Lhermitte-Duclos Disease and Cowden Syndrome: A Case Report and Literature Review

AbstractLhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma of the cerebellum, is a rare, usually benign, slow-growing tumor, that commonly affects patients aged 30 to 50 years-old. The manifestations of dysplastic cerebellar gangliocytoma are nonspecific and are related both to the mass effect produced by its growth and to the location of the lesion. Cerebellar symptoms such as ataxia are often present. In 40% of cases, the tumor is associated with Cowden syndrome, which is part of a group of genetic disorders called polypoid hamartoma complex. In this case report, the patient presented expansive lesion in the posterior fossa, compatible with LDD, associated with macrocephaly. These findings are considered major criteria for Cowden syndrome. When together, they confirm the diagnoses. To our knowledge, this is the first report of the association of LDD and Cowden syndrome in Brazil.

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A giant solid cystic inferior fourth ventricle subependymoma – Case report and literature review

Romanian Neurosurgery ◽

10.1515/romneu-2016-0073 ◽

2016 ◽

Vol 30 (4) ◽

pp. 455-460

Author(s):

A. Giovani ◽

Narcisa Bucur ◽

Ana Gheorghiu ◽

Lena Papadopol ◽

R.M. Gorgan

Keyword(s):

Case Report ◽

Literature Review ◽

Posterior Fossa ◽

Fourth Ventricle ◽

Precursor Cells ◽

Benign Tumors ◽

Total Resection ◽

Who Grade ◽

Slow Growing ◽

Glial Precursor

Abstract Subependymomas are a rare subtype of ependymomas, slow growing WHO grade I tumors that develop either intracranial from the subependymal glial precursor cells layer of the ventricles or intramedullary. These tumors originate in the undifferentiated Subependymal layer of cells that can become either ependymocytes or astrocytes. Most of the subependymomas are located inside the fourth ventricle (50-60%). We reviewed the case of a 40 years old woman with a giant solid cystic fourth ventricle ependymoma. The patient underwent total resection of the tumor through a subociipital transvermian approach. We discussed the characteristics of these benign tumors and reviewed the literature on this subject and concluded that total resection is the treatment of choice for symptomatic Subependymomas localized in posterior fossa.

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Blake's Pouch Cyst: A Case Report

Indian Journal of Neurosurgery ◽

10.1055/s-0037-1601357 ◽

2017 ◽

Vol 06 (02) ◽

pp. 122-125

Author(s):

Saurabh Verma ◽

M. Sridhar ◽

S. Shashivadhanan ◽

Manish Garg

Keyword(s):

Genetic Counseling ◽

Case Report ◽

Literature Review ◽

Posterior Fossa ◽

Clinical Management ◽

In Utero ◽

Prenatal Ultrasound ◽

Male Child ◽

Imaging Studies ◽

Initial Imaging

AbstractIt is a rare and underdiagnosed entity. The adagium “one only sees what one knows” is certainly true in cases of Blake's pouch cyst, as all types of posterior fossa cysts and cyst-like malformations may present nearly identical on initial imaging studies. Only one case of Blake's pouch cyst has been reported from this country, except for a case in utero, in which a diagnosis of Blake's pouch cyst was made on prenatal ultrasound and later confirmed by MRI. In this report we describe a case of Blake's pouch cyst in a 9-month-old male child along with the principles of diagnosis of Blake's pouch cyst, in combination with literature review. Differentiating Blake's pouch cyst from other posterior fossa cysts and cyst-like malformations and recognizing the accompanying hydrocephalus that are essentially noncommunicating have important implications not only on clinical management but also on genetic counseling, which is unnecessary in case of Blake's pouch cyst.

