scholarly journals Management challenges associated with a pineal region chordoma: illustrative case

2021 ◽  
Vol 1 (24) ◽  
Author(s):  
Ji-Eyon Kwon ◽  
So Young Ji ◽  
Kihwan Hwang ◽  
Kyu Sang Lee ◽  
Gheeyoung Choe ◽  
...  

BACKGROUND Chordomas, which are rare malignant neoplasms arising from notochordal remnants, often cause gradually progressive clinical symptoms. Intradural cranial chordomas (ICCs) are extremely rare and generally have a favorable prognosis. However, the authors reported the case of a primary ICC originating in the pineal gland presenting with recurrent thalamic hemorrhage and displaying an aggressive postoperative clinical course. OBSERVATIONS A 41-year-old man arrived at the emergency department with morning headaches and recurrent syncope that had lasted several months. Computed tomography and magnetic resonance imaging (MRI) revealed a pineal gland mass causing obstructive hydrocephalus and a subacute hematoma in the right thalamus. Three weeks after an endoscopic third ventriculostomy was performed, recurrent hemorrhage was observed in the right thalamus. The tumor was promptly removed surgically. The yellowish-white tumor did not exhibit abundant bleeding. No evidence of intratumoral hemorrhage around the hematoma pocket was found. Histopathological examination revealed the characteristics of a chordoma with minimal vascularity. MRI performed 10 weeks postoperatively for worsening headaches revealed abnormal enhancement of multiple cranial nerves, suggesting leptomeningeal seeding (LMS) of the tumor. LESSONS Despite radiotherapy and intrathecal chemotherapy, the patient’s neurological status worsened; he died 2 years postoperatively. A pineal ICC may cause recurrent thalamic hemorrhage and potentially fatal LMS, even in the early postoperative period.


2021 ◽  
Vol 2 (6) ◽  
Author(s):  
Renato J. Galzio ◽  
Mattia Del Maestro ◽  
Diamantoula Pagkou ◽  
Massimo Caulo ◽  
Sofia Asioli ◽  
...  

BACKGROUND The authors reported the first documented case of intracranial extraaxial nonneurofibromatosis type 1–related nontriton malignant peripheral nerve sheath tumor (MPNST) originating from the falx cerebri. OBSERVATIONS A 34-year-old man with headache, short-term memory deficit, postural instability, and blurred vision presented with a large heterogenous contrast-enhanced intraventricular cystic lesion originating from the free margin of the falx cerebri. The patient received surgery using the right posterior interhemispheric approach. Gross total resection was performed, and the inferior border of the falx cerebri was resected. The postoperative course was uneventful. Histological examination revealed hypercellular foci of neoplastic spindle cells with hyperchromatic and wavy nuclei. Hence, a diagnosis of MPNST was made based on concomitant immunochemistry findings, including mouse double minute 2 homolog focal positivity and geographic loss of H3K27me3. The patient received adjuvant radiotherapy, and recurrence was not observed. LESSONS Intracranial MPNSTs are extremely rare tumors, typically originating from the cranial nerves in the posterior cranial fossa. An even rarer variant of these tumors, referred to as malignant intracerebral nerve sheath tumors, may directly arise from the brain parenchyma. The authors reported the first case of an intracranial MPNST originating from the dura mater of the falx cerebri, acting as an extraaxial lesion with prevalent expansion in the right ventricle.



2009 ◽  
Vol 110 (5) ◽  
pp. 858-860 ◽  
Author(s):  
Eelco W. Hoving ◽  
Mehrnoush Rahmani ◽  
Leonie I. Los ◽  
Victor W. Renardel de Lavalette

A serious ophthalmological complication of an endoscopic third ventriculostomy that created an iatrogenic Terson syndrome is described. A patient with an obstructive hydrocephalus was treated endoscopically, but due to the inadvertent use of a pressure bag during rinsing, in combination with a blocked outflow channel, a steep rise in intracranial pressure occurred. Postoperatively the patient experienced disturbed vision caused by bilateral retinal hemorrhages, and an iatrogenic Terson syndrome was diagnosed. The pathogenesis of Terson syndrome is discussed based on this illustrative case.



