scholarly journals Suprasellar pleomorphic xanthoastrocytoma: A case report

2019 ◽  
Vol 10 ◽  
pp. 72 ◽  
Author(s):  
Edvin Telemi ◽  
Nikolay L. Martirosyan ◽  
Mauricio J. Avila ◽  
Ashley L. Lukefahr ◽  
Christopher Le ◽  
...  

Background: Pleomorphic xanthoastrocytoma (PXA) is a rare form of astrocytic neoplasm most commonly found in children and young adults. This neoplasm, which is classified as a Grade II tumor by the World Health Organization classification of tumors of the central nervous system, carries a relatively favorable outcome. It is usually found supratentorially in cortical regions of the cerebral hemispheres, and as such, presenting symptoms are similar to other supratentorial cortical neoplasms; with seizures being a common initial symptom. Due to the rarity of this type of neoplasm, PXA arising elsewhere in the brain is often not included in the initial differential diagnosis. Case Description: This report presents an extremely rare patient with PXA arising in the suprasellar region who presented with progressive peripheral vision loss. Magnetic resonance imaging of the brain demonstrated a heterogeneous suprasellar mass with cystic and enhancing components initially; the most likely differential diagnosis was craniopharyngioma. The patient underwent endoscopic endonasal resection of the tumor. Microscopically, the tumor was consistent with a glial neoplasm with variable morphology. Based on these findings along with further immunohistochemical workup, the patient was diagnosed with a PXA arising in the suprasellar region. At the 1-year follow-up, the patient remained free of recurrence. Although rare PXA originating in other uncommon locations, such as the spinal cord, cerebellum, the ventricular system, and the pineal region have been previously described. Conclusion: Although rare, PXA should be included in the differential diagnosis for solid-cystic tumors arising in the suprasellar region in young adults.

Author(s):  
Brian P O’Neill ◽  
Jeffrey Allen ◽  
Mitchell S. Berger ◽  
Rolf-Dieter Kortmann

Pilocytic astrocytoma (PA) (World Health Organization (WHO) grade I). A relatively circumscribed, slow-growing, often cystic astrocytoma occurring in children and young adults, histologically characterized by a biphasic pattern with varying proportions of compacted bipolar cells associated with Rosenthal fibres and loose-textured multipolar cells associated with microcysts and eosinophilic granular bodies. Most PAs are localized, macrocystic, and only marginally infiltrative. However some PAs, such as those arising in the optic pathways, are rarely cystic and may have an extensive infiltrative pattern but within a neuroanatomic pathway. Pleomorphic xanthoastrocytoma (PXA) (WHO grade II). An astrocytic neoplasm with a relatively favourable prognosis, typically encountered in children and young adults, with superficial location in the cerebral hemispheres and involvement of the meninges; characteristic histological features include pleomorphic and lipidized cells expressing glial fibrillary acidic protein and often surrounded by a reticulin network as well as eosinophilic granular bodies. Subependymal giant cell astrocytoma (SEGA) (WHO grade I). A benign, slow-growing tumour typically arising in the wall of the lateral ventricles and composed of large ganglioid astrocytes. It is the most common CNS neoplasm in patients with tuberous sclerosis.


2020 ◽  
Vol 24 (3) ◽  
pp. 63-75
Author(s):  
Yu. A. Stepanova ◽  
M. Z. Alimurzaeva ◽  
D. A. Ionkin

