scholarly journals Metastatic extramammary paget disease, a remarkable clinical case and a brief review of a rare disease

2020 ◽  
Vol 12 (2) ◽  
Author(s):  
Leonor Fernandes ◽  
Joana Graça ◽  
Leonor Vasconcelos de Matos ◽  
Rita Sampaio ◽  
Mafalda Miranda Baleiras ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Rare Disease ◽  
Poor Prognosis ◽  
Clinical Case ◽  
Standard Of Care ◽  
Primary Treatment ◽  
Metastatic Site ◽  
Paget Disease ◽  
The Central Nervous System ◽  
Current Guidelines

Extramammary Paget Disease (EMPD) is an often-misdiagnosed rare disorder, whose cause remains unknown. Diagnosis is confirmed by skin biopsy. Primary treatment for EMPD is surgery. Recurrence is common in the first two years and prognosis is good if the disease is localized and there is no underlying associated cancer. Patients with invasive and metastatic EMPD are uncommon and exhibit a poor prognosis, even when there is good response to a first chemotherapy line. Multiple chemotherapeutic regimens, with varying levels of success, have been attempted, but standard of care is not established. The central nervous system seems to be a common metastatic site with better survival than visceral metastasis.We report a case of metastatic EMPD that addresses the difficulties associated with the treatment of this rare disease, that has no current guidelines.

scholarly journals Acute Disseminated Encephalomyelitis (ADEM): A Demyelinating Disease with Specific Morphological Features

2021 ◽  
pp. 106689692199356
Author(s):  
Fleur Cordier ◽  
Lars Velthof ◽  
David Creytens ◽  
Jo Van Dorpe
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Differential Diagnosis ◽  
Clinical Case ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
The Central Nervous System

Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated inflammatory and demyelinating disorder of the central nervous system. Its characteristic perivenular demyelination and inflammation aid in the differential diagnosis with other inflammatory demyelinating diseases. Here, we present a clinical case of ADEM, summarize its histological hallmarks, and discuss pitfalls concerning the most important neuropathological differential diagnoses.

Dural Melanoma Associated with Ocular Melanosis and Multiple Blue Nevi

2001 ◽  
Vol 5 (5) ◽  
pp. 381-385 ◽  
Author(s):  
Jason K. Rivers ◽  
Shelly Bhayana ◽  
Magda Martinka
Keyword(s):  
Central Nervous System ◽  
Poor Prognosis ◽  
Surgical Intervention ◽  
Asian Woman ◽  
Total Resection ◽  
Aggressive Disease ◽  
Nevus Of Ota ◽  
The Central Nervous System ◽  
Aggressive Tumors ◽  
Surgical Extirpation

Background: Primary meningeal melanomas of the central nervous system (CNS) are a rare malignant process with the majority originating from the leptomeninges. Primary dural melanomas have been reported to occur in isolation or in conjunction with Nevus of Ota. The association of primary dural melanoma with multiple cutaneous blue nevi has not been reported previously. Objective: To describe a case of a 41-year-old Asian woman patient with a primary dural melanoma that arose in association with ocular melanosis and multiple cutaneous blue nevi. The patient is alive almost more than 8 years after subtotal and subsequent total resection of her primary tumor. Primary dural melanomas, Nevus of Ota, and blue nevi are discussed in relation to their coexistence and potential for intracranial melanoma. Conclusion: CNS melanoma is regarded as an extremely aggressive disease with poor prognosis. This case and previous reports of dural melanomas occurring in isolation or with Nevus of Ota have demonstrated relatively prolonged survival after surgical intervention. We conclude that dural melanomas are less aggressive tumors requiring surgical extirpation only.

scholarly journals Aspergillus infection causing cerebral aneurysm

2018 ◽  
Vol 21 (3) ◽  
pp. 182-186
Author(s):  
Moisés Heleno Vieira Braga1 ◽  
Juliana Naves Ravanini ◽  
Sergio Rosemberg ◽  
Maurício Brígido Mandel ◽  
Bernardo Assunção De Mônaco
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Rare Disease ◽  
Cerebral Aneurysm ◽  
Cerebral Aneurysms ◽  
Growing Up ◽  
Review Of Literature ◽  
Aspergillus Infection ◽  
The Central Nervous System

Background: Aspergillosis infection of the central nervous system (CNS) is a rare disease and corticosteroid use has increased the number of cases. It presents a high lethality rate and has to be aggressively treated. Methods: A review of literature was done and 38 cases were identified involving cerebral aneurysms caused by this fungus to date. Conclusion: Aspergillus infection is very rare worldwide but its frequency is currently in expansion because immunocompetence failure is growing up: it has no specific presentation, making diagnosis difficult. Its prognosis is poor, so the disease has to betreated as early as posible.  

scholarly journals Endoscopic Transsphenoidal Approach for Hemangiopericytoma in Sellar Region: A Case Report and Review of the Literature

2021 ◽  
Vol 6 (6) ◽  
Author(s):  
Wang P ◽  
Wang J ◽  
Zou D ◽  
Pan J ◽  
Wu N
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Case Report ◽  
Aggressive Behavior ◽  
Clinical Case ◽  
Transsphenoidal Approach ◽  
Sellar Region ◽  
Review Of The Literature ◽  
The Central Nervous System

Background: Hemangiopericytomas (HPCs) with aggressive behavior are rare in the central nervous system and extremely rare in the sellar region. Here we presented a clinical case of HPC in sellar region.