Folia Medica ◽  
2019 ◽  
Vol 61 (1) ◽  
pp. 143-147
Author(s):  
Ivo Kehayov ◽  
Vladimir Nakov ◽  
Borislav Kitov ◽  
Hristo Zhelyazkov ◽  
Toma Spiriev

Abstract We report on a case of a solid adamantinomatous variant of craniopharyngioma located entirely within the third ventricle causing asymmetric obstructive hydrocephalus in a 43-year-old male patient. The patient complaints included intermittent severe headache and progressive bilateral visual field loss. Initially, the lesion was accessed via the bifrontal interhemispheric translamina terminalis approach but total removal was not possible due to short anterior communicating artery which limited the exposure. In the second stage, we used the right interhemispheric transcallosal transforaminal approach and achieved total tumor removal followed by microscopic third ventriculostomy. The present article discusses the selection of appropriate surgical approach based on concise literature review that provides favorable surgical management of these rare lesions.



2019 ◽  
Vol 16 (3) ◽  
pp. 68-71
Author(s):  
Abhishek Tamrakar ◽  
Pritam Gurung ◽  
Samir Acharya ◽  
Pravesh Rajbhandhari ◽  
Basant Pant

Lhermitte–Duclos Disease is a rare entity characterized by diffuse or focal enlargement of cerebellar folia. Clinical manifestations are usually related to a mass effect and secondary obstructive hydrocephalus. Increased intracranial pressure symptoms and cerebellar symptoms are the most frequent patient complaints. We present a 10-month-old male child with his mother who presented to the outpatient department with complaints of enlargement of size of head since the last one week. Magnetic Resonance Imaging scan revealed the features of Lhermitte–Duclos Disease involving the right cerebellar hemisphere with marked post contrast gyral enhancement. He underwent endoscopic third ventriculostomy. Lhermitte–Duclos Disease is very rare in early childhood and should be considered in the differential diagnosis of posterior fossa lesions.



2018 ◽  
Vol 16 (4) ◽  
pp. E130-E133
Author(s):  
Derek G Southwell ◽  
Jonathan D Breshears ◽  
William R Lyon ◽  
Michael W McDermott

Abstract BACKGROUND AND IMPORTANCE Skull base surgery involves the microdissection and intraoperative monitoring of cranial nerves, including cranial nerve XI (CN XI). Manipulation of CN XI can evoke brisk trapezius contraction, which in turn may disturb the surgical procedure and risk patient safety. Here we describe a method for temporarily silencing CN XI via direct intraoperative application of 1% lidocaine. CLINICAL PRESENTATION A 41-yr-old woman presented with symptoms of elevated intracranial pressure and obstructive hydrocephalus secondary to a hemangioblastoma of the right cerebellar tonsil. A far-lateral suboccipital craniotomy was performed for resection of the lesion. During the initial stages of microdissection, vigorous trapezius contraction compromised the course of the operation. Following exposure of the cranial and cervical portions of CN XI, lidocaine was applied to the course of the exposed nerve. Within 3 min, trapezius electromyography demonstrated neuromuscular silencing, and further manipulation of CN XI did not cause shoulder movements. Approximately 30 min after lidocaine application, trapezius contractions returned, and lidocaine was again applied to re-silence CN XI. Gross total resection of the hemangioblastoma was performed during periods of CN XI inactivation, when trapezius contractions were absent. CONCLUSION Direct application of lidocaine to CN XI temporarily silenced neuromuscular activity and prevented unwanted trapezius contraction during skull base microsurgery. This method improved operative safety and efficiency by significantly reducing patient movement due to the unavoidable manipulation of CN XI.



2021 ◽  
Vol 9 (11) ◽  
pp. 454-458
Author(s):  
Reem Mohammed Alshiakh ◽  
◽  
Ahmad Omar Alghamdi ◽  
Suhad Ali Aljuhani ◽  
Hadeel Hamid Ahmad ◽  
...  