The incidence of focal lesions in the spleen is 3.2–4.2% per 100,000 population. Spleen cysts are rare (incidence 0.75 per 100,000). These are single or multiple, thin- and smooth-walled cavities filled with a transparent liquid. Distinguish between primary (or true) cysts, lined with epithelium, and secondary (or false), devoid of epithelial lining. Among the primary cysts, there are congenital cysts formed in the embryonic period due to the migration of peritoneal cells into the spleen tissue, dermoid and epidermoid cysts. A special group of primary cysts are parasitic cysts. Cystic tumors of the spleen include lymphangioma and lymphoma.The main difficulties in the diagnosis and differential diagnosis of cysts and cystic tumors of the spleen are associated with the rarity of this pathology and, as a consequence, a small number of works, including a significant number of the cases. However, in those works where a large number of the cases are described, most often this is one morphological form and an analysis of its various characteristics.Purpose. Based on the analysis of our own examination data of a significant number of patients with cysts and cystic tumors of the spleen, to assess the possibility of differential diagnosis of individual morphological forms according to ultrasound data.Materials and methods. 323 patients with cysts and cystic tumors of the spleen from 15 to 77 years old (men – 105 (32.5%); women – 218 (67.5%) were treated at A.V. Vishnevsky National Medical Research Center of Surgery for the period from 1980 to 2020. All patients underwent ultrasound during examination. Surgical treatment was carried out in various ways – (85.1%), when making a preoperative diagnosis of an uncomplicated spleen cyst of small size, dynamic observation was carried out (verification by puncture biopsy data).Results. Morphological verification of cysts and cystic tumors of the spleen was presented as follows (taking into account possible difficulties in identifying the epithelial lining): true cyst – 182 (56.4%); dermoid cyst – 3 (0.9%) (malignant – in 1 case); pseudocyst – 16 (5.0%); pancreatogenic – 34 (10.5%); echinococcus – 52 (16.1%); lymphangioma – 24 (7.4%); lymphoma – 10 (3.1%); ovarian cancer metastasis – 2 (0.6%). The article describes the ultrasound signs of the above forms of the lesions with an emphasis on the complexity of diagnosis.Conclusions. Primary and parasitic spleen cysts are well differentiated according to ultrasound; false cysts of the spleen, depending on the cause of their occurrence, can create difficulties in their identification and differentiation (they require careful dynamic control); cystic tumors of the spleen should be differentiated from malignant tumors and metastases of a cystic structure, as a result of which such vigilance should always be present when they are detected.


2020 ◽  
Vol 10 (01) ◽  
pp. e134-e136
Author(s):  
Nida Mirza ◽  
Smita Malhotra ◽  
Anupam Sibal

AbstractProgressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of autosomal recessive disorders of childhood which presents with intermittent or progressive episodes of cholestasis, with jaundice and pruritus as most common presenting symptoms. PFIC type 3 occurs due to mutations in the ABCB4 gene, mutation in this gene has wide spectrum of features which include intrahepatic stones, cholelithiasis, PFIC type 3, and intrahepatic cholestasis of pregnancy. Here, we are reporting a peculiar case of young male adolescent with novel variant compound heterozygote missense mutation in ABCB4 gene who had gall stone as initial symptom, followed by symptoms of PFIC and eventually decompensated chronic liver disease.


2012 ◽  
Vol 2012 ◽  
pp. 1-4
Author(s):  
B. Dhamija ◽  
D. Kombogiorgas ◽  
I. Hussain ◽  
G. A. Solanki

Background. Primary hyperparathyroidism in childhood is a very rare entity, often being diagnosed late after the onset of its presenting symptoms. It most commonly affects patients in their fourth decade of life and beyond. The inclusion of primary hyperparathyroidism in the differential diagnosis is necessary when evaluating patients presenting with nonspecific symptoms such as polyuria, fatigue, weight loss, abdominal pain, nausea, and vomiting.Methods. We report the case of an eleven-year-old girl presenting with three years history of headaches, visual disturbance, along with episodes of emotional lability. Neuroimaging confirmed a large posterior fossa arachnoid cyst. It was decided to manage this lesion conservatively with surveillance. Only after further hospital admissions with recurrent loss of consciousness, dizziness, and nausea to add to her already existing symptoms, a full biochemical and endocrine assessment was performed to look for more specific causes for her presentation. These pointed to a diagnosis of primary hyperparathyroidism.Conclusions. The inclusion of primary hyperparathyroidism in the differential diagnosis should be considered when evaluating paediatric patients presenting with nonspecific (neurological, gastrointestinal, and renal) symptoms in order to establish a prompt diagnosis of the disorder and to avoid severe complications of prolonged hypercalcaemia and end-organ damage.


