scholarly journals Dysembryoplastic Neuroepithelial Tumor: A Rare Brain Tumor with Excellent Seizure Control after Surgical Resection

Author(s):  
Rajnish Kumar Arora ◽  
Pranshu Bhargava ◽  
Poonam Arora ◽  
Prashant Joshi ◽  
Garga Basu ◽  
...  

Abstract Introduction Dysembryoplastic neuroepithelial tumor (DNET) is a rare cause of intractable epilepsy, which has excellent results in terms of seizure control after surgical resection. We present one such case, because of its rarity, to highlight the effect of tumor removal on seizure control, particularly DNET. Materials and Methods/Case Summary A 9-year-old male patient presented with sudden onset of partial seizures for the past 6 months. There were five episodes. In each episode, the patient engaged in irrelevant talk, followed by deviation of mouth to left and twitching movements. The episode lasted 5 minutes and there was no loss of consciousness. There was no aura or tongue bite, and in one of the episodes, the patient lost consciousness. There was no other significant positive history. On examination, the child was consciously alert, without focal neurological deficit or features of meningitis. There was no papilledema. The patient was on phenytoin sodium, phenobarbitone and clobazam. Magnetic resonance imaging (MRI) of brain was done with and without contrast. MRI revealed a lesion approximately 4.1 × 3.6 × 3.2 cm in the right medial temporal lobe. It was hypointense to brain on T1 and fluid-attenuated inversion recovery (FLAIR), and hyperintense on T2-weighted images. Diffusion restriction was present and there was minimal contrast uptake. There was no evidence of mass effect or midline shift After discussing the risks and benefits with parents, the patient underwent preanesthetic checkup, and was taken up for craniotomy and excision of tumor. Gross total excision was done. The child was started orally on day 1 postoperatively and ambulated. There were no further seizure episodes. The patient was continued on phenytoin and clobazam, and phenobarbitone was tapered gradually. At 6 months, the child was seizure-free. Conclusions DNET are rare tumors occurring early in life and presenting with intractable seizures. Surgical resection offers a good and safe chance for long-term seizure control.

2009 ◽  
Vol 124 (5) ◽  
pp. 552-556 ◽  
Author(s):  
F Barbieri ◽  
F Fiorino

AbstractIntroduction: Mastoid hyperpneumatisation predisposes to intracranial pneumatocoele development, due to the risk of rupture of the thin, bony walls. Intracranial pneumatocoele may be precipitated by even minor head trauma or an abrupt change in middle-ear pressure, with the potential risk of infectious or compressive intracranial complications.Case report: A 19-year-old man with mastoid hyperpneumatisation developed a chronic intracranial–epidural pneumatocoele of traumatic origin in the right parieto-occipital area, in contiguity with the posterior mastoid cells. Eighteen months later, after a common cold, the patient developed signs of intracranial hypertension, due to the pneumatocoele spreading to the right epidural anterior fossa. A large right mastoidectomy extended to the retrosigmoid cells was performed, and a watertight seal applied over a large retrosigmoid cell using bovine pericardium and a mixture of bone powder and fibrin glue.Results: The patient was discharged on post-operative day three with no symptoms. Ten days after surgery, computed tomography monitoring showed complete reabsorption of the pneumatocoele.Conclusion: In cases of chronic, otogenic, epidural pneumatocoele, the possibility of the sudden onset of serious complications suggests the need for early repair of the communication between the temporal bone and the intracranial compartments. Closure of the fistula using autogenic and/or allogenic materials is usually adequate to resolve the pneumatocoele.


2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Edward C. Mader ◽  
Bruce J. Fisch ◽  
Nicole R. Villemarette-Pittman ◽  
Piotr W. Olejniczak ◽  
Michael E. Carey

Intractable epilepsy with painful partial motor seizures is a relatively rare and difficult disorder to treat. We evaluated the usefulness of botulinum toxin to reduce ictal pain. Two patients received two or four botulinum toxin (BTX) injections at one-to-two-month intervals. Patient 1 had painful seizures of the right arm and hand. Patient 2 had painful seizures involving the left foot and leg. Injections were discontinued after improved seizure control following resective surgery. Both patients received significant pain relief from the injections with analgesia lasting at least two months. Seizure severity was reduced, but seizure frequency and duration were unaffected. For these patients, BTX was effective in temporarily relieving pain associated with muscle contraction in simple partial motor seizures. Our findings do not support the hypothesis that modulation of motor end-organ feedback affects focal seizure generation. BTX is a safe and reversible treatment that should be considered as part of adjunctive therapy after failure to achieve control of painful partial motor seizures.


