This site needs JavaScript to work properly. 10.1016/S0140-6736(04)17594-6. Contrast enhancement may be present and a focal cortical dysplasia is commonly associated with it. However, there have been incidents where the tumour was malignant. The prognosis after surgery is favourable. A case of recurrent epilepsy-associated rosette-forming glioneuronal tumor with anaplastic transformation in the absence of therapy. Long-Term Seizure Outcomes and Predictors in Patients with Dysembryoplastic Neuroepithelial Tumors Associated with Epilepsy. Neuronal markers (synaptophysin, neuron- specific enolase) and glial markers (GFAP, S-100) are positive. As our patient refused to have a cerebral biopsy, we decided to perform a complementary imaging exploration, which could offer us more details about the tumor.
Seizures in children with dysembryoplastic neuroepithelial tumors of Rugg-Gunn FJ, Simister RJ, Squirrell M, Holdright DR, Duncan JS: Cardiac arrhythmias in focal epilepsy: a prospective long-term study. Epub 2019 Sep 11. 2009, 9 (22): 16-18.
Watch and Wait | The Brain Tumour Charity There is little correlation between the lesion site and epileptogenic foci of the ictal onset zone as well as the irritative zone. This article is published under license to BioMed Central Ltd. Updated August 2016. PathologyOutlines.com website. For patients with breast cancer who are older but physically strong and otherwise healthy, all treatment options available to younger patients should be considered, including surgery if the patient has operable breast cancer. The seizures are known to cause central apnea by direct propagation of the electrical discharge to the respiratory center. usually, these are the first symptoms, after a few months from these symptoms, the tumor begins to lead to epilepsy problems. Check for errors and try again. Most meningioma tumors (85-90 percent) are categorized as benign, with the remaining 10-15 percent being atypical meningioma or malignant meningioma (cancerous). On the other hand, if resections are not performed, and the tumour is not completely removed, then the patient is still at risk of experiencing the seizures. This website is intended for pathologists and laboratory personnel but not for patients. The "specific glioneuronal elements" are pathognomonic.
Temporal lobe tumor surgery questions | Epilepsy Foundation [2] Some familial accounts of DNTs have been documented, though the genetic ties have not yet been fully confirmed. Dysembryoplastic neuroepithelial tumors (DNET) typically present in childhood or in young adults (mean age at onset 10 years) and can account for 20% of medically refractory epilepsy.1,2 Diagnostic criteria include partial seizure disorder that begins before age 20, no neurological deficits, and a cortically based tumor. To the best of our knowledge, this is the first case of probable sudden unexplained death in symptomatic epilepsy due to dysembryoplastic neuroepithelial tumor with natural history. When such growths are at the belt line or underneath the bra strap or on the chest where the seat belt in a car rubs across it, they can becomes symptomatic; "they get inflamed, they get irritated . Acta Neuropathol Commun. Many of these tumors are benign (not cancerous). AJNR Am J Neuroradiol. Incidence of Adult-Onset Epilepsy and the Contributory Role of Neurocysticercosis in a Five-Year, Population-Based, Prospective Study in Rural Ecuador. [ 1 3 ] These well-circumscribed glial-neuronal neoplasms commonly arise within the supratentorial cortical gray We were particularly interested in the level of congruence of EEG and MRI data and the need for intracranial recordings. However, we cannot answer medical or research questions or give advice. A fourth subunit is sometimes noted as a mixed subunit. Lhatoo SD, Faulkner HJ, Dembny K, Trippick K, Johnson C, Bird JM: An electroclinical case-control study of sudden unexpected death in epilepsy. [4] This evidence shows that surgery and complete resections are one of the better approaches in treating dysembryoplastic neuroepithelial tumours.
Dysembryoplastic Neuroepithelial Tumor (DNET) | American Journal of Lung Cancer in Older Adults: Treatment and Prognosis - Verywell Health 2003;24 (5): 829-34. EEG showing interictal spikes and polyspikes.
