Diagnosis and Management of Adult Intracranial Neurenteric Cysts

Neurosurgery ◽  
2011 ◽  
Vol 68 (1) ◽  
pp. 44-52 ◽  
Author(s):  
Liang. Wang ◽  
Junting. Zhang ◽  
Zhen. Wu ◽  
Guijun. Jia ◽  
Liwei. Zhang ◽  
...  

Abstract BACKGROUND: Intracranial neurenteric cysts are rare, benign lesions of the central nervous system. OBJECTIVE: To analyze our experience with these lesions and conduct a review of the literature with the aim of identifying a rational approach to diagnosis and management. METHODS: Seven patients underwent surgical treatment for intracranial neurenteric cysts from July 2000 to December 2008. The general strategy was to perform complete resection whenever possible. The clinical, radiological, operative, and pathological findings of the patients were reviewed retrospectively. RESULTS: This series included 3 women and 4 men. The age of hospitalized patients ranged from 19 to 55 years, with an average age of 34 years. Headache and dizziness were the most common chief symptoms. The imaging spectrum for these lesions is broad, leading to several preoperative misdiagnoses. All 7 lesions were resected by a far lateral transcondylar or retrosigmoid approach. Total tumor resection was achieved in 3 patients. Four patients presented transient fever postoperatively. All patients were healthy and showed no signs of tumor recurrence at their latest follow-up. CONCLUSION: Intracranial neurenteric cysts ventral to the brainstem are rare lesions occurring in young adults. These lesions should be considered in the differential diagnosis for intracranial extra-axial cystic lesions anterior to the brainstem. Total surgical resection seems to be the most effective therapeutic method, although radical resection at the risk of impairing the brainstem is not recommended. This benign lesion has a favorable overall prognosis.

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii326-iii326
Author(s):  
Shimpei Kusano ◽  
Junya Fujimura ◽  
Megumi Fujiwara ◽  
Akinori Yaguchi ◽  
Takeshi Ishibashi ◽  
...  

Abstract Embryonal tumor with multilayered rosettes (ETMR) is new entity defined in the 4th revised edition of the WHO classification of tumors of the central nervous system. Although radical resection, radiotherapy, and multiagent chemotherapy are considered to be necessary for ETMR, the efficacy of chemotherapy for ETMR in Japan has not been established. Here, we report different clinical courses for two children with localized ETMR treated with the St. Jude medulloblastoma-96 (SJMB96) regimen, which consists of four cycles of high-dose chemotherapy with autologous peripheral blood stem cell transplantation. For both children, the diagnosis of ETMR, C19MC-altered was confirmed after gross total tumor resection. Multiagent chemotherapy was administered following cranio-spinal irradiation with local boost. One month after completion of the treatment, one patient experienced local recurrence but has been in remission for over 2 years after tumor resection and stereotactic irradiation with a CyberKnife and treatment every three weeks with bevacizumab. The other patient also experienced local recurrence after the third cycle of chemotherapy and several times thereafter. Although she again underwent tumor resection and local irradiation, her tumor grew larger and invaded. Because her prognosis was very poor, her parents choose only palliative care. Based on our experience, we believe that continuous chemotherapy at conventional doses is preferred over intensive-dose chemotherapy such as SJMB96. However, the number of reports on chemotherapy for ETMR is still small, and a prospective multicenter trial is needed to establish effective chemotherapy for ETMR.


2013 ◽  
Vol 12 (3) ◽  
pp. 274-280 ◽  
Author(s):  
Smruti K. Patel ◽  
James K. Liu

Neurenteric cysts are rare and benign lesions that consist of ectopic alimentary tissue residing in the central nervous system. They tend to occur most frequently in an intraspinal rather than intracranial location. Intracranial neurenteric cysts are a rare occurrence in the pediatric population. These lesions typically present as unilateral cystic structures in the lower cerebellopontine angle and craniocervical junction. To the authors' knowledge, there have been no reported cases of bilateral localization of intracranial neurenteric cysts. In this report, they present an unusual case of a 10-year-old girl who was found to have bilateral intracranial neurenteric cysts at the pontomedullary junction. The patient was successfully treated with staged, bilateral far-lateral transcondylar resection of the cysts. The authors also provide a brief overview of the literature describing intracranial neurenteric cysts in children.


2005 ◽  
Vol 19 (2) ◽  
pp. 1-7 ◽  
Author(s):  
James K. Liu ◽  
William T. Couldwell

Neurenteric cysts are rare benign lesions of the central nervous system that are lined by endodermal cell–derived epithelium. Although they occur mostly in the spine, they can occur intracranially, most often in the posterior fossa. Neurenteric cysts that are located in the anterior cervicomedullary junction are even rarer and often require a skull base approach for adequate resection. The authors describe two cases of neurenteric cysts arising from the cervicomedullary junction that were resected via a far-lateral transcondylar approach. They discuss the surgical approach and operative nuances involved in removing these lesions, and review the clinical presentation of neurenteric cysts in this region as well as the neuroimaging characteristics, histopathological findings, and surgical management. Intraoperative videos are presented.


