Diffuse Leptomeningeal Glioneuronal Tumor in an Adult: A Diagnostic Challenge

AbstractHere we report a rare case of diffuse leptomeningeal glioneuronal tumor (DLGNT) in a 35-year-old man, who was misdiagnosed twice as having tuberculosis meningitis and later racemose neurocysticercosis. His delayed diagnosis of DLGNT might be due to prevalence of tuberculosis in our country, similarity in magnetic resonance imaging finding of prominent leptomeningeal enhancement in different cisterns of brain, and extreme rarity of DLGNT in the adults. So, it should be differentiated clinically and radiographically from granulomatous or infectious conditions. Hence, a timely histologic diagnosis through a leptomeningeal biopsy of the brain and spinal cord in case of unusual leptomeningeal enhancement with uncertain laboratory findings is essential because cytological examination of the cerebrospinal fluid in DLGNT is known to be negative.

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A Case of Diffuse Leptomeningeal Glioneuronal Tumor Misdiagnosed as Chronic Tuberculous Meningitis without Brain Biopsy

Case Reports in Neurological Medicine ◽

10.1155/2018/1391943 ◽

2018 ◽

Vol 2018 ◽

pp. 1-7 ◽

Cited By ~ 5

Author(s):

Jung koo Lee ◽

Hak-cheol Ko ◽

Jin-gyu Choi ◽

Youn Soo Lee ◽

Byung-chul Son

Keyword(s):

Tuberculous Meningitis ◽

Glioneuronal Tumor ◽

Communicating Hydrocephalus ◽

Laboratory Findings ◽

Cytologic Examination ◽

Mental Confusion ◽

Leptomeningeal Enhancement ◽

Diffuse Leptomeningeal Glioneuronal Tumor ◽

The Brain ◽

Basal Cisterns

Here we report a rare case of diffuse leptomeningeal glioneuronal tumor (DLGNT) in a 62-year-old male patient misdiagnosed as having tuberculous meningitis. Due to its rarity and radiologic findings of leptomeningeal enhancement in the basal cisterns on magnetic resonance imaging (MRI) similar to tuberculous meningitis, DLGNT in this patient was initially diagnosed as communicating hydrocephalus from tuberculous meningitis despite absence of laboratory findings of tuberculosis. The patient’s symptoms and signs promptly improved after a ventriculoperitoneal shunting surgery followed by empirical treatment against tuberculosis. Five years later, mental confusion and ataxic gait developed in this patient again despite well-functioning ventriculoperitoneal shunt. Aggravation of leptomeningeal enhancement in the basal cisterns was noted in MRI. An additional course of antituberculosis medication with steroid was started without biopsy of the brain. Laboratory examinations for tuberculosis were negative again. After four months of improvement, his mental confusion, memory impairment, dysphasia, and ataxia gradually worsened. A repeated MRI of the brain showed further aggravation of leptomeningeal enhancement in the basal cisterns. Biopsy of the brain surface and leptomeninges revealed a very rare occurrence of DLGNT. His delayed diagnosis of DLGNT might be due to prevalence of tuberculosis in our country, similarity in MRI finding of prominent leptomeningeal enhancement in the basal cisterns, and extreme rarity of DLGNT in the elderly. DLGLT should be considered in differential diagnosis of medical conditions presenting as communicating hydrocephalus with prominent leptomeningeal enhancement. A timely histologic diagnosis through a leptomeningeal biopsy of the brain and spinal cord in case of unusual leptomeningeal enhancement with uncertain laboratory findings is essential because cytologic examination of the cerebrospinal fluid in DLGNT is known to be negative.

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Diffuse leptomeningeal glioneuronal tumor with high-grade features masquerading as tubercular meningitis—a case report

Egyptian Journal of Radiology and Nuclear Medicine ◽

10.1186/s43055-021-00522-0 ◽

2021 ◽

Vol 52 (1) ◽

Author(s):

Sameer Peer ◽

Vivek Murumkar ◽

Karthik Kulanthaivelu ◽

Chandrajit Prasad ◽

Shilpa Rao ◽

...