Embryonal tumors - Overview - Mayo Clinic Lee Y, Yang J, Choi SA, Kim SK, Park SH, Park HJ, Kim JI, Phi JH. 2019 Dec;132:347-355. doi: 10.1016/j.wneu.2019.08.221. The presenting symptom is typically treatment-resistant complex . 2 Clinical Features Most patients pres ent with a long-standing history of partial complex seizures that are poorly responsive or resistant to standard antiepileptic therapy. Neurology. I'm from Poland. McWilliams GD, SantaCruz K, Hart B et-al. They consist of a variety of tumor entities that either arise primarily from the ventricular system . The differential diagnosis also depends on the location of the tumor. Typically seen as a cortical lesion with hardly any surrounding vasogenic edema. Article Thom M, Toma A, An S, et al. 2007, 69 (5): 434-441. [4] EEG are predominantly localized with DNT location in the brain, however there are nonspecific cases in which the location of the tumour is abnormal and not localized. There is no reason to believe that our patient's next of kin would object to publication. By using this website, you agree to our Carmen-Adella Srbu. 2020;8(1):21. gliomas, glioneuronal tumors, and neuronal tumors, diffuse astrocytoma, MYB- or MYBL1-altered, polymorphous low-grade neuroepithelial tumor of the young, diffuse low-grade glioma, MAPK pathway-altered, pediatric-type diffuse high-grade gliomas, diffuse hemispheric glioma, H3 G34-mutant, diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype, diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (provisional inclusion), supratentorial ependymoma, ZFTA fusion-positive, supratentorial ependymoma, YAP1 fusion-positive, medulloblastoma, SHH-activated and TP53-wildtype, medulloblastoma, SHH-activated and TP53-mutant, cribriform neuroepithelial tumor (provisional inclusion), CNS tumor with BCOR internal tandem duplication, circumscribed meningeal melanocytic neoplasms, with normal to simplified cortical pattern, microcephaly with extensive polymicrogyria, malformations secondary to inborn errors of metabolism, mitochondrial and pyruvate metabolic disorders, cerebellar hypoplasias, not otherwise specified, focal cerebellar cortical dysplasias/heterotopia, lissencephaly with agenesis of corpus callosum and cerebellar dysplasia, associated with diffuse cerebral polymicrogyria. As performed in this case, gross total resection of the DNET and adjacent cortical dysplasia, if present, is the treatment of choice in DNET. California Privacy Statement, This means they are malignant (cancerous) and fast-growing.
low grade glial dnet tumor temporal lobe - Brain tumors - Inspire Dilated perivascular spaces with adjacent signal changes, View Frank Gaillard's current disclosures, View Yuranga Weerakkody's current disclosures, see full revision history and disclosures, desmoplastic infantile astrocytomas and ganglioglioma, multinodular and vacuolating neuronal tumors (MVNT), oligodendroglioma, IDH-mutant, and 1p/19q-codeleted, high-grade astrocytoma with piloid features, desmoplastic infantile ganglioglioma/astrocytoma, diffuse leptomeningeal glioneuronal tumor, multinodular and vacuolating neuronal tumor, embryonal tumor with multilayered rosettes, pineal parenchymal tumor of intermediate differentiation, desmoplastic myxoid tumor of the pineal region, SMARCB1-mutant, glioma treatment response assessment in clinical trials, World Health Organization (WHO) oncology response criteria, Response Evaluation Criteria in Solid Tumors (RECIST), lissencephaly type I:subcortical band heterotopia spectrum, mild malformations of cortical development, Dysembryoplastic neuroepithelial tumours (DNET)'s, Dysembryoplastic neuroepithelial tumour (DNET), glial nodules and a multinodular architecture. Neuro-Oncology. Journal of Medical Case Reports Other tumors have symptoms that develop slowly. DNETs are most often located in the temporal lobe although all parts of the CNS containing grey matter are potential locations. Review of seizure outcomes after surgical resection of dysembryoplastic neuroepithelial tumors. 1999, 34 (4): 342-356. Renew or update your current subscription to Applied Radiology. Disclaimer. Despite benign behavior, it may have a high MIB-1 labeling index. Histopathology. Benign means that the growth does not spread to other parts of the body. Patira R, Nathan C, Zubkov S, Gutierrez C, Munyon C, Mukherjee A, Jacobson M. Epilepsy Behav Case Rep. 2017 Sep 12;8:92-95. doi: 10.1016/j.ebcr.2017.09.001. Two cases of multinodular and vacuolating neuronal tumour. We evaluated seizure outcomes at last follow-up. 2010, 68 (6): 898-902.