Torulosis of the Central Nervous System

1943 ◽  
Vol 89 (374) ◽  
pp. 42-51 ◽  
Author(s):  
Donald Blair
Keyword(s):  
United States ◽  
Central Nervous System ◽  
Nervous System ◽  
Rare Disease ◽  
Mental Hospital ◽  
The United States ◽  
Observation Unit ◽  
The Third ◽  
The World ◽  
The Central Nervous System

In March, 1939. there was admitted under my care at the St. Pancras Hospital Mental Observation Unit a case of torulosis of the nervous system. This is a very rare disease in this country and the present case is only the third recorded in British medical history (Greenfieldet al., 1938; Smith and Crawford, 1930), and the first one to have come under mental hospital supervision. Although such a rarity here, torulosis is more common in the United States, and cases have been reported from nearly every part of the world.

Thyroid Storm in a Young Woman: A Clinical Case

2020 ◽  
Vol 5 (10) ◽  
pp. 453-455
Author(s):  
Cristiane Venturini Garlet ◽  
Isadora de Macedo Tomitão ◽  
Kayque Wellek Delgado do Amaral ◽  
Tiffany Ayumi Negrete Bomura ◽  
Ana Carla Barbosa Lima ◽  
...  
Keyword(s):  
Heart Failure ◽  
Central Nervous System ◽  
Young Woman ◽  
Digestive System ◽  
Clinical Case ◽  
Rare Condition ◽  
Thyroid Storm ◽  
Graves Disease ◽  
The Central Nervous System ◽  
Threat To Life

Thyroid storm is a rare condition, but it can be characterized as a threat to life. It presents as an increase in the functions of the thyroid gland due to dysfunction of the central nervous system. It is characterized by pyrexia, tachycardia, heart failure and alterations in the digestive system. The objective of this report is to describe the case of a young woman with no previously reported diseases, who was affected by the thyroid storm, without the presence of Graves' disease.

scholarly journals Meningioma of the Pineal Region — Differential Radiologic Aspects of Pineal Region Tumors Based on a Clinical Case

2018 ◽  
Vol 37 (02) ◽  
pp. 145-147
Author(s):  
Tiago Avelar ◽  
Aline Paiva ◽  
Márcio Costa ◽  
Guilherme Aguiar ◽  
João Vitorino ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Case ◽  
Pineal Region ◽  
Differential Diagnoses ◽  
Benign Lesions ◽  
Pineal Region Tumor ◽  
Pineal Region Tumors ◽  
The Central Nervous System ◽  
Velum Interpositum

AbstractPineal region tumors are uncommon among neoplasm of the central nervous system, with this region being the most heterogeneous in terms of histological types. Meningiomas are rarer still, but can be found at this site, with origins in either the velum interpositum or falcotentorial junction. Neuroimaging exams can distinguish malignant from benign lesions besides helping to define the origin of the lesion as the pineal parenchymal or surrounding structures. We report the case of a woman with a pineal region tumor in which differential diagnoses included meningioma and germinoma, with confirmation of the former based on radiological characteristics and histopathology. In addition, a brief review of differential diagnoses and approaches for cases of lesions in this region is provided.

scholarly journals Erdheim-Chester Disease of the Central Nervous System: New Manifestations of a Rare Disease

2011 ◽  
Vol 32 (11) ◽  
pp. 2126-2131 ◽  
Author(s):  
P. Sedrak ◽  
L. Ketonen ◽  
P. Hou ◽  
N. Guha-Thakurta ◽  
M.D. Williams ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Rare Disease ◽  
Erdheim Chester Disease ◽  
The Central Nervous System ◽  
Erdheim Chester ◽  
Chester Disease

scholarly journals Association between a Primitive Brain Tumor and Cerebral Aspergillosis

2012 ◽  
Vol 2012 ◽  
pp. 1-5
Author(s):  
Siegfried Hélage ◽  
Charles Duyckaerts ◽  
Danielle Seilhean ◽  
Jean-Jacques Hauw ◽  
Jacques Chiras
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Brain Tumor ◽  
Invasive Aspergillosis ◽  
Therapeutic Approach ◽  
Poor Prognosis ◽  
The Central Nervous System ◽  
Immunocompetent Patients

Cerebral aspergillosis is a rare pathology of poor prognosis in spite of the use of adapted antifungal treatments. This infection of the central nervous system is generally the complication of an invasive aspergillosis with hematogenic scattering from pulmonary focal spots. It can arise in immunocompetent patients treated with prolonged corticotherapy or chemoradiotherapy for cancer. A case of lethal cerebral aspergillosis in a patient with an infiltrative glioma treated with corticotherapy and radiotherapy is reported. Clinicopathological aspects and therapeutic approach are described.

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