Keyword(s):

Case Report ◽

Early Diagnosis ◽

Histopathological Examination ◽

Glioneuronal Tumor ◽

Communicating Hydrocephalus ◽

High Grade ◽

Endemic Region ◽

Tubercular Meningitis ◽

Leptomeningeal Enhancement ◽

Diffuse Leptomeningeal Glioneuronal Tumor

Abstract Background Diffuse leptomeningeal glioneuronal tumor (DLGNT) has been recently described in the literature. The complete neuroimaging spectrum and histopathological characteristics of this entity are yet to be elucidated. In an endemic region, diffuse leptomeningeal enhancement on neuroimaging with associated communicating hydrocephalus is usually suggestive of infective meningitis and the patients are started on empirical anti-microbial therapy. However, it is important to consider other differential diagnosis of leptomeningeal enhancement in such cases, particularly if the clinical condition does not improve on anti-microbial therapy. An early diagnosis of a neoplastic etiology may be of particular importance as the treatment regimens vary considerably depending on the underlying disease condition. Case presentation In this case report, we describe a case of DLGNT with high-grade histopathological features which was initially managed as tubercular meningitis based on the initial neuroimaging findings. Due to worsening of the clinical course and subsequent imaging findings at follow-up, a diagnosis of DLGNT was considered and subsequently proven to be DLGNT with features of anaplasia on histopathological examination of leptomeningeal biopsy specimen. Conclusion This case highlights the importance of recognizing certain subtle finding on MRI which may help in an early diagnosis of DLGNT which is crucial for appropriate treatment.

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Epidemiology, Clinical and Laboratory Manifestations, and Outcomes of Brucellosis Among 104 Patients in Referral Hospitals of Tehran, Iran

Archives of Clinical Infectious Diseases ◽

10.5812/archcid.111546 ◽

2021 ◽

Vol 16 (2) ◽

Author(s):

Amirreza Keyvanfar ◽

Shahnaz Sali ◽

Amir Zamani

Keyword(s):

Treatment Failure ◽

Financial Burden ◽

Delayed Diagnosis ◽

Clinical Manifestations ◽

Diagnostic Challenge ◽

Laboratory Findings ◽

Elevated Alkaline Phosphatase ◽

Cross Sectional ◽

Dairy Consumption ◽

Phone Calls

Background: Brucellosis is the most common zoonotic disease in Iran, imposing a significant financial burden on the healthcare system. The diversity of non-specific clinical manifestations of this disease can lead to misdiagnosis or delayed diagnosis. Therefore, it is important to pay attention to other aspects of this disease. Objectives: This study aimed to determine the epidemiology, clinical and laboratory manifestations, and outcomes of brucellosis. Methods: In this cross-sectional study, brucellosis patients, admitted to three hospitals, affiliated to Shahid Beheshti University of Medical Sciences (Tehran, Iran) from April 2015 to September 2020, were examined. The patients' medical records were reviewed for epidemiological, clinical, and laboratory findings. The outcomes of the disease were evaluated by phone calls. Results: Of the 104 patients included in this study, 53.8% were male, and 46.2% were female. The mean age of the patients was 43.07 ± 18.521 years. Unpasteurized dairy consumption and contact with livestock were reported in 60.6 and 27.9% of the patients, respectively. Also, 23.1% of the patients had high-risk occupations. The most common symptoms included fever (80.8%), chills (58.7%), backache (55.8%), and sweating (51%). The most common complication was osteoarticular involvement (21.2%), followed by neurobrucellosis (6.7%). Elevated alkaline phosphatase (89.7%), anemia (67.3%), increased C-reactive protein (57.7%), and increased erythrocyte sedimentation rate (48.1%) were common laboratory findings. The rates of improvement, treatment failure, and relapse were 86.9, 9.1, and 4%, respectively. There was no significant relationship between the rate of improvement and the antibiotic regimen. However, the most common cause of treatment failure was the patient’s poor compliance with treatment. Conclusions: The diversity of non-specific clinical manifestations of brucellosis is a diagnostic challenge. Therefore, physicians must request laboratory tests to evaluate brucellosis after taking a precise epidemiological and clinical history of suspected cases.

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Refractory Ascites as the Only Presenting Symptom of Chronic Calcified Constrictive Pericarditis: A Diagnostic Challenge

The Heart Surgery Forum ◽

10.1532/hsf98.2013251 ◽

2014 ◽

Vol 17 (1) ◽

pp. 42

Author(s):

Shi-Min Yuan

Keyword(s):

Computed Tomography ◽

Liver Cirrhosis ◽

Female Patient ◽

Constrictive Pericarditis ◽

Delayed Diagnosis ◽

Young Female ◽

Refractory Ascites ◽

Diagnostic Challenge ◽

Massive Ascites ◽

History Of

Extracardiac manifestations of constrictive pericarditis, such as massive ascites and liver cirrhosis, often cover the true situation and lead to a delayed diagnosis. A young female patient was referred to this hospital due to a 4-year history of refractory ascites as the only presenting symptom. A diagnosis of chronic calcified constrictive pericarditis was eventually established based on echocardiography, ultrasonography, and computed tomography. Cardiac catheterization was not performed. Pericardiectomy led to relief of her ascites. Refractory ascites warrants thorough investigation for constrictive pericarditis.