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Bilateral Recurrent Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) in Cowden Syndrome: A Case Report and Literature Review

World Neurosurgery ◽

10.1016/j.wneu.2019.03.131 ◽

2019 ◽

Vol 127 ◽

pp. 319-325 ◽

Cited By ~ 1

Author(s):

Umang Khandpur ◽

Kristin Huntoon ◽

Matthew Smith-Cohn ◽

Andrew Shaw ◽

James Bradley Elder

Keyword(s):

Case Report ◽

Literature Review ◽

Cowden Syndrome

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Posterior fossa ependymoma with preoperative cerebrospinal metastases: a case report with literature review

British Journal of Neurosurgery ◽

10.1080/02688697.2020.1853044 ◽

2020 ◽

pp. 1-4

Author(s):

Hanlu Tang ◽

Tao Wu ◽

Fu Zhao ◽

Jing Zhang ◽

Zhijun Yang ◽

...

Keyword(s):

Case Report ◽

Literature Review ◽

Posterior Fossa

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Colonic ganglioneuromatous polyposis and metastatic adenocarcinoma in the setting of Cowden syndrome: a case report and literature review

Human Pathology ◽

10.1016/j.humpath.2011.06.022 ◽

2012 ◽

Vol 43 (4) ◽

pp. 601-604 ◽

Cited By ~ 26

Author(s):

Joshua W. Trufant ◽

Laura Greene ◽

Deborah L. Cook ◽

Wendy McKinnon ◽

Marc Greenblatt ◽

...

Keyword(s):

Case Report ◽

Literature Review ◽

Cowden Syndrome ◽

Metastatic Adenocarcinoma

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Twelfth nerve paresis induced by an unusual posterior fossa arachnoid cyst: Case report and literature review

British Journal of Neurosurgery ◽

10.3109/02688697.2013.841851 ◽

2013 ◽

Vol 28 (4) ◽

pp. 528-530 ◽

Cited By ~ 4

Author(s):

Roberto Tarantino ◽

Daniele Marruzzo ◽

Davide Colistra ◽

Cristina Mancarella ◽

Roberto Delfini

Keyword(s):

Case Report ◽

Literature Review ◽

Posterior Fossa ◽

Arachnoid Cyst ◽

Nerve Paresis

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Bilateral Dysplastic Gangliocytoma with Concurrent Polyostotic Fibrous Dysplasia: A Case Report and Literature Review

World Neurosurgery ◽

10.1016/j.wneu.2020.06.071 ◽

2020 ◽

Vol 141 ◽

pp. 421-424

Author(s):

Cheng Jiang ◽

Wei-xin Lu ◽

Gui-zhong Yan ◽

Ruo-bing Bai ◽

Ze-ning Wang ◽

...

Keyword(s):

Case Report ◽

Literature Review ◽

Fibrous Dysplasia ◽

Polyostotic Fibrous Dysplasia ◽

Dysplastic Gangliocytoma

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Presenting symptoms of giant fibrovascular polyp of the oesophagus: case report and literature review

Annals of The Royal College of Surgeons of England ◽

10.1308/rcsann.2016.0127 ◽

2016 ◽

Vol 98 (5) ◽

pp. e71-e73 ◽

Cited By ~ 5

Author(s):

S Sestini ◽

M Gisabella ◽

U Pastorino ◽

A Billé

Keyword(s):

Case Report ◽

Gastrointestinal Tract ◽

Literature Review ◽

Case History ◽

Curative Resection ◽

Gastrointestinal Neoplasms ◽

Presenting Symptoms ◽

Uncommon Presentation ◽

Cervical Approach ◽

Slow Growing

Introduction Lipomas of the gastrointestinal tract are rare, slow-growing lesions that comprise 0.4% of all gastrointestinal neoplasms. They can cause dysphagia, dyspnoea or sudden choking. Case History Due to rarity of this condition and its uncommon presentation, a literature review was carried out (PubMed). This search revealed 290 articles, of which 74 were considered pertinent and were evaluated. We report a case of a 13cm pedunculated oesophageal lipoma that presented with increasing dysphagia and two episodes of suffocation. The patient underwent curative resection through a cervical approach. Conclusions Resection is recommended for large (>5 cm) or symptomatic polyps. Outcomes are excellent given that lesions are universally benign and oesophageal resection is not required.

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