Malignant neoplasms of the colon and rectum are often missed in patients younger than 45 years, the uncertainty of the diagnosis by the surgical team due to unavailability of clear screening guidelines in this population may be one of the reasons. The objective of this case report is to highlight the occurrence of colon cancer in a patient younger than 45 years to avoid the delay in management and the need for clear screening guidelines. Our patient, a 30 years old lady with an average risk of colon cancer demonstrated clinical symptoms and signs suggestive of partial bowel obstruction. After full radiological and laboratory investigations, she was initially diagnosed with inflammatory vs infectious cause of this obstruction. Colonoscopy showed a mass in the right hepatic flexure and sessile polyp in the transverse colon, Histopathology result showed moderately differentiated colon cancer. The patient was taken to the operation theater, right extended hemicolectomy was done, final histopathology result showed stage IIIc cancer, the patient was sent for oncology. In a conclusion, Colon cancer in patients younger than 45 years old requires a high index of suspicion by the surgeon and the managing team despite the unavailability of clear guidelines for screening at a young age.



Author(s):  
Anne Soejbjerg ◽  
Suzan Dyve ◽  
Steen Baerentzen ◽  
Georg Thorsell ◽  
Per L Poulsen ◽  
...  

Summary Solitary sellar plasmacytomas are exceedingly rare and difficult to distinguish from other pituitary tumors. We report a case of a 62-year-old woman presenting with blurred vision of the right eye and tenderness of the right temporal region, which was interpreted as temporal arteritis. MRI revealed a pituitary mass lesion (20mm×14mm×17mm) without compression of the optic chiasm and her pituitary function was normal. Pituitary surgery was undertaken due to growth of the lesion, and histopathological examination showed a highly cellular neoplasm composed of mature monoclonal plasma cells. Subsequent examinations revealed no evidence of extrasellar myeloma. The patient received pituitary irradiation and has remained well and free of symptoms apart from iatrogenic central diabetes insipidus. Until now, only eight cases of solitary sellar plasmacytoma have been reported. Most frequent symptoms stem from compression of the cranial nerves in the cavernous sinus (III, IV, V), whereas the anterior pituitary function is mostly intact. Learning points A solitary plasmacytoma is a rare cause of a sellar mass lesion. The radiological and clinical features are nonspecific, but cranial nerve affection and intact pituitary function are usually present. The diagnosis is made histologically and has important therapeutic implications.



2016 ◽  
Vol 44 (1) ◽  
pp. 5
Author(s):  
Ozlem Ozmen ◽  
Yusuf Sinan Sirin

Background: Insulinomas are tumors of the pancreatic islet beta cells that secreting insulin. They are malignant neoplasms and rarely seen in dogs. Because insulinomas secrete excessive insulin and hypoglycemia occur. The most common clinical symptoms are seizures, extreme weakness, and other neurological abnormalities. The tumors typically release insulin episodically, with clinical signs being seen intermittently as a result. These are most often associated with prolonged starvation or prolonged periods of exertion. Insulinoma occurs most often in middle-aged and older dogs, averaging nine years in age. They affect both sexes and are more likely in larger breeds. The most commonly afflicted breeds are German Shepherds, Irish Setters, Boxers, Golden Retrievers and Terriers. Case: In this study, a case of malignant insulinoma with multiorgan metastasis in a 7-year-old hunting dog that brought to the clinic with complaints of constipation and inappetence were evaluated by clinical, cytological, histopathological and immunohistochemical fndings. Owner stated that the animal suffers from constipation for 4 days, but no vomitus or nervous symptoms were present. Clinical efforts not solved the constipation. For to the defnitive diagnosis the dog decided to underwent laparotomy. After a midline abdominal approach multiple whitish, hard and irregular metastatic tumoral masses were observed in different abdominal organs. Because of the poor prognosis and according to owner’s acceptance the dog euthanatized at the same time. At necropsy, a bloody serous fluid approximately 500 mL was found in the abdominal cavity. The intestines were hyperemic and hemorrhagic in some areas. A hemorrhagic 9x5x3 cm in diameter mass was observed on the pancreas. Mesenterial adhesions were formed due to mass between duodenum and jejunum. The mass was moderately hard and cut surface was bloody, necrosis was observed in some areas. At the gross examination, whitish color, various sized and multiple metastatic masses were noted in spleen, liver, lymph nodes and lungs. Before formalin fxation, impression smears were made from the pancreatic mass. In cytological preparations of the lesion, epithelial cells in different shapes and sizes were observed. At the histopathological examination, the tumor mass consisted of anaplastic, pleomorphic cells that have pale cytoplasm, vesicular nucleus and prominent nucleolus. Diffuse necrotic areas were noted in the mass. Similar metastatic masses were seen in liver, spleen, lungs and kidneys. The tumor cells were positive for insulin and negative for glucagon immunohistochemically. Discussion: Insulinomas generally caused nervous symptoms due to hypoglycemia. Metastasis may occur in some cases and generally lymph node metastasis occurs. Spleen metastasis are very rare in insulinoma cases. The mean age of the occurrence of insulinoma is generally older than 9-year-old. In this case, a case of malignant insulinoma with multiorgan metastasis and caused constipation due to intestinal adhesions in a dog was presented with clinical and pathological fndings. Best of the author’s knowledge insulinoma as a cause of constipation not previously reported in a dog. The aim of this study was to report a case of metastatic insulinoma without neurological symptoms but constipation in a 7 years old hunting dog by clinical, pathological, cytological and immunohistochemical fndings. Keywords: Malignant insulinoma, constipation, cytology, histopathology, immunohistochemistry, dog.