1984 ◽  
Vol 29 (2) ◽  
pp. 132-134 ◽  
Author(s):  
L.B. Raschka

Most violence connected with sleep disorder is assumed to be related to sleep walking. It is less well known that other sleep disorders can also give rise to violence. The role of narcolepsy in car accidents is mentioned. Sleep drunkenness can lead to confusion resulting in violent behaviour especially on forced awakening. This condition is associated to sleep apnea. Primary or central sleep apnea is caused by disorders of the brain stem affecting the respiratory center. Secondary or upper airway sleep apnea can be caused by virtually any condition that results in cessation of the airflow due to occlusion of the upper airway. The author describes one patient who engaged in assaultive behaviour on forced awakening following earlier alcohol consumption. The pathomechanism of violent behaviour generated by a combination of sleep apnea and respiratory pathology is described. The differential diagnosis, prevention and treatment is outlined. The use of polysomnography in diagnosis and the potentially dangerous effects of drugs with respiratory depressing effects is highlighted.


2009 ◽  
Vol 133 (3) ◽  
pp. 423-438 ◽  
Author(s):  
Olca Basturk ◽  
Ipek Coban ◽  
N. Volkan Adsay

Abstract Context.—Cystic lesions of the pancreas are being recognized with increasing frequency and have become a more common finding in clinical practice because of the widespread use of advanced imaging modalities and the sharp drop in the mortality rate of pancreatic surgery. Consequently, in the past 2 decades, the nature of many cystic tumors in this organ has been better characterized, and significant developments have taken place in the classification and in our understanding of pancreatic cystic lesions. Objective.—To provide an overview of the current concepts in classification, differential diagnosis, and clinical/biologic behavior of pancreatic cystic tumors. Data Sources.—The authors' personal experience, based on institutional and consultation materials, combined with an analysis of the literature. Conclusions.—In contrast to solid tumors, most of which are invasive ductal adenocarcinomas with dismal prognosis, cystic lesions of the pancreas are often either benign or low-grade indolent neoplasia. However, those that are mucinous, namely, intraductal papillary mucinous neoplasms and mucinous cystic neoplasms, constitute an important category because they have well-established malignant potential, representing an adenoma-carcinoma sequence. Those that are nonmucinous such as serous tumors, congenital cysts, lymphoepithelial cysts, and squamoid cyst of pancreatic ducts have no malignant potential. Only rare nonmucinous cystic tumors that occur as a result of degenerative/necrotic changes in otherwise solid neoplasia, such as cystic ductal adenocarcinomas, cystic pancreatic endocrine neoplasia, and solid-pseudopapillary neoplasm, are also malignant and have variable degrees of aggressiveness.


PEDIATRICS ◽  
1962 ◽  
Vol 30 (6) ◽  
pp. 1018-1018

Cystic Fibrosis—NCFRF. 16 mm., color, sound, showing time 32 minutes. Produced in 1961 by Samuel L. Schulman for the National Cystic Fibrosis Research Foundation, medical supervision by Giulio J. Barbero, M.D. Philadelphia. Procurable on purchase from National Cystic Fibrosis Research Foundation, 521 Fifth Avenue, New York City 17. Procurable on loan from American Medical Association, Motion Picture Library, 535 N. Dearborn Street, Chicago 10. This film has been prepared to aid physicians in making a correct diagnosis and to instruct them in the techniques currently being used to prolong life in cases of cystic fibrosis. Following the introductoy remarks there is a good discussion of the presenting symptoms, the differential diagnosis, the multiple system involvement, hereditary aspects, therapy, and prognosis. The film brings out well the panexocrine involvement, the clinical variability, and the fact that the disease is not an all or none phenomenon but rather a disease of all grades of severity and is a disease in which no single test is applicable to the exclusion of others. The diagrams and patient demonstrations are good. Perhaps too much review of older methods of therapy and diagnosis is given, but this serves as a background for the newer recommended procedures. More emphasis could have been given to the tremendous burden, both financial and emotional, this disease is on parents. This is an excellent instructive film and it emphasizes the many problems of cystic fibrosis as related to the diagnostic tools available and to forms of therapy. The photography and sound are satisfactory. It is recommended for pediatricians, general practitioners, house staff, and medical students.


