scholarly journals MPC-7 Clinical features of Diffuse hemispheric glioma, H3 G34-mutant in children and young adults

2021 ◽  
Vol 3 (Supplement_6) ◽  
pp. vi17-vi17
Author(s):  
Yuki Takeshima ◽  
Jyun-Ichiro Kuroda ◽  
Tatsuya Kawano ◽  
Jin Matsuura ◽  
Hiroaki Matsuzaki ◽  
...  

Abstract INTRODUCTION: H3F3A G34R/V mutated gliomas are seen predominantly in children and young adults, and have been proposed as “Diffuse hemispheric glioma, H3 G34-mutant” in cIMPACT-NOW Update 6. However, the clinical features of the tumor have not been fully elucidated. METHODS: We retrospectively reviewed 4 cases with H3G34R mutation among 40 cases diagnosed as glioblastoma under 30 years old or primitive neuroectodermal tumor (PNET) in our hospital. RESULTS: There were one male and three female patients with a median age of 21.5 years (range: 17–27 years). All lesions were localized in the cerebral hemispheres, and the initial symptoms were headache in two cases and seizures in two cases. On imaging, there was one case with poor contrast, and unlike the infiltrative growth pattern of the other three contrasted cases, it showed a well-defined mass lesion. DWI showed high signal in all four cases, reflecting the high cell density in histopathology. All cases were IDH-wildtype. CONCLUSION: Although the patient background and genetic characteristics of the glioma with H3 G34R/V mutation at our institution were generally consistent with previous reports, there were some cases with atypical imaging findings. Further investigation is required for a deeper understanding of the clinical features of this tumor.

2005 ◽  
Vol 129 (1) ◽  
pp. 107-110 ◽  
Author(s):  
Raoulin Soulard ◽  
Valère Claude ◽  
Philippe Camparo ◽  
Jean-Philippe Dufau ◽  
Patrick Saint-Blancard ◽  
...  

Abstract Ewing sarcoma/primitive neuroectodermal tumor is classically a tumor of the soft tissue or bone in children and young adults, but several cases have been described in patients of all ages. Within the last decade, the clinicopathologic spectrum of Ewing sarcoma/primitive neuroectodermal tumor has been markedly expanded by recognition that the tumor may also have a visceral origin. We describe a case of primitive neuroectodermal tumor arising in the stomach of a 66-year-old woman. The neoplasm was excised using a radical surgical procedure. Microscopically, the tumor was made up of solid nests and sheets of round cells. Immunohistochemically, the tumor cells showed immunoreactivity for CD99, S100, neuron-specific enolase, and vimentin. A multiplex real-time polymerase chain reaction assay detected an EWS-ERG fusion. To our knowledge, this is the first description of a primitive neuroectodermal tumor arising in the stomach.


2013 ◽  
Vol 88 (6 suppl 1) ◽  
pp. 113-115 ◽  
Author(s):  
Carla Andréa Avelar Pires ◽  
Brena Andrade de Sousa ◽  
Gabriela Athayde Amin ◽  
Maraya de Jesus Semblano Bittencourt ◽  
Mario Fernando Ribeiro de Miranda ◽  
...  

Tufted angioma is an acquired vascular proliferation with specific histological characteristics. The most common clinical features are erythematous macules. It occurs predominantly in children and young adults, especially in the chest and neck. We report the case of a male patient, 12 years old, with an increased right ear auricle associated with erythematous macules and increased local temperature, referred with a diagnosis of lepromatous leprosy; however, this diagnosis was questioned. Histopathology was consistent with tufted angioma.


1999 ◽  
Vol 21 (4) ◽  
pp. 309
Author(s):  
K. Klopfenstein ◽  
K. Mutabagani ◽  
S. Teich ◽  
J. Groner ◽  
A. Rauck ◽  
...  

Author(s):  
Mohammadreza Emamhadi ◽  
Sama Noroozi Guilandehi ◽  
Nooshin Zaresharifi ◽  
Iraj Baghi ◽  
Alireza Mehrvarz ◽  
...  

Primitive neuroectodermal tumor (PNET) is a highly aggressive tumor and mostly develops in children and young adults. PNETs of peripheral nerves are uncommon. Ulnar nerve, in particular, is an extremely peculiar origin for PNET and to the best of our knowledge only few well-documented cases have been yet reported.


2016 ◽  
Vol 47 (2) ◽  
pp. 173-176
Author(s):  
Sumit Mehndiratta ◽  
Ritika Singhal ◽  
Krishnan Rajeshwari ◽  
Anand P Dubey

Dengue fever has classically been described as a disease of children and young adults. Infants are naturally protected by virtue of maternally derived immunoglobulins, especially in endemic countries. The resurgence of dengue, coupled with the availability of early and sensitive diagnostic methods and a high degree of clinical suspicion, has led to an increasing number of infants being diagnosed. There is a wide spectrum of clinical manifestations, particularly in infancy. Here we describe three cases presenting with diverse clinical features, their subsequent management and outcome.


2015 ◽  
Vol 143 (9-10) ◽  
pp. 619-622
Author(s):  
Helena Maric ◽  
Radovan Cvijanovic ◽  
Igor Ivanov ◽  
Ljiljana Gvozdenovic ◽  
Dejan Ivanov ◽  
...  

Introduction. Primitive neuroectodermal tumor or Ewing?s sarcoma is a tumor of undifferentiated small round cells that arise from the soft tissues, and is believed to be of neural origin. It occurs most often in children, followed by adolescents and young adults. Case Outline. A case of a 24-year-old patient with ulcerostenosans Ewing?s sarcoma of the initial part of the small intestine is presented in our paper. Reviewing the literature and using as an example the case of a female patient with signs of sideropenic anemia caused by primitive neuroectodermal tumor of the small intestine, an attempt was made to clarify the etiology, clinical presentation, diagnosis and therapy with the aim of its rapid detection and treatment. Conclusion. Mesenteric primitive neuroectodermal tumor is a rare neoplasm in adults, while it usually occurs in children and young adults. Surgical resection of the lesions with the application of chemotherapy is the main form of treatment of patients suffering from this disease.


Author(s):  
Jenny Weinbrand-Goichberg ◽  
Efrat Ben Shalom ◽  
Choni Rinat ◽  
Sapir Choshen ◽  
Shimrit Tzvi-Behr ◽  
...  

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