scholarly journals Aggressive pituitary lesion with a remarkably high Ki-67

2014 ◽  
Vol 58 (6) ◽  
pp. 656-660 ◽  
Author(s):  
Pedro Marques ◽  
Manuela Mafra ◽  
Carlos Calado ◽  
Anabela Martins ◽  
Joaquim Monteiro ◽  
...  

The uncommon aggressive pituitary tumors are named carcinomas when metastases are detected, either in the central nervous system and/or systemically. Some cases are associated with hormonal overproduction, but most are diagnosed because of local symptoms. These neoplasias are generally refractory to current treatments. A 51 year-old woman presented sudden onset of headache, left arm paresis and left facial hypoesthesia. Computed tomography scan and magnetic resonance imaging revealed a pituitary tumor invading the left sphenoidal and cavernous sinuses. Laboratory data excluded hormonal hypersecretion. The patient underwent transsphenoidal surgery and histological findings showed a neoplasia with Ki-67 estimated at 75%. Medical imaging excluded both a primary occult tumor and central nervous system or systemic dissemination. Three weeks postoperatively, neurological condition worsened, with new onset of ataxia, bilateral ptosis, ophthalmoplegia and an increase in the size of the lesion, leading to surgical intervention by craniotomy, followed by only a few sessions of radiotherapy, because of severe disease progression. Patient died nearly 2 months after the initial manifestations. This case illustrates the aggressiveness of some pituitary lesions, the limited efficacy of current treatment modalities such as surgery or radiotherapy and the pitfalls of the current pituitary tumors classification. To our knowledge, this case corresponds to one of the most aggressive pituitary neoplasms reported so far, with a very high Ki-67 index (75%) and short survival (2 months). Ki-67 index could be of prognostic value in pituitary tumors. Pituitary tumors World Health Organization (WHO) classification could be revisited.

Author(s):  
Katja Bender ◽  
Eilís Perez ◽  
Mihaela Chirica ◽  
Julia Onken ◽  
Johannes Kahn ◽  
...  

Abstract Purpose High-grade astrocytoma with piloid features (HGAP) is a recently described brain tumor entity defined by a specific DNA methylation profile. HGAP has been proposed to be integrated in the upcoming World Health Organization classification of central nervous system tumors expected in 2021. In this series, we present the first single-center experience with this new entity. Methods During 2017 and 2020, six HGAP were identified. Clinical course, surgical procedure, histopathology, genome-wide DNA methylation analysis, imaging, and adjuvant therapy were collected. Results Tumors were localized in the brain stem (n = 1), cerebellar peduncle (n = 1), diencephalon (n = 1), mesencephalon (n = 1), cerebrum (n = 1) and the thoracic spinal cord (n = 2). The lesions typically presented as T1w hypo- to isointense and T2w hyperintense with inhomogeneous contrast enhancement on MRI. All patients underwent initial surgical intervention. Three patients received adjuvant radiochemotherapy, and one patient adjuvant radiotherapy alone. Four patients died of disease, with an overall survival of 1.8, 9.1, 14.8 and 18.1 months. One patient was alive at the time of last follow-up, 14.6 months after surgery, and one patient was lost to follow-up. Apart from one tumor, the lesions did not present with high grade histology, however patients showed poor clinical outcomes. Conclusions Here, we provide detailed clinical, neuroradiological, histological, and molecular pathological information which might aid in clinical decision making until larger case series are published. With the exception of one case, the tumors did not present with high-grade histology but patients still showed short intervals between diagnosis and tumor progression or death even after extensive multimodal therapy.


