of NB is described. ALK gene aberrations are more often detected duringrelapse and refractory course of the disease. Because of its tissue specificity, ALK protein is an ideal target for targeted therapy. This article presents a literature review of the role of ALK in NB.

Embryonic Tumor with Abundant Neuroepithelium and True Rosettes (ETANTR): Case Report and Literature Review

The embryonal tumor with abundant neuropil and true rosettes (ETANTR) is an extremely rare variant of the primitive neuroectodermal tumor (PNET). About 80 cases have been reported since its first description in the literature, in 2000. The ETANTR occurs in very young patients, especially children under 6 years of age. It is found mainly in the cerebral cortex. Headache, focal neurological signs, seizures, increased head circumference and psychomotor developmental delay are the most frequent symptoms of ETANTR. Histologically, it displays the features of an ependymoblastoma and a neuroblastoma, showing areas of neuroepithelium fibrillar rosettes with ependymoblastic zones and interposed undifferentiated neuroepithelial cells. The ETANTR is distinguishable from other embryonal tumors due to the abundance of neuroepithelium. Genetic studies have demonstrated the presence of polysomy of chromosome 2 and chromosome 19q13.42 amplification. This is an extremely aggressive tumor with a mean survival ranging from 9 to 48 months. We present the first report in Brazil, published in indexed literature, of an ETANTR case involving a young child.

Retinoblastoma: An overview of modern management

Retinoblastoma (RB) is an embryonic tumor originating from the retinal cells. RB is the most common intraocular cancer of childhood and accounts for 4% of all pediatric malignancies.

MEDULLOBLASTOMA – CASE PRESENTATION IN AN ADULT PATIENT

The medulloblastoma is an infratentorial, rapidly growing embryonic tumor that arises in the posterior part of the cerebellar vermis and neuroepithelial roof of the fourth ventricle. It is frequent in children relatively uncommon in adults. Medulloblastoma is also named infratentorial primitive neuroectodermal tumor (PNET).We present a case of 28 year old male diagnosed with medulloblastoma in our clinic.

Metastatic Mandibular Neuroblastoma: A Rare Cause of Tooth Mobility

Neuroblastoma (NBL), a malignant embryonic tumor derived from neural crest cells, is the most common tumor worldwide among children less than 1 year of age. Metastasis to the mandible is uncommon. This article reports the case of a 15-month-old male diagnosed with NBL with bone metastasis including the mandible which resulted in severe tooth mobility. Dentists or pediatricians should consider the primary or metastatic tumors of the maxillofacial region in the differential diagnosis in children presenting with premature loss of teeth related to tooth mobility.