Pathology Outlines - Dysembryoplastic neuroepithelial tumor Additional locations include the occipital and parietal lobes, deep cerebral nuclei (particularly caudate nuclei), cerebellum, and brainstem. Cancers (Basel).
Cancer and Aging | Cancer.Net [2], Three subunits of DNTs have been commonly identified:[2], There currently exists some debate over where to make the proper division for the subunits of DNTs. Short-term outcome is influenced by older age at surgery and longer duration of epilepsy. Zhang ZY, Mo ZQ, Zhang YM, Yang H, Yao B, Ding H. BMC Med Imaging. Other neurological impairments besides seizures are not common. 2022 Dec 22;13(1):24. doi: 10.3390/brainsci13010024. Tomoscintigraphy (single-photon emission CT) with Tc99m MIBI indicated no tumor metabolic activity. Not a CDC funded Page. Part of The long history together with the clinical and imaging data led us to the diagnosis of DNP. . She was treated with carbamazepine, phenytoin, valproic acid and topiramate in diverse doses and combinations without effect on seizures, which continued once or several times a day. [5] Since its prevalence is small among the population, it often goes misdiagnosed or even at times goes undiagnosed. [4] The most common symptom of DNTs are complex partial seizures. DNET tumor Tue, 02/02/2016 - 04:10. DNTs have a benign course, but there are some reports with malignant transformation. PMC Treatment for DNT is surgical resection; however, there is no cohort of untreated control patients. A clinical report and review of the literature. ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers. Today, DNT refers to polymorphic tumors that appear during embryogenesis. Non contrast-enhanced CT scans show well-demarcated lesions that are hypodense relative to the surrounding brain, sometimes with intratumoral calcification and multicystic appearance. Embryonal tumors can occur at any age, but most often occur in babies and young children. Srbu, CA. The spells varied, occurring during the night or day. Al-Hajri A, Al-Mughairi S, Somani A, An S, Liu J, Miserocchi A, McEvoy AW, Yousry T, Hoskote C, Thom M. J Neuropathol Exp Neurol. Dysembryoplastic neuroepithelial tumors (DNET) are benign (WHO Grade 1) slow growing glioneuronal tumors arising from either cortical or deep grey matter. Surgery can resolve the seizures. This news has forced us to take action and he is now going for brain surgery in 3 weeks time. (A) First CT scan show a left temporoparietal diffuse hypodense area, quite inhomogeneous without mass effect. The effectiveness of surgery on seizure outcome has been established. Epub 2016 Feb 27. The presence of secondary generalized seizures, an extratemporal irritative zone and a structural lesion in extratemporal regions correlate with sudden unexplained death in epilepsy (SUDEP). The seizures started at the age of 11, and were of the complex partial atonic type. Polymorphous Low-Grade Neuroepithelial Tumor of the Young: A Case Report with Genomic Findings. Cerebral MRI performed four years later confirmed the diagnosis of brain tumor.
Low Grade Glioma - Conditions - University of Rochester Cardiac dysrhythmias during the interictal state is another potentially fatal condition because of chronic autonomic dysfunction, effects of antiepileptic medication and a common genetic susceptibility [6, 7]. Intractable occipital lobe epilepsy: clinical characteristics, surgical treatment, and a systematic review of the literature. The cystic (bubbly) appearance can help to differentiate the lesion from other tumors.
Dysembryoplastic Neuroepithelial Tumors | Neupsy Key For the tumor to be completely removed doctors need to perform resections consisting an anterior temporal lobectomy or amygdalo-hippocampectomy. MeSH
Incidence of primary brain tumors - UpToDate Malignant Transformation of a Dysembryoplastic Neuroepithelial Tumor (DNET) Characterized by Genome-Wide Methylation Analysis. Therefore postoperative radiation and chemotherapy are not needed, and in infancy and childhood they may be deleterious, so the recognition of surgically curable clinicopathological entities is mandatory. Proposed modification of LEAT classification, Representative imaging features in adolescent, Surgical resection of epileptogenic tumor, Specific glioneuronal (pathognomonic) component, Partially arranged as columnar structures, Dysembryoplastic neuroepithelial tumor (DNET), Sign up for our What's New in Pathology e-newsletter. Yang PF, Jia YZ, Lin Q, Mei Z, Chen ZQ, Zheng ZY, Zhang HJ, Pei JS, Tian J, Zhong ZH. Young adults and children are most affected. MeSH Focal epilepsy associated with dysembryoplastic neuroepithelial tumor in the area of the caudate nucleus.