PEDIATRICS ◽  
1958 ◽  
Vol 22 (4) ◽  
pp. 675-688
Author(s):  
Fred T. Matthes ◽  
Rudolph Kirschner ◽  
Martha D. Yow ◽  
James C. Brennan

Four cases of pneumonitis associated with the inhalation of mercury vapor are described in detail. Three of the four cases were fatal. The illness was characterized by the sudden onset of tachypnea, cough, fever, gastrointestinal disturbances and central nervous system manifestations. Ordinary laboratory tests were not helpful in establishing the diagnosis. Quantitative analysis of the urine for mercury was suggestive but not diagnostic. Roentgenograms of the lungs revealed interstitial pneumonitis. Analysis of tissue for mercury in the lungs, liver and spleen of two of the cases showed substantially increased mercury content. The outstanding pathologic features in the three fatal cases were erosive bronchitis and bronchiolitis, with severe interstitial pneumonitis. The presently accepted mode of therapy for acute poisoning due to ingestion of mercury is early use of BAL. Its use is probably also indicated in acute poisoning due to inhalation of mercury vapor. Supportive care in the form of oxygen, mist, and parenteral fluids is indicated.


2021 ◽  
Vol 10 (Supplement_2) ◽  
pp. S8-S8
Author(s):  
Jaimee M Hall ◽  
Peter L Havens ◽  
Errin A Mitchell ◽  
Gabriel N De Vela ◽  
Lauren L Titus ◽  
...  

Abstract Background Blastomycosis is an endemic mycosis of immunocompetent individuals, typically seen after exposure to wooded areas near rivers, lakes, and streams in rural locations, and often not considered a disease of urban environments. Disease can be isolated to lung, or disseminate to skin, bone, or central nervous system. Factors influencing disease acquisition and severity in children are unknown. Methods We analyzed acquisition risk factors and disease characteristics of blastomycosis in children treated at a tertiary care center from 1998–2018 to identify exposure source and measure disease severity, to identify cases without “typical exposure”, and to measure the effect of race on disease severity. Results Of 64 children, median age was 13.3 years, 72% were male, 38% resided in urban counties, and 50% had typical environmental exposure. Isolated pulmonary infection occurred in 33 (52%). The remaining children had evidence of dissemination including skin (N=13), bone (N=16; 7 clinically silent), and central nervous system (N=7; 3 clinically silent). Infection was moderate/severe in 19 (30%). Two children (3%) died. 79% of children with moderate/severe disease (p=0.008) and 71% of urban children (p=0.007) had no typical environmental exposure. Comparing children from urban counties to other residences, 63% versus 5% were black (p<0.001) and 71% versus 35% developed extrapulmonary dissemination (p=0.006). Moderate/severe disease occurred in 7/17 (42%) black and 12/47 (26%) children of other race (p=0.23). Conclusions Blastomycosis, endemic in urban children in the absence of typical exposure history, has frequent, sometimes clinically silent, extrapulmonary dissemination, with a trend toward more severe disease in black children.


2014 ◽  
Vol 33 (04) ◽  
pp. 347-351
Author(s):  
Pedro Tadao Hamamoto Filho ◽  
Vitor César Machado ◽  
Flávio Ramalho Romero ◽  
Luis Gustavo Ducati ◽  
Marco Antônio Zanini ◽  
...  

AbstractBrain metastases are the most common tumors within the central nervous system. Recent advances on diagnosis and treatment modalities have allowed for longer survival. In this paper we review the indication of each modality of treatment: surgery, whole brain radiotherapy and stereotactic radiosurgery, as also recent advances on the knowledge of brain metastases biology that may improve the use of medical treatment and chemotherapy.


2002 ◽  
Vol 76 (22) ◽  
pp. 11688-11703 ◽  
Author(s):  
Lucia Labrada ◽  
Xiao Huan Liang ◽  
Wei Zheng ◽  
Christine Johnston ◽  
Beth Levine