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Emergency Department and Radiological Cost of Delayed Diagnosis of Cannabinoid Hyperemesis

Journal of Addiction ◽

10.1155/2019/1307345 ◽

2019 ◽

Vol 2019 ◽

pp. 1-4 ◽

Cited By ~ 8

Author(s):

David I. Zimmer ◽

Ross McCauley ◽

Varun Konanki ◽

Joseph Dynako ◽

Nuha Zackariya ◽

...

Keyword(s):

Emergency Department ◽

Delayed Diagnosis ◽

The United States ◽

Clinical Syndrome ◽

Cannabis Use ◽

Diagnostic Challenge ◽

High Clinical Suspicion ◽

Ed Visits ◽

Inappropriate Utilization ◽

Utilization Of Healthcare

Background. Chronic cannabis use has become prevalent with decriminalization, medical prescription, and recreational legalization in numerous US states. With this increasing incidence of chronic cannabis use a new clinical syndrome has become apparent in emergency departments and hospitals across the country, termed Cannabinoid Hyperemesis (CH). CH has been described as cyclical vomiting and abdominal pain in the setting of chronic cannabis use, which is often temporarily relieved by hot showers. CH presents a diagnostic challenge to clinicians who do not have a high clinical suspicion for the syndrome and can result in high costs and resource utilization for hospitals and patients. This study investigates the expenditures associated with delayed CH evaluation and delayed diagnosis. Methods. This is a retrospective observational study of 17 patients diagnosed with CH at three medical centers in the United States from 2010 to 2015, consisting of two academic centers and a community hospital. Emergency department (ED) costs were calculated and analyzed for patients eventually diagnosed with CH. Results. For the 17 patients treated, the total cost for combined ED visits and radiologic evaluations was an average of $76,920.92 per patient. On average these patients had 17.9 ED visits before the diagnosis of CH was made. Conclusion. CH provides a diagnostic challenge to clinicians without a high suspicion of the syndrome and may become increasingly prevalent with current trends toward cannabis legalization. The diagnosis of CH can be made primarily through a thorough history and physical examination. Awareness of this syndrome can save institutions money, prevent inappropriate utilization of healthcare resources, and save patients from unnecessary diagnostic tests.

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Pancreaticopleural Fistula: A Cause of Recurrent Pleural Effusions in Chronic Pancreatitis

Journal of Gastrointestinal and Abdominal Radiology ◽

10.1055/s-0040-1719222 ◽

2021 ◽

Author(s):

Ayah Megahed ◽

Rahul Hegde ◽

Pranav Sharma ◽

Rahmat Ali ◽

Anas Bamashmos

Keyword(s):

Chronic Pancreatitis ◽

Pancreatic Duct ◽

Pleural Fluid ◽

Rare Complication ◽

Delayed Diagnosis ◽

Magnetic Resonance Cholangiopancreatography ◽

Pleural Effusions ◽

Diagnostic Challenge ◽

Abdominal Symptoms ◽

Pancreatic Duct Stenting

AbstractPancreaticopleural fistula is a rare complication of chronic pancreatitis caused by disruption of the pancreatic duct and fistulous communication with the pleural cavity. It usually presents with respiratory symptoms from recurrent large volume pleural effusions. Paucity of abdominal symptoms makes it a diagnostic challenge, leading often to delayed diagnosis. Marked elevation of pleural fluid amylase, which is not a commonly performed test, is a sensitive marker in its detection. Imaging with magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography can help delineate the fistula. In this report, we present the clinical features, imaging, and management of a 59-year-old male patient with pancreaticopleural fistula, wherein the diagnosis was suspected only after repeated pleural fluid drainages were performed for re-accumulating pleural effusions and it was eventually successfully treated with pancreatic duct stenting. We review the literature with regards to the incidence, presentation, diagnosis, and management of this rare entity.

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Sequential Magnetic Resonance Imaging Finding of Intramedullary Spinal Cord Abscess including Diffusion Weighted Image: a Case Report

Korean Journal of Radiology ◽

10.3348/kjr.2011.12.2.241 ◽

2011 ◽

Vol 12 (2) ◽

pp. 241 ◽

Cited By ~ 5

Author(s):

Jae Eun Roh ◽

Seung Young Lee ◽

Sang-Hoon Cha ◽

Bum Sang Cho ◽

Min Hee Jeon ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Spinal Cord ◽

Case Report ◽

Magnetic Resonance ◽

Imaging Finding ◽

Magnetic Resonance Imaging Finding ◽

Diffusion Weighted Image ◽

Resonance Imaging ◽

Intramedullary Spinal Cord ◽

Intramedullary Spinal Cord Abscess

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The ability of clinical and laboratory findings to predict in-hospital death in patients with thrombotic thrombocytopenic purpura in an internal and emergency medicine department