Dysembryoplastic neuroepithelial tumor | Radiology - Radiopaedia ADHD in Adults with Epilepsy | Epilepsy Foundation Daumas-Duport C, Varlet P: Tumeurs neuroepitheliales dysembryoplasiques. [3], A dysembryoplastic neuroepithelial tumour is commonly diagnosed in patients who are experiencing seizures with magnetic resonance imaging (MRI), electroencephalogram (EEG). Children with brain tumors often have a better prognosis than adults with a similar condition, and most children and adolescents who are diagnosed with a brain tumor will survive. Seizure control after surgery is good with 80-90% seizure free. DNETs are typically predominantly cortical and well-circumscribed tumors. Copyright 2019 Elsevier Inc. All rights reserved. Patients with refractory epilepsy should have complete sleep disorder and cardiology assessments including electrocardiogram evaluation of cardiac rhythm disturbances, which could be performed at the same time as the EEG. Only one case of malignant transformation has been reported 5. dysembryoplastic neuroepithelial tumors (DNET) contrast enhancement uncommon "bubbly appearance" common oligodendroglioma calcifications common older age group: middle-aged adults, most commonly in the 4 th and 5 th decades of life desmoplastic infantile ganglioglioma young children dural involvement prominent large often multiple lesions Epilepsia. 2000, 19 (2): 57-62. 2010 Jan;5(1):123-30. doi: 10.3171/2009.8.PEDS09368.
dnet tumor in older adults government site. PubMed Zhang H, Hu Y, Aihemaitiniyazi A, Li T, Zhou J, Guan Y, Qi X, Zhang X, Wang M, Liu C, Luan G. Brain Sci. The site is secure. [1] The mean age of onset of seizures for children with DNTs is 8.1 years old.
Elimination of seizures after surgery reduces mortality rates in individuals with epilepsy to a level indistinguishable from that of the general population [15]. Bale T. FGFR- Gene Family Alterations in Low-Grade Neuroepithelial Tumors. Defined as "a usually supratentorial glial-neuronal neoplasm occurring in children and young adults and characterized by a predominantly cortical location and by drug . sharing sensitive information, make sure youre on a federal Accurate numbers are not readily available for all types of tumors, often because they are rare or are hard to classify. [citation needed]. Types of embryonal tumors include: Medulloblastomas. Cite this article. Epub 2012 Jul 17.
Neuronal and Mixed Neuronal-Glial Tumors | Cedars-Sinai Problems with retaining saliva 2005;64 (5): 419-27. Our patient was found by her mother in a prone position at the time of death. The tumor will have slow to no growth over years and can remodel the adjacent calvarium. 2015 Jan;157(1):63-75. doi: 10.1007/s00701-014-2217-3. ", "A rare case of dysembryoplastic neuroepithelial tumor", https://en.wikipedia.org/w/index.php?title=Dysembryoplastic_neuroepithelial_tumour&oldid=1103971359. Dysembryoplastic neuroepithelial tumor (DNET) is a benign glioneuronal tumor frequently associated with intractable localization-related seizures in children and young adults. Unable to process the form. Prolonged postictal generalized electroencephalographic suppression, greater than 50 seconds, appears to identify refractory epilepsy patients who are at risk of SUDEP [11]. Prepared by Dr. Moore while practicing at Barrow Neurological Insitute, Phoenix, AZ; and Dr. Cornejo, Dr. Jorgensen, and Dr. Towbin while practicing at Phoenix Childrens Hospital, Phoenix, AZ. 10.1212/WNL.0b013e3181a55f90. DNETs are a mixed glioneuronal neoplasm with a multinodular architecture and a heterogeneous cellular composition. eCollection 2017. Search 16 social services programs to assist you. Some of the common ways cancer treatments can affect older adults are explained below. MR spectroscopy allows the determination of certain biochemical properties of the brain in vivo and reflects the biologic characteristics of benign tumor.
