Coexistence of orbital rhabdomyosarcoma and adrenal neuroblastic tumor in a child

2021 ◽  
Author(s):  
Jia‐Jian Hu ◽  
Huan‐Min Wang
Keyword(s):  
Neuroblastic Tumor

scholarly journals Schwann cell plasticity regulates neuroblastic tumor cell differentiation via epidermal growth factor-like protein 8

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Tamara Weiss ◽  
Sabine Taschner-Mandl ◽  
Lukas Janker ◽  
Andrea Bileck ◽  
Fikret Rifatbegovic ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Primary Repair ◽  
Nerve Repair ◽  
Peripheral Nerve Regeneration ◽  
Neuroblastoma Cells ◽  
Tumor Development ◽  
Matricellular Protein ◽  
Neuroblastic Tumor ◽  
Plastic Potential ◽  
Neuroblastic Tumors

AbstractAdult Schwann cells (SCs) possess an inherent plastic potential. This plasticity allows SCs to acquire repair-specific functions essential for peripheral nerve regeneration. Here, we investigate whether stromal SCs in benign-behaving peripheral neuroblastic tumors adopt a similar cellular state. We profile ganglioneuromas and neuroblastomas, rich and poor in SC stroma, respectively, and peripheral nerves after injury, rich in repair SCs. Indeed, stromal SCs in ganglioneuromas and repair SCs share the expression of nerve repair-associated genes. Neuroblastoma cells, derived from aggressive tumors, respond to primary repair-related SCs and their secretome with increased neuronal differentiation and reduced proliferation. Within the pool of secreted stromal and repair SC factors, we identify EGFL8, a matricellular protein with so far undescribed function, to act as neuritogen and to rewire cellular signaling by activating kinases involved in neurogenesis. In summary, we report that human SCs undergo a similar adaptive response in two patho-physiologically distinct situations, peripheral nerve injury and tumor development.

scholarly journals Identification and Pharmacological Inactivation of the MYCN Gene Network as a Therapeutic Strategy for Neuroblastic Tumor Cells

2014 ◽  
Vol 290 (4) ◽  
pp. 2198-2212 ◽  
Author(s):  
Olesya Chayka ◽  
Cosimo Walter D'Acunto ◽  
Odette Middleton ◽  
Maryam Arab ◽  
Arturo Sala
Keyword(s):  
Tumor Cells ◽  
Gene Network ◽  
Therapeutic Strategy ◽  
Neuroblastic Tumor ◽  
Mycn Gene

scholarly journals Retrospective accuracy analysis of MRI based lesion size measurement in neuroblastic tumors: which sequence should we choose?

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Sebastian Gassenmaier ◽  
Ilias Tsiflikas ◽  
Simon Maennlin ◽  
Cristian Urla ◽  
Steven W. Warmann ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Tumor Size ◽  
Three Dimensional ◽  
Lesion Size ◽  
Three Dimensions ◽  
Neuroblastic Tumor ◽  
Significant Difference ◽  
Dimensional Measurements ◽  
Neuroblastic Tumors ◽  
Mri Measurements

Abstract Background MR imaging of neuroblastic tumors is widely used for assessing the effect of chemotherapy on tumor size. However, there are some concerns that MRI might falsely estimate lesion diameters due to calcification and fibrosis. Therefore, the aim of our study was to compare neuroblastic tumor size based on MRI measurements to histopathology measurements of the resected specimens as standard of reference. Methods Inclusion criteria were diagnosis of a neuroblastic tumor, MR imaging within 100 days to surgery and gross total resection without fragmentation of the tumor between 2008 and 2019. Lesion diameters were measured by two radiologists according to RECIST 1.1 in axial plane in T2w turbo spin echo (TSE), diffusion-weighted imaging (DWI), and in T1w pre- and postcontrast sequences. Furthermore, the largest lesion size in three-dimensions was noted. The largest diameter of histopathology measurements of each specimen was used for comparison with MRI. Results Thirty-seven patients (mean age: 5 ± 4 years) with 38 lesions (neuroblastoma: n = 17; ganglioneuroblastoma: n = 11; ganglioneuroma: n = 10) were included in this retrospective study. There was excellent intra-class correlation coefficient between both readers for all sequences (> 0.9) Tumor dimensions of reader 1 based on axial MRI measurements were significantly smaller with the following median differences (cm): T1w precontrast − 1.4 (interquartile range (IQR): 1.8), T1w postcontrast − 1.0 (IQR: 1.9), T2w TSE: -1.0 (IQR: 1.6), and DWI -1.3 (IQR: 2.2) (p < 0.001 for all sequences). However, the evaluation revealed no significant differences between the three-dimensional measurements and histopathology measurements of the resected specimens regardless of the applied MRI sequence. Conclusions Axial MRI based lesion size measurements are significantly smaller than histopathological measurements. However, there was no significant difference between three-dimensional measurements and histopathology measurements of the resected specimens. T2w TSE and T1w postcontrast images provided the lowest deviation and might consequently be preferred for measurements.