Italian Journal of Medicine ◽

10.4081/itjm.2011.269 ◽

2012 ◽

pp. 269-273

Author(s):

Filippo Pieralli ◽

Antonio Mancini ◽

Alberto Camaiti ◽

Giancarlo Berni ◽

Carlo Nozzoli

Keyword(s):

Emergency Medicine ◽

Thrombotic Thrombocytopenic Purpura ◽

Symptom Onset ◽

Delayed Diagnosis ◽

Disease Process ◽

Hemodynamic Instability ◽

Hospital Death ◽

Laboratory Findings ◽

Thrombocytopenic Purpura ◽

Lactate Levels

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening syndrome characterized by microangiopathic anemia, thrombocytopenia, diffuse microvascular thrombosis, and ischemia. It is associated with very low levels of ADAMTS-13. Measurement of ADAMTS-13 levels is used for diagnostic and prognostic purposes, but in every-day clinical practice, this type of analysis is not always readily available. In this retrospective study, we evaluated prognostic value of clinical and laboratory findings in patients with TTP. Materials and methods: We retrospectively investigated patients with clinically diagnosed TTP treated in a unit of Internal and Emergency Medicine (1996-2007). Clinical and laboratory findings were collected and analyzed in order to assess their ability to predict in-hospital death. Results: Twelve patients were identified (mean age 59 + 22 years; 58% were women). Five (42%) died during the hospitalization, and the variables significantly associated with this outcome were: a delay between diagnosis and symptom onset (HR 1.36; 95% CI 1.04-1.78; p < 0.05); a higher severity score (HR 1.48; 95%CI 1,23-3.86; p < 0.05); hemodynamic instability with hypotension and/or shock (HR 3.35; 95%CI 3.02-9.26; p < 0.01); a higher schistocyte count on blood smear (HR 1.84; 95%CI 1.04-3.27; p < 0.05); and higher lactate values (HR 1.85; 95%CI 1.08- 3.16; p < 0.05). Conclusions: TTP is a rare and potentially fatal disease with protean manifestations. Delayed diagnosis after symptom onset is a major determinant of poor outcome. Hypotension and shock are also prognostically unfavourable. Laboratory evidence of cardiocirculatory compromise (i.e., elevated lactate levels) and extension of the disease process (i.e., schistocyte count > 3) are predictive of in-hospital death, independently of the hemodynamic profile on admission.

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LGG-26. DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR (DLGNT) IN CHILDREN: DIFFERENT CLINICAL PRESENTATIONS AND OUTCOMES

Neuro-Oncology ◽

10.1093/neuonc/noaa222.408 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii371-iii371

Author(s):

Andge Valiakhmetova ◽

Ludmila Papusha ◽

Ludmila Yasko ◽

Alexander Druy ◽

Alexander Karachunsky ◽

...

Keyword(s):

Mapk Pathway ◽

Braf Inhibitor ◽

Complete Response ◽

Mek Inhibitor ◽

Braf V600e ◽

Glioneuronal Tumor ◽

Low Grade ◽

Conventional Chemotherapy ◽

Diffuse Leptomeningeal Glioneuronal Tumor ◽

Braf Fusion

Abstract Diffuse leptomeningeal glioneuronal tumor (DLGNT) is an extremely rare disease, newly recognized in the 2016 WHO classification of tumors of the CNS. Most DLGNTs are low-grade neuroepithelial tumors with variable elements of neuronal/neurocytic and glial differentiation, have diffuse leptomeningeal enhancement on MRI, and typically harbor KIAA1549-BRAF fusions. Other alterations, such as the BRAF V600E substitution, are less common. Here, we present three cases of DLGNT with different presentations and outcomes. The first patient is a 2yr-old male with KIAA1549-BRAF fusion, and was treated with Carbo/VCR chemotherapy after a biopsy, with resultant ongoing stable disease for 3.5 years. The second patient, an 8yr-old male had the BRAF V600E point mutation and was treated with conventional chemotherapy (VCR/carboplatin). On progression, he received the BRAF inhibitor vemurafenib, achieving a complete response which last 14 month. The third patient, a 27 month old male, harbored a KIAA1549-BRAF fusion and was treated at diagnosis with the MEK inhibitor trametinib. The tumor has been radiographically stable in the context of clinical improvement for 21 months since the treatment initiation, ongoing 24 month. In summary, we present further evidence of MAPK pathway alterations in children with DLGNT. We describe a range of molecular presentations and clinical outcomes, including one patient treated with conventional chemotherapy with further stabilization of disease during 3.5 years and two patients who were successfully treated with targeted therapy.

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