2019 ◽  
Vol 08 (03) ◽  
pp. 151-154
Author(s):  
Prarthana Saxena ◽  
Sharad Pandey ◽  
Pankaj Kumar ◽  
Kaviraj Kausik ◽  
Shiv Lal Soni

Abstract Background With the recent advances in optical and mechanical instrumentation, endoscopic third ventriculostomy (ETV) has emerged as the procedure of choice for the treatment of hydrocephalus in selected patients. Objective: To study the role of ETV in therapeutic management of hydrocephalus in a tertiary care center. Materials and Methods The present study was conducted in the Department of Neurosurgery at Dr. Ram Manohar Lohia Hospital. Endoscopic third ventriculostomy was performed as a therapeutic procedure in patients diagnosed with hydrocephalus requiring surgical intervention and admitted from June 2017 to July 2018. ETV success was defined by resolution or improvement in clinical symptoms and ETV failure was considered in patients whose symptoms either deteriorated or did not improve from the baseline and required ventriculoperitoneal shunt. Results A total of 85 patients were included in this study who underwent the therapeutic ETV. In the present study, the overall ETV success rate was 75.2% (64/85). 86.7% cases of aqueductal stenosis, 74.4% cases of post meningitis (including tubercular meningitis) hydrocephalus, and 71% of posterior fossa tumors showed resolution or improvement in clinical symptoms. Conclusion ETV is an effective treatment modality for obstructive hydrocephalus and with better results in post meningitis hydrocephalus and post tubercular meningitis hydrocephalus. However, more extensive studies dedicated to ETV with a larger sample size are required to further study its efficacy in various etiologies.



2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Rong Guo ◽  
Mingming Zhou ◽  
Xiaohui Wei ◽  
Ling Niu

Objective. To investigate the clinical characteristics of tracheobronchopathia osteochondroplastica (TO). Methods. The clinical data of six patients with TO from November 2016 to November 2018 were retrospectively analyzed. The etiology, clinical manifestations, diagnosis, and treatment of TO were summarized. Result. All six patients with TO were middle-aged males, confirmed by histopathological examination. The main clinical symptoms were cough, sputum, hemoptysis, chest pain, and repeated pulmonary infection. Some patients could make a preliminary diagnosis by chest CT, and bronchoscopy showed that TO mainly occurred in the trachea and the main bronchus and was more likely to invade the right bronchus. The treatment mainly includes anti-infection, phlegm-resolving, and other symptomatic treatment. Conclusion. TO is a benign disease predisposing to adults, and males are more likely to be affected. Its clinical manifestations are lack of specificity, and the cause may be related to chronic infection. Bronchoscopy combined with histopathological examination is the primary approach for the diagnosis of TO. There is no well-recognized treatment standard for TO, and the judgment of therapeutic effect is inconsistent. It is necessary to improve the understanding of this disease from a clinical perspective.



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