scholarly journals Incidence of Horner syndrome associated with neuroblastic disease

2020 ◽  
Author(s):  
Katarzyna Kuchalska ◽  
Monika Barełkowska ◽  
Katarzyna Derwich ◽  
Katarzyna Jończyk-Potoczna ◽  
Anna Gotz-Więckowska
Keyword(s):  
Adrenal Gland ◽  
Retrospective Analysis ◽  
Surgical Procedures ◽  
Pediatric Oncology ◽  
Surgical Procedure ◽  
Pediatric Patients ◽  
Study Group ◽  
Horner Syndrome ◽  
Neuroblastic Tumor ◽  
Frequent Localization

Abstract Purpose Horner syndrome (HS) manifests in unilateral ptosis, miosis, enophthalmos, and anhedonia. It is most commonly caused by trauma or surgical procedures, but can also occur in pediatric patients as a result of tumors, especially neuroblastoma (NBL). The objective of this study was to analyze the incidence of HS in patients diagnosed with NBL. Methods A retrospective analysis of data collected at the Department of Pediatric Oncology, Hematology, and Transplantology from 2004 to 2019 was performed. The study group included 119 patients younger than 18 years old, with 62 girls and 57 boys. All of them were diagnosed with a neuroblastic tumor. Results Among the 119 patients, eight children (6.72%) were diagnosed with HS associated with NBL. Three of these patients presented to the clinic with HS, whereas HS developed after the surgical procedure to remove the tumor in four patients. The adrenal gland was the most frequent localization of the tumor. However, HS occurred more frequently in patients with mediastinum tumors. As a presenting symptom, HS occurred in 2 of 11 cases (18.18%) with mediastinum localization. All of the patients with HS were younger than 2 years old. Conclusion Investigation of the cause of isolated HS is crucial because it can be the first symptom of NBL. However, the surgical procedure itself increases the risk of HS as a complication of NBL treatment.

scholarly journals Chromosome 17 Abnormalities in Pediatric Neuroblastic Tumor With Abundant Neuropil and True Rosettes

2006 ◽  
Vol 126 (2) ◽  
pp. 277-283 ◽  
Author(s):  
Christine Fuller ◽  
Maryam Fouladi ◽  
Amar Gajjar ◽  
James Dalton ◽  
R. Alex Sanford ◽  
...  
Keyword(s):  
Chromosome 17 ◽  
Neuroblastic Tumor

Mediastinal extramedullary hematopoiesis mimicking a neuroblastic tumor in a patient with beta-thalassemia

2013 ◽  
Vol 60 (4) ◽  
pp. 711-712 ◽  
Author(s):  
Ernesto Montaruli ◽  
Francesca Marchetti Rosa ◽  
Anu Paul ◽  
Hélène Martelli ◽  
Catherine Guettier ◽  
...  
Keyword(s):  
Extramedullary Hematopoiesis ◽  
Beta Thalassemia ◽  
Neuroblastic Tumor

scholarly journals Retrospective Accuracy Analysis of MRI Based Lesion Size Measurement in Neuroblastic Tumors: Which Sequence Should We Choose?

