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

2016 ◽  
Vol 30 (4) ◽  
pp. 455-460
Author(s):  
A. Giovani ◽  
Narcisa Bucur ◽  
Ana Gheorghiu ◽  
Lena Papadopol ◽  
R.M. Gorgan

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.

2020 ◽  
Vol 82 (01) ◽  
pp. 090-094
Author(s):  
Alexey Tokarev ◽  
Viacheslav Rak ◽  
Valentin Stepanov ◽  
Sergey Zuev ◽  
Olga Evdokimova ◽  
...  

AbstractIntracranial schwannomas (ICS) unrelated to the cranial nerves are extremely rare; around 70 cases have been reported worldwide. The available literature consists of case reports and small series that present variable characteristics distinguishing these lesions. Brain parenchyma schwannomas are typically benign tumors with currently unknown origins. Diagnosis of intraparenchymal schwannoma is almost never made preoperatively. The management of these tumors usually consists of gross total resection, chemotherapy, and radiotherapy in cases of recurrence. The authors present a case of fractionated Gamma Knife radiosurgical treatment of intracerebral schwannoma following partial microsurgical resection.


2019 ◽  
Vol 38 (04) ◽  
pp. 319-323
Author(s):  
Mylena Miki Lopes Ideta ◽  
Mylla Christie Oliveira Paschoalino ◽  
Louise Makarem Oliveira ◽  
Nelson Brancaccio dos Santos ◽  
Marco Rodrigo Valdivia Sanz ◽  
...  

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.


2017 ◽  
Vol 06 (02) ◽  
pp. 122-125
Author(s):  
Saurabh Verma ◽  
M. Sridhar ◽  
S. Shashivadhanan ◽  
Manish Garg

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.


2019 ◽  
Vol 7 (2) ◽  
pp. 141
Author(s):  
Moowan Park ◽  
Eun Kyeong Hong ◽  
Sang Hyen Lee ◽  
Ho-Shin Gwak

2020 ◽  
Vol 18 (1) ◽  
Author(s):  
Patrick Kirchweger ◽  
Helwig Valentin Wundsam ◽  
Ines Fischer ◽  
Christiane Sophie Rösch ◽  
Gernot Böhm ◽  
...  

Abstract Background Ganglioneuromas (GNs) are extremely rare, slowly growing, benign tumors that can arise from Schwann cells, ganglion cells, and neuronal or fibrous tissues. Due to their origin from the sympathetic neural crest, they show neuroendocrine potential; however, most are reported to be hormonally inactive. Nevertheless, complete surgical removal is recommended for symptom control or for the prevention of potential malignant degeneration. Case Report A 30-year-old female was referred to our oncologic center due to a giant retroperitoneal and mediastinal mass detected in computed tomography (CT) scans. The initial symptoms were transient nausea, diarrhea, and crampy abdominal pain. There was a positive family history including 5 first- and second-degree relatives. Presurgical biopsy revealed a benign ganglioneuroma. Total resection (TR) of a 35 × 25 × 25 cm, 2550-g tumor was obtained successfully via laparotomy combined with thoracotomy and partial incision of the diaphragm. Histopathological analysis confirmed the diagnosis. Surgically challenging aspects were the bilateral tumor invasion from the retroperitoneum into the mediastinum through the aortic hiatus with the need of a bilateral 2-cavity procedure, as well as the tumor-related displacement of the abdominal aorta, the mesenteric vessels, and the inferior vena cava. Due to their anatomic course through the tumor mass, the lumbar aortic vessels needed to be partially resected. Postoperative functioning was excellent without any sign of neurologic deficit. Conclusion Here, we present the largest case of a TR of a GN with retroperitoneal and mediastinal expansion. On review of the literature, this is the largest reported GN resected and was performed safely. Additionally, we present the first systematic literature review for large GN (> 10 cm) as well as for resected tumors growing from the abdominal cavity into the thoracic cavity.


Author(s):  
Hanlu Tang ◽  
Tao Wu ◽  
Fu Zhao ◽  
Jing Zhang ◽  
Zhijun Yang ◽  
...  

2020 ◽  
Vol 36 (11) ◽  
pp. 2693-2700
Author(s):  
Stephanie T. Jünger ◽  
Felipe Andreiuolo ◽  
Martin Mynarek ◽  
Evelyn Dörner ◽  
Anja zur Mühlen ◽  
...  

Abstract Introduction Young age is an adverse prognostic factor in children with ependymomas. Treatment of these infants is challenging since beneficial therapeutic options are limited. As ependymomas are considered a biologically heterogeneous group, we aimed to characterize infant ependymomas with regard to their histological and genetic features. Materials and methods We analyzed 28 ependymomas occurring in children younger than 18 months at diagnosis enrolled into the HIT2000-E protocols with the aim to postpone irradiation until the age of 18 months if possible. All cases underwent neuropathological review, including immunohistochemical characterization. Genome-wide copy number alterations (CNA) were assessed by molecular inversion probe assays, and RELA and YAP1 fusions were detected by RT-PCR and sequencing. Results All infant ependymomas were anaplastic (WHO grade III). Twenty-one (75%) cases were located in the posterior fossa. Gross total resection was accomplished in 12 (57%) of these cases. All posterior fossa tumors showed loss of H3-K27me3 characteristic of PFA ependymomas. CNA analysis showed a stable genome in all cases with lack of chromosome 1q gain, an adverse prognostic marker in PFA ependymomas of older children. However, after a median follow-up of 5.4 years, 15 (71%) relapsed, and 9 (43%) died. Seven ependymomas (25%) occurred in the supratentorial region. Gross total resection could be achieved in only two of these cases. Four tumors carried C11orf95-RELA fusions, and two cases had typical YAP1-MAMLD1 fusions (one case was not analyzable). The RELA-fused cases did not display CDKN2A loss as an adverse indicator of prognosis in this disease entity. Although three infants (43%) with supratentorial ependymomas relapsed, all patients survived (median follow-up, 8.0 years). Conclusion Infant ependymomas seem to fall into three biological entities, with supratentorial tumors carrying RELA or YAP fusions and PFA posterior fossa ependymomas. The latter showed a poor outcome even though chromosome 1q gain was absent.


2019 ◽  
Vol 08 (03) ◽  
pp. 191-192
Author(s):  
Sushil Kumar ◽  
Sandeep Sharma ◽  
Rajneesh Misra ◽  
Kundan Kumar

AbstractEpidermoid cysts of the fourth ventricle are slow-growing benign rare lesions. We report a case of fourth ventricle epidermoid in a young lady with short duration of symptoms.


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