2010 ◽  
Vol 6 (6) ◽  
pp. 586-591 ◽  
Author(s):  
Hilary Highfield Nickols ◽  
Lola B. Chambless ◽  
Robert P. Carson ◽  
Cheryl M. Coffin ◽  
Matthew M. Pearson ◽  
...  

Intramedullary spinal cord teratomas are rare entities in infants. Management of these lesions is primarily surgical, with outcome dependent on rapid surgical decompression and complete gross-total tumor resection. The lesions are typically of the mature type, with immature teratomas displaying unique pathological features. The authors report a case of an extensive intramedullary immature teratoma in an infant with resolution of quadriplegia following grosstotal radical resection. At the 1-year follow-up, there was radiographic evidence of tumor, and surgical reexploration yielded portions of immature teratoma and extensive gliosis.


BMC Cancer ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Cristian Urla ◽  
Steven W. Warmann ◽  
Monika Sparber-Sauer ◽  
Andreas Schuck ◽  
Ivo Leuschner ◽  
...  

Abstract Background Biliary rhabdomyosarcoma (RMS) is the most common biliary tumor in children. The management of affected patients contains unique challenges because of the rarity of this tumor entity and its critical location at the porta hepatis, which can make achievement of a radical resection very difficult. Methods In a retrospective chart analysis we analysed children suffering from biliary RMS who were registered in three different CWS trials (CWS-96, CWS-2002P, and SoTiSaR registry). Results Seventeen patients (12 female, 5 male) with a median age of 4.3 years were assessed. The median follow-up was 42.2 months (10.7–202.5). The 5-year overall (OS) and event free survival (EFS) rates were 58% (45–71) and 47% (34–50), respectively. Patients > 10 years of age and those with alveolar histology had the worst prognosis (OS 0%). Patients with botryoid histology had an excellent survival (OS 100%) compared to those with non-botryoid histology (OS 38%, 22–54, p = 0.047). Microscopic complete tumor resection was achieved in almost all patients who received initial tumor biopsy followed by chemotherapy and delayed surgery. Conclusion Positive predictive factors for survival of children with biliary RMS are age ≤ 10 years and botryoid tumor histology. Primary surgery with intention of tumor resection should be avoided.


2020 ◽  
pp. 1-13 ◽  
Author(s):  
Matteo Zoli ◽  
Giacomo Sollini ◽  
Laura Milanese ◽  
Emanuele La Corte ◽  
Arianna Rustici ◽  
...  

OBJECTIVESurgical treatment of orbital lesions is challenging because complex approaches with a high risk of postoperative sequelae are required. Recently, minimally invasive endoscopic approaches through endonasal (EEA) and transpalpebral (ETP) routes have been proposed. The objective of this study was to assess outcomes of EEA and ETP in the authors’ series of patients with orbital lesions.METHODSData from all patients who underwent operations for an orbital tumor through an endoscopic approach at the authors’ institution from 2002 to 2018 were retrospectively collected. All patients underwent preoperative MRI and ophthalmological evaluation, which was repeated 3 months after surgery and then at regular follow-up intervals. A systematic review of the literature was also performed using Medline, Embase, and Web of Science databases.RESULTSThe series includes 23 patients (14 males); the mean patient age was 48 ± 23.9 years. Most of the lesions were intraconal (n = 19, 83%). The more frequent histotype was cavernous hemangioma (n = 5, 22%). Exophthalmos was the most common symptom (21 of 23 patients). EEA was performed in 16 cases (70%) and ETP in 7 (30%). The aim of the surgery was achieved in 94% of the cases after an EEA (successful biopsy in 5 of 6 cases and radical resection in all 10 remaining patients), and in 86% after an ETP (successful biopsy in 2 cases and radical tumor resection in 4 of 5 cases). Complications consisted of 3 cases (13%) of transitory diplopia. One recurrence (4%) was observed at follow-up (mean 59 ± 55 months).CONCLUSIONSThe EEA and ETP have demonstrated to be safe and effective for tumors located respectively in medial and lateral quadrants, permitting one to approach orbital lesions endoscopically at 360°. Innovative surgical tools, including intraoperative ultrasonography, may be useful to potentially reduce surgical morbidity. Larger series are needed to validate these preliminary suggestions.


Author(s):  
Saravana Selvan V. ◽  
Mekaladevi A. ◽  
Ramya M.