Childhood Dysembryplastic Neuroepithelial Tumor (DNET) [5] Most of the tumours observed in patients are benign tumours, and once taken out do not cause neurological deficits. Results: Three histological forms are recognized 5: Focal cortical dysplasiais commonly seen in association with DNETs, and unless a component can be identified clearly separate from tumor cells, then it does not warrant a concurrent separate diagnosis. DNET was first proposed as a specific entity by Daumas-Duport et al. 2016 Apr;75(4):358-65. doi: 10.1093/jnen/nlw007.
Dnet Tumor Symptoms, Causes, Diagnosis, Treatment - CancerWORLD Thus, all efforts should be undertaken to eliminate this seizure including abstract epilepsy surgery. Takahashi A, Hong SC, Seo DW et-al. 1,2 Diagnostic criteria include partial seizure disorder that begins before age 20, no neurological deficits, and a cortically based tumor. Surg Neurol. Chang EF, Christie C, Sullivan JE, Garcia PA, Tihan T, Gupta N, Berger MS, Barbaro NM. They characteristically cause intractable focal seizures (see temporal lobe epilepsy). [2] Simple DNTs more frequently manifest generalized seizures. Of 1162 articles, 200 relevant studies have been selected. The mean age was 33.3 years (range: 5-56 years). 2023 Feb 9;15(4):1120. doi: 10.3390/cancers15041120.
Dysembryoplastic Neuroepithelial Tumour (DNET) - Brain Tumour It has been found that if the tumour is removed by performing resections patients are then recognized as seizure free. J Neurooncol. This page was last edited on 11 August 2022, at 21:14. The relationship of DNT to the epileptogenic foci can be determined by extensive interictal and ictal EEG recordings. Bethesda, MD 20894, Web Policies
DNET presenting with bleed: An infrequent event - ScienceDirect Dysembryoplastic neuroepithelial tumor s (DNTs) commonly abbreviated DNT or DNET are usually benign tumor s of neuroepithelial origin arising from the cortical gray matter . 2. Unauthorized use of these marks is strictly prohibited. Importantly, DNETs are negative for IDH mutations, TP53 mutations, and do not demonstrate 1p19q co-deletion 8. Clin Neuropathol. Retrospective cohort of 23 patients seen at two major epilepsy centers, with localization-related epilepsy associated with histopathologically demonstrated DNETs. Dysembryoplastic neuroepithelial tumor and probable sudden unexplained death in epilepsy: a case report. CDC funded page. 2015. Five patients required intracranial EEG. FOIA 9. [citation needed], The most common course of treatment of DNT is surgery. Google Scholar. Am J Med Genet Part A 171A:195201. Neuronal cells in the lesion may also secrete neurotransmitters or express receptors. 4th Edition Revised". Patients with DNETs typically present with longstanding treatment-resistant focal seizures (in 90% of cases the first seizure occurred before the age of 20 8) without associated or progressive neurological deficit 5. Single-photon emission CT has been used in limited fashion with DNTs, and this shows hypoperfusion or poor isotope uptake. African Americans. The most common symptoms are: Changes in the person's mental function; Headaches; Seizures (especially in older adults) [1] This classification by WHO only covers the simple and complex subunits. The typical radiological pattern is a magnetic resonance imaging (MRI) T1-hypointense, T2-, and fluid-attenuated inversion-recovery hyperintense multicystic lesion involving the cerebral cortex with no edema. A brain tumor occurs when there is a genetic alteration in the normal cells in the brain. Dysembryoplastic neuroepithelial tumors (DNET) are proliferative mass of tissue arising from glial cells in the central nervous system commonly presenting in childhood years, though, there have been reports of DNET in adults as well [1], [2], [3].They were first described by Daumas-Duport in 1988 as "a surgically curable tumor of young patients with intractable partial . The radiologist found out by cortical topography and found out with the help of no mass effect and sometimes perilesional edema. [4] A DNT is most commonly diagnosed in children who are experiencing seizures, and when given medication do not respond to them.
Dysembryoplastischer neuroepithelialer Tumor - Wikipedia [4] In this case, a second operation has to be done in order to completely remove the malignant tumour. Springer Nature. 2014;2 (1): 7. Sci Rep. 2023 Jan 13;13(1):682. doi: 10.1038/s41598-022-26636-7.