ABSTRACT Several different mammalian neurotropic viruses produce an age-dependent encephalitis characterized by more severe disease in younger hosts. To elucidate potential factors that contribute to age-dependent resistance to lethal viral encephalitis, we compared central nervous system (CNS) gene expression in neonatal and weanling mice that were either mock infected or infected intracerebrally with a recombinant strain, dsTE12Q, of the prototype alphavirus Sindbis virus. In 1-day-old mice, infection with dsTE12Q resulted in rapidly fatal disease associated with high CNS viral titers and extensive CNS apoptosis, whereas in 4-week-old mice, dsTE12Q infection resulted in asymptomatic infection with lower CNS virus titers and undetectable CNS apoptosis. GeneChip expression comparisons of mock-infected neonatal and weanling mouse brains revealed developmental regulation of the mRNA expression of numerous genes, including some apoptosis regulatory genes, such as the proapoptotic molecules caspase-3 and TRAF4, which are downregulated during development, and the neuroprotective chemokine, fractalkine, which is upregulated during postnatal development. In parallel with increased neurovirulence and increased viral replication, Sindbis virus infection in 1-day-old mice resulted in both a greater number of host inflammatory genes with altered expression and greater changes in levels of host inflammatory gene expression than infection in 4-week-old mice. Only one inflammatory response gene, an expressed sequence tag similar to human ISG12, increased by a greater magnitude in infected 4-week-old mouse brains than in infected 1-day-old mouse brains. Furthermore, we found that enforced neuronal ISG12 expression results in a significant delay in Sindbis virus-induced death in neonatal mice. Together, our data identify genes that are developmentally regulated in the CNS and genes that are differentially regulated in the brains of different aged mice in response to Sindbis virus infection.


2015 ◽  
Vol 5 ◽  
pp. 41
Author(s):  
Sanghyeon Kim ◽  
Myongjin Kang ◽  
Sunseob Choi ◽  
Dae Cheol Kim

Pilomyxoid astrocytoma (PMA) is a rare central nervous system tumor that has been included in the 2007 World Health Organization Classification of Tumors of the Central Nervous System. Due to its more aggressive behavior, PMA is classified as Grade II neoplasm by the World Health Organization. PMA predominantly affects the hypothalamic/chiasmatic region and occurs in children (mean age of occurrence = 10 months). We report a case of a 24-year-old man who presented with headache, nausea, and vomiting. Brain CT and MRI revealed a mass occupying only the third ventricle. We performed partial resection. Histological findings, including monophasic growth with a myxoid background, and absence of Rosenthal fibers or eosinophilic granular bodies, as well as the strong positivity for glial fibrillary acidic protein were consistent with PMA.


Author(s):  
Martina Piloni ◽  
Filippo Gagliardi ◽  
Michele Bailo ◽  
Lina Raffaella Barzaghi ◽  
Marcella Callea ◽  
...  

Abstract Background Occurrences of suprasellar central nervous system (CNS) embryonal tumors in adults are extremely rare. Hemorrhagic onset is further uncommon, with only anecdotic cases reported in the literature. The authors describe the case of a 57-year-old man affected by a suprasellar CNS embryonal tumor, with hemorrhagic onset and a unique diffusion pattern along the optic pathways. Material and Methods A 57-year-old man presenting with acute visual acuity worsening and left homonymous hemianopia was referred to our hospital. Neuroradiologic studies demonstrated an infiltrating, high-grade lesion involving the optic chiasm and right retrochiasmatic pathways with a hemorrhagic area in the ipsilateral pulvinar. Results The patient underwent microsurgical biopsy. Pathologic assessment confirmed the diagnosis of CNS embryonal tumor, not otherwise specified (NOS) according to the 2016 World Health Organization (WHO) classification of CNS tumors. The patient was referred to a multimodal adjuvant treatment; he eventually died 4 months after surgery. Competent literature has been systematically reviewed in the light of the relevant changes made in the last version of the WHO classification. Conclusion Embryonal tumors should be considered in the differential diagnosis for sellar and suprasellar space-occupying lesions, despite the rarity of the disease and the uncommon features at time of presentation. As per our knowledge, this is the first case ever described of hemorrhagic suprasellar embryonal tumor with a diffusion pattern along white matter fibers. Histogenesis, biomolecular and neuroradiologic features, and classification of embryonal tumors are an open field of research, with considerable implications for the definition of better diagnostic pitfalls and therapeutic regimens.


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