<p class="abstract">Oncocytic laryngeal cyst is an uncommon benign lesion of larynx that develops in the supraglottic area. Oncocytic laryngeal cyst arises from the ventricle and the patient presented with hoarseness of voice. Management of these lesions is conservative and consists of local excision. It is metaplasia of respiratory or glandular salivary epithelium with no risk of malignancy although recurrence after excision can still occur. It usually presents with hoarseness of voice although acute and chronic dyspnea may occur as well. Although oncocytic cysts are benign lesions, follow-up is recommended, as recurrence is possible.</p>


Neurosurgery ◽  
2008 ◽  
Vol 62 (4) ◽  
pp. 825-832 ◽  
Author(s):  
Liang Chen ◽  
Ying Mao ◽  
Hong Chen ◽  
Liang-Fu Zhou

Abstract OBJECTIVE Intracranial malignant peripheral nerve sheath tumors (MPNSTs) are rare and generally carry a poor prognosis. We have analyzed our experience with MPNSTs and conducted a review of the literature in an attempt to identify a rational approach to the management of these tumors. METHODS Eight patients underwent surgical treatment for intracranial MPNSTs during a 10-year period from 1996 to 2005. The general strategy was to perform complete resection whenever possible and to provide adjuvant radiotherapy for residual tumor. Chemotherapy was not used in this group. The clinical, radiological, operative, and pathological findings of the patients were reviewed retrospectively. RESULTS Six of the eight patients underwent primary operations; two of the eight patients had previously undergone other transcranial surgery operations. Total tumor resection was achieved in five patients. At this time, two have been recurrence-free for 3.5 and 5 years, respectively, and the other three patients had a mean postoperative survival of 7 months. There was one case of near total (&gt;90%) and two cases of partial (&lt;90%) tumor removal; the postoperative survival rate was 4, 4, and 2 months, respectively. Only two patients in our group accepted radiotherapy after surgery; one survived only 4 months and the other has been recurrence-free for 5 years. CONCLUSION MPNSTs are fast-growing, invasive tumors with rather unsatisfactory outcomes. Total surgical resection seems to be the most effective therapeutic method, and radiotherapy may play a role in local control.


2020 ◽  
Vol 31 (1) ◽  
pp. 45-51
Author(s):  
Federica Pozzati ◽  
Francesca Moro ◽  
Martina Leombroni ◽  
Valentina Bertoldo ◽  
Nausica Trivellizzi ◽  
...  

ObjectiveUltrasound examination represents the most important diagnostic method to preoperatively assess gynecological diseases. However, the ultrasound characteristics of vaginal pathologies are poorly investigated. The aim of this study was to describe the clinical and ultrasound characteristics of vaginal lesions detected at ultrasound.MethodsThis was a single center, prospective, observational study including patients with vaginal masses examined from January 2017 to May 2019. Morphologic sonographic characteristics of the lesions were described as unilocular, multilocular, unilocular-solid, multilocular-solid, and solid. For the analysis, patients were grouped into a 'malignant group', including patients with confirmed malignancy at final histology, and a 'benign group', including patients with a confirmed benign pathology at final histology and patients without a histological diagnosis but with a lesion that manifested no changes during follow-up.Results44 patients were enrolled. 22 (50%) of 44 lesions were benign: 12 (54.5%) of these underwent ultrasound follow-up and did not show any changes at the 12 month follow-up whereas 10 (45.5%) lesions had surgical excision which confirmed the benign nature. The remaining 22 (50%) of 44 lesions underwent surgery because of suspicion of malignancy: histology confirmed a malignancy in 20 (90.9%) of 22 cases. Benign lesions were described as follow: 11/24 (45.8%) unilocular, 3/24 (12.5%) multilocular with two locules, and 10/24 (41.7%) solid lesions. Malignant lesions were solid in 19/20 (95%) cases and multilocular-solid in 1/20 (5%). Most benign lesions had a color score of 1–2 (20/24, 83.4%) while malignant lesions had a color score of 3–4 (18/20, 90%).ConclusionA typical ultrasound image of a benign lesion was a unilocular cyst or hypoechoic solid mass with no or minimal vascularization on color Doppler examination. Malignant vaginal lesions were hypoechoic solid tumors with irregular margins and moderate/rich vascularization or multilocular-solid. Ultrasound should be used to supplement the clinician in the management of vaginal lesions.


Author(s):  
Sara Kammerer ◽  
Monika Mueller-Eschner ◽  
Arne Lauer ◽  
Anna-Luisa Luger ◽  
Johanna Quick-Weller ◽  
...  

Purpose Intracranial subependymomas are rare, mostly asymptomatic tumours, which are often found incidentally and therefore did not receive much attention in previous literature. By being classified as benign grade I in the WHO classification of tumours of the central nervous system, they are given a special status compared to the other ependymal tumours. Tumor recurrences are a rarity, spinal “drop metastases” do not occur. While etiological, pathological and therapeutic characteristics have been subject of several publications over the last few decades and have meanwhile been well studied, the imaging characteristics are much less well received. Material and method Retrospective analysis of our relatively large group of 33 patients with subependymoma, including 4 patients with a mixture of subependymomas with ependymal cell fractions in terms of imaging and clinical aspects and with reference to a current literature review. Results Subependymomas have typical image morphologic characteristics that differentiate them from tumors of other entities, however, the rare subgroup of histopathological mixtures of subependymomas with ependymal cell fractions has no distinctly different imaging properties. Conclusions Knowing the imaging characteristics of subpendymoma and their differential diagnoses is of particular importance in order to be able to decide between the necessity of follow-up controls, an early invasive diagnosis or, depending on the entity, tumor resection. Key