2020 ◽  
Vol 11 ◽  
pp. 249
Author(s):  
Jiahua Huang ◽  
Finn Ghent ◽  
Michael Rodriguez ◽  
Mark Davies

Background: Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare entity which can occur at intracranial and spinal locations. Clinical presentation is due to local mass effect rather than tissue infiltration. Lesions causing significant symptoms or are showing radiological progression require surgical resection. Maximal surgical resection is considered curative for this non-neoplastic entity with only two cases of recurrence reported in the literature. Cranial nerve involvement is extremely rare and the presenting neurological deficit is unlikely to improve even with surgical intervention. Case Description: We describe a case of CAPNON at the right posterior clinoid process with involvement of the right oculomotor nerve in a 38-year-old male. Computed tomography demonstrated an amorphous mass which had intermediate to low T1 and T2 signal on magnetic resonance imaging. The oculomotor nerve was compressed with sign of atrophy. The patient underwent maximal surgical debulking for progressive symptoms of worsening pain and ophthalmoplegia. Postoperatively, the patient’s symptoms were stable but did not improve. Conclusion: Preoperative diagnosis of CAPNON is difficult due to its rarity and nonspecific clinical and radiological findings. Surgical resection is considered in cases with worsening symptoms, progression on serial imaging, or uncertain diagnosis. Relatively inaccessible lesions with little or no clinical symptoms can be observed.


2012 ◽  
Vol 2 ◽  
pp. 60 ◽  
Author(s):  
Harsha Guduru ◽  
Jun K. Shen ◽  
Harish S. Lokannavar

We present a rare case of dysembryoplastic neuroepithelial tumor, a rare benign glioneuronal tumor of the central nervous system. It generally occurs in the supratentorial region and the temporal cerebral cortex in children and young adults. The most common presentation is epilepsy. The supratentorial tumor without any signs of mass effect or peritumoral edema is the conventionally accepted diagnostic criteria. In this case of a 19-year-old male with intractable epilepsy, atypical features such as the location of the tumor and the presence of mass effect and peritumoral edema made imaging diagnosis difficult. Diagnosis was confirmed through histopathology. Due to its recent discovery and relatively rare occurrence it is important for radiologists to recognize this disease entity.


2010 ◽  
Vol 5 (1) ◽  
pp. 123-130 ◽  
Author(s):  
Edward F. Chang ◽  
Catherine Christie ◽  
Joseph E. Sullivan ◽  
Paul A. Garcia ◽  
Tarik Tihan ◽  
...  

Object Dysembryoplastic neuroepithelial tumors (DNETs) are a subset of relatively rare glioneuronal tumors that typically present with epilepsy during childhood. The authors' aim was to identify factors that predict seizure control following excision. Methods The authors reviewed the cases of 50 patients who underwent resection of DNETs at the University of California, San Francisco, between 1990 and 2006. Demographic, seizure history, radiographic, and histopathological data were collected and analyzed for statistical association with postoperative seizure control. Results Of the 50 patients, 86% presented with intractable epilepsy. The median age at surgery was 21 years (range 4–46 years; 40% were < 18 years old at time of surgery), with a median duration of 8 years from onset of seizures (24% were adult-onset seizures). Fifty-two percent of the cases were associated with adjacent focal cortical dysplasia. Complete resection was achieved in 78% of cases. Intraoperative electrocorticography in 23 patients identified extralesional interictal activity in 16 cases, which led to extended lesionectomy or lobectomy. The remaining patients underwent lesionectomy alone. The median follow-up was 5.6 years, during which time tumor progression occurred after subtotal resection. The proportional estimates of seizure freedom (Engel Class I outcome) were 0.86 at 1 year and 0.85 at 5 years. Seizure freedom was predicted by complete or extended resection (OR 1.68, 95% CI 1.39–2.03; p < 0.0001) and extratemporal location (OR 1.20, 95% CI 1.02–1.42; p = 0.03) on multivariate analysis. Secondary analysis for intraoperative electrocorticography cases demonstrated that seizure outcome was better when extralesional spiking foci were detected (94% seizure free) compared with when they were absent (43% seizure free). Conclusions Excision of DNETs and, when present, adjacent dysplastic cortex was highly effective for seizure control. Excellent seizure-free outcomes and tumor control were seen with lesionectomy alone in most cases. Electrocorticography with extended resection was useful for patients with pharmacoresistant epilepsy.


2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
S Youssef ◽  
S Iftikhar ◽  
M Haris ◽  
S Sundararajan ◽  
Q Abid

Abstract Introduction Cystic mediastinal lymphangiomas (CML) are exceedingly rare, representing &lt;1% of cystic lymphangiomas (CL), and typically present before 2 years of age. Few cases describe adult presentations of solitary CML and none describe symptomatic cases with ‘mass-effect’ complications. We describe an incidental finding of a symptomatic solitary CML causing mass effect complications and our definitive surgical management. Case Report A 44-year-old Caucasian male presented with reduced responsiveness due to mixed drug overdose of fluoxetine and ibuprofen. Plain chest radiograph (CXR) revealed incidental new mediastinal widening. Thoracic computed tomography (CT) showed a well-defined lesion (75x63mm) in the right paratracheal region, with associated compression of the superior vena cava, left brachiocephalic vein and displacement of the azygos vein. Further questioning revealed 6-months history of non-productive cough, worsening dyspnoea on exertion and chest pain radiating to the right of the chest. Complete surgical resection was performed via posterolateral thoracotomy. It was seen compressing the 2nd and 3rd intercostal nerves. Enlarged lymph nodes at station 4 and 10 were excised and sent for analysis with aspirate from the excised lesion. Pathological gross examination showed a multiloculated cystic lesion containing thin straw-coloured fluid. Microscopically, it was thin walled with smooth muscle and scattered clusters of lymphocytes; its lining was markedly attenuated comprising bland flat endothelial cells, positive for CD-31. Lymph node samples showed reactive changes. Conclusions Although CMLs are benign, their location and growth in the mediastinum can result in compression of surrounding structures. Early detection and complete surgical resection are paramount to prevent such complications and lesion recurrence.