The 2021 WHO Classification of Tumors of the - Wiley Online Library Dysembryoplastic Neuroepithelial Tumor (DNET) - Boston Children's Hospital Medications can be given through the bloodstream to reach cancer cells throughout the body. Long-Term Seizure Outcomes and Predictors in Patients with Dysembryoplastic Neuroepithelial Tumors Associated with Epilepsy. In 60% of cases, the event was related to sleep, which might indicate involvement of a sleep-related event. Although benign, it can develop with local recurrence, even after complete resection. These types of treatments affect your whole body. Noninvasive recording and careful mapping show that a structural lesion is not the source of epileptic activity. These tumours, with a glial and/or neuronal component, are challenging in terms of diagnosis and therapeutic management. Human and animal data suggest that specific genetic factors might play a role in some cases.
Rare Neuronal, Glial and Glioneuronal Tumours in Adults Non-enhancing lesions on MRI were located in the temporal lobe in 17 patients, the frontal lobe in 3 patients and the parietal/occipital region in 2 patients. Careers. . Thom M, Gomez-Anson B, Revesz T, Harkness W, O'Brien CJ, Kett-White R, Jones EW, Stevens J, Scaravilli F: Spontaneous intralesional haemorrhage in dysembryoplastic neuroepithelial tumours: a series of five cases. J Clin Pharmacol. Most commonly found in the temporal lobe, DNTs have been classified as benign tumours. Statdx Web Site. Epub 2014 Oct 3. Below are the links to the authors original submitted files for images. Most commonly found in the temporal lobe, DNTs have been classified as benign tumours.
Dysembryoplastic Neuroepithelial Tumors: What You Need to Know eCollection 2022. Halfpenny A, Ferris SP, Grafe M, Woltjer R, Selden N, Nazemi K, Perry A, Solomon DA, Gultekin SH, Moore S, Olson S, Lawce H, Lucas L, Corless CL, Wood MD. Approximately one-third of tumors are malignant and the remainder are benign or borderline malignant [ 1,2 ]. Older Adults. Together, your brain and spinal cord make up your central nervous system (CNS). In the revised World Health Organization classification, DNTs have been incorporated into the category of neuronal and mixed neuronoglial tumors [3]. One hundred and one dysembryoplastic neuroepithelial tumors: an adult epilepsy series with immunohistochemical, molecular genetic, and clinical correlations and a review of the literature. Manage cookies/Do not sell my data we use in the preference centre.
Adult-onset epilepsy associated with dysembryoplastic neuroepithelial Two treated cases characterized by an atypical presentation have been reviewed. Neuronal and mixed neuronal-glial tumors are a group of rare tumors that occur in the brain or spinal cord. We have been monitoring his tumor for 2 years now and MRI scans tell us that it has been growing - in 2 years it has grown 1 cm all round. Abdelzaher, E. Dysembryoplastic neuroepithelial tumor (DNET). They are cortically based tumours usually arising from grey matter. Lancet. Beijing Da Xue Xue Bao Yi Xue Ban.
Bone cancer - Symptoms and causes - Mayo Clinic - Mayo Clinic - Mayo Clinic Acta Neurochir (Wien). 5. Nervous hunger. PubMed Central No products in the cart. The alteration causes the cells to undergo a series of changes that result in a growing mass of abnormal cells. statement and
Dysembryoplastic neuroepithelial tumor and calcifying - Mayo Clinic Am J Trop Med Hyg. One patient had a DNET that involved both frontal and temporal areas. "WHO Classification of Tumours of the Central Nervous System. 8600 Rockville Pike Nolan MA, Sakuta R, Chuang N, Otsubo H, Rutka JT, Snead OC, Hawkins CE, Weiss SK: Dysembryoplastic neuroepithelial tumors in childhood: long-term outcome and prognostic features. Embryonal tumors of the central nervous system are cancerous (malignant) tumors that start in the fetal (embryonic) cells in the brain. Neuropathology. 2004, 62 (12): 2270-2276. Methods: Thirteen cases of DNET were identified from patient records at the Massachusetts General Hospital Brain Tumor Center. Google Scholar. Childhood brain tumors are less likely to change from low-grade (slow growing, less aggressive) to high-grade (fast growing, more serious).
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