2020 ◽  
Author(s):  
Sebastian Gassenmaier ◽  
Ilias Tsiflikas ◽  
Simon Maennlin ◽  
Cristian Urla ◽  
Steven W. Warmann ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Tumor Size ◽  
Spin Echo ◽  
Three Dimensional ◽  
Lesion Size ◽  
Three Dimensions ◽  
Neuroblastic Tumor ◽  
Significant Difference ◽  
Dimensional Measurements ◽  
Neuroblastic Tumors

Abstract Background: MR imaging of neuroblastic tumors is widely used for assessing the effect of chemotherapy on tumor size. However, there are some concerns that MRI might falsely estimate lesion diameters due to calcification and fibrosis.Therefore, the aim of our study was to compare neuroblastic tumor size based on MRI measurements to histopathology as standard of reference.Methods: Inclusion criteria were diagnosis of a neuroblastic tumor, MR imaging within 100 days to surgery and gross total resection without fragmentation of the tumor between 2008-2019. Lesion diameters were measured by two radiologists according to RECIST 1.1 in axial plane in T2w turbo spin echo (TSE), diffusion-weighted imaging (DWI) and in T1w pre- and postcontrast sequences. Furthermore, the largest lesion size in three-dimensions was noted. The largest diameter of histopathology measurements was used for comparison with MRI.Results: Thirty-seven patients (mean age: 5±4 years) with 38 lesions (neuroblastoma: n=17; ganglioneuroblastoma: n=11; ganglioneuroma: n=10) were included in this retrospective study. There was excellent intra-class correlation coefficient between both readers for all sequences(>0.9)Tumor dimensions of reader 1 based on axial MRImeasurements were significantly smaller with the following median differences (cm): T1w precontrast-1.4 (interquartile range (IQR): 1.8 ), T1w postcontrast -1.0 (IQR: 1.9),T2w TSE:-1.0 (IQR: 1.6) and DWI -1.3 (IQR: 2.2) (p<0.001 for all sequences). However, the evaluation revealed no significant differences between the three-dimensional measurements and histopathology regardless of the applied MRI sequence.Conclusions: Axial MRI based lesion size measurementsare significantly smaller than histopathological measurements. However, there was no significant difference between three-dimensional measurements and histopathology. T2w TSE and T1w postcontrast images provided the lowest deviation and might consequently be preferred for measurements.

Peripheral neuroblastic tumor of the kidney: case report and review of literature

2018 ◽  
Vol 104 (6) ◽  
pp. NP34-NP37
Author(s):  
Carla Moscheo ◽  
Alessandro Campari ◽  
Marta Giorgia Podda ◽  
Giovanna Riccipetitoni ◽  
Paola Collini ◽  
...  
Keyword(s):  
Rare Case ◽  
Adrenal Glands ◽  
Conservative Surgery ◽  
Sympathetic Ganglia ◽  
Pediatric Tumors ◽  
Review Of Literature ◽  
Case Presentation ◽  
Neuroblastic Tumor ◽  
Neuroblastic Tumors ◽  
Localized Disease

Introduction: Peripheral neuroblastic tumors (PNTs) account for 8%–10% of all pediatric tumors. Adrenal glands and sympathetic ganglia are the commonest site of tumor growth. In the clinicopathologic spectrum of PNTs, neuroblastoma and ganglioneuroma are the most primitive and the most mature tumor form, while ganglioneuroblastoma represents an intermediate state of maturation. Surgical resection is the therapy of choice in localized disease, but can lead to serious complications when performed in the presence of certain imaging-defined risk factors. Case presentation: We present a rare case of primary intrarenal ganglioneuroblastoma diagnosed in a teenager who underwent conservative surgery and, despite this, developed upper pole renal ischemia without loss of parenchymal function. Conclusion: We underline the complex management of these extremely rare cases of neuroblastic tumors, which require a dedicated multidisciplinary team.

Laparoscopic Left Adrenalectomy for Potential Neuroblastic Tumor

Videoscopy ◽  
2020 ◽  
Vol 30 (2) ◽  
Author(s):  
Zi Qin Ng ◽  
Alisha Gupta ◽  
Japinder Khosa
Keyword(s):  
Neuroblastic Tumor
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