2021 ◽  
Vol 19 ◽  
Author(s):  
Mohamed Said Boulkrane ◽  
Victoria Ilina ◽  
Roman Melchakov ◽  
Mikhail Arisov ◽  
Julia Fedotova ◽  
...  

: The World Health Organization declared the pandemic situation caused by SARS-CoV-2 (Severe Acute Respiratory Syndrome Coronavirus-2) in March 2020, but the detailed pathophysiological mechanisms of Coronavirus disease 2019 (COVID-19) are not yet completely understood. Therefore, to date, few therapeutic options are available for patients with mild-moderate or serious disease. In addition to systemic and respiratory symptoms, several reports have documented various neurological symptoms and impairments of mental health. The current review aims to provide the available evidence about the effects of SARS-CoV-2 infection on mental health. The present data suggest that SARS-CoV-2 produces a wide range of impairments and disorders of the brain. However, a limited number of studies investigated the neuroinvasive potential of SARS-CoV-2. Although the main features and outcomes of COVID-19 are linked to severe acute respiratory illness. The possible damages on the brain should be considered, too.


2018 ◽  
Author(s):  
Barbara Dworetzky ◽  
Jong Woo Lee

Epilepsy is a chronic disorder of the brain characterized by recurrent unprovoked seizures. A seizure is a sudden change in behavior that is accompanied by electrical discharges in the brain. Many patients presenting with a first-ever seizure are surprised to find that it is a very common event. A reversible or avoidable seizure precipitant, such as alcohol, argues against underlying epilepsy and therefore against treatment with medication. This chapter discusses the epidemiology, etiology, and classification of epilepsy and provides detailed descriptions of neonatal syndromes, syndromes of infancy and early childhood, and syndromes of late childhood and adolescence. The pathophysiology, diagnosis, and differential diagnosis are described, as are syncope, migraine, and psychogenic nonepileptic seizures. Two case histories are provided, as are sections on treatment (polytherapy, brand-name versus generic drugs, surgery, stimulation therapy, dietary treatments), complications of epilepsy and related disorders, prognosis, and quality measures. Special topics discussed are women?s issues and the elderly. Figures illustrate a left midtemporal epileptic discharge, wave activity during drowsiness, cortical dysplasias, convulsive syncope, rhythmic theta activity, right hippocamal sclerosis, and right temporal hypometabolism. Tables describe international classifications of epileptic seizures and of epilepsies, epilepsy syndromes and related seizure disorders, differential diagnosis of seizure, differentiating epileptic versus nonepileptic seizures, antiepileptic drugs, status epilepticus protocol for treatment, when to consider referral to a specialist, and quality measures in epilepsy.  This review contains 7 figures, 10 tables, and 33 references. Key Words: Seizures, focal (partial)seizure, generalized seizures, Myoclonic seizures, Atonic seizures, Concurrent electromyographyTonic-clonic (grand mal) seizures


1998 ◽  
Vol 56 (4) ◽  
pp. 803-807 ◽  
Author(s):  
PAULO HENRIQUE AGUIAR ◽  
WEI LIU CHING ◽  
HELIO LEITÃO ◽  
F. ISSA ◽  
GUILHERME LEPSKI ◽  
...  

Cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome is a condition characterized by seizures, facial asymmetry, contralateral hemiplegia or hemiparesis, and mental retardation. These findings are due to cerebral injury that may occur early in life or in utero. The radiological features are unilateral loss of cerebral volume and associated compensatory bone alterations in the calvarium, like thickening, hyperpneumatization of the paranasal sinuses and mastoid cells and elevation of the petrous ridge. The authors describe three cases. Classical findings of the syndrome are present in variable degrees according to the extent of the brain injury. Pathogenesis is commented.


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