2017 ◽  
Vol 31 (1) ◽  
pp. 27-31
Author(s):  
FM Moinuddin ◽  
Novita Ikbar Khairunnisa ◽  
Hirofumi Hirano ◽  
Tomoko Hanada ◽  
Tsubasa Hiraki ◽  
...  

This 48-year-old-man who had undergone right thyroid lobectomy for undifferentiated thyroid carcinoma nine years earlier developed generalized seizures. His cerebrospinal fluid was xanthochromic with elevation of total protein. Computed tomography (CT) showed mixed-density bilateral ventricular masses. Magnetic resonance imaging (MRI) revealed multiple nodules in both lateral ventricles; they were heterogeneously enhanced by gadolinium. Diffuse hyperintensity in the right medial temporal lobe and bilateral subependymal area was noted on fluid-attenuated inversion recovery images. Susceptibility-weighted imaging showed low intensity in the masses and cerebellar sulci suggesting hemorrhage and hemosiderin deposition. The preoperative diagnosis was disseminated malignant tumor with recurring hemorrhage. Histological examination of biopsy specimens showed clusters of cells with small uniform nuclei embedded in a dense fibrillary matrix of glial cells and microcystic degeneration. Pseudo-rosettes indicating ependymoma were absent. Microhemorrhages and hemosiderin deposits were noted. Immunohistochemically, the background fibrillary matrix and neoplastic cells were positive for glial fibrillary acidic protein. Mutated isocitrate dehydrogenase-1 was negative. The MIB-1 index was 1.5%. The tumor was pathologically diagnosed as subependymoma containing microhemorrhages and hemosiderin deposits. The extensive multiplicity and hemorrhage encountered in this case have rarely been reported in patients with subependymoma.


2014 ◽  
Vol 17 (4) ◽  
pp. 196
Author(s):  
Erhan Kaya ◽  
Halit Yerebakan ◽  
Daniel Spielman ◽  
Omer Isik ◽  
Cevat Yakut

Occlusion of a coronary artery by an acute type A aortic dissection presents a life-threatening emergency that is rarely seen and easy to misdiagnose. We present the case of a 75-year-old male who experienced sudden onset of severe left-sided chest pain due to an acute type A aortic dissection that obstructed the right coronary artery. Following an initial misdiagnosis of acute coronary syndrome, imaging revealed the presence of an aortic dissection. An emergency modified Bentall procedure was performed, in which the damaged aorta and aortic valve were replaced.


2011 ◽  
Vol 26 (10) ◽  
pp. 1246-1251 ◽  
Author(s):  
Dhruman Goradia ◽  
Harry T. Chugani ◽  
Rajkumar Munian Govindan ◽  
Michael Behen ◽  
Csaba Juhász ◽  
...  

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Lubna Bakr ◽  
Hussam AlKhalaf ◽  
Ahmad Takriti

Abstract Background Primary cardiac tumours are extremely rare. Most of them are benign. Sarcomas account for 95% of the malignant tumours. Prognosis of primary cardiac angiosarcoma remains poor. Complete surgical resection is oftentimes hampered when there is extensive tumour involvement into important cardiac apparatus. We report a case of cardiac angiosarcoma of the right atrium and ventricle, infiltrating the right atrioventricular junction and tricuspid valve. Case presentation Initially, a 22-year-old man presented with dyspnoea. One year later, he had recurrent pericardial effusion. Afterwards, echocardiography revealed a large mass in the right atrium, expanding from the roof of the right atrium to the tricuspid valve. The mass was causing compression on the tricuspid valve, and another mass was seen in the right ventricle. Complete resection of the tumour was impossible. The mass was resected with the biggest possible margins. The right atrium was reconstructed using heterologous pericardium. The patient’s postoperative course was uneventful. Postoperative echocardiography showed a small mass remaining in the right side of the heart. Histopathology and immunohistochemistry confirmed the diagnosis of angiosarcoma. The patient underwent adjuvant chemotherapy and radiotherapy later on. He survived for 1 year and 5 days after the surgery. After a diagnosis of lung and brain metastases, he ended up on mechanical ventilation for 48 h and died. Conclusions Surgical resection combined with postoperative chemotherapy and radiotherapy is feasible even in patients with an advanced stage of cardiac angiosarcoma when it is impossible to perform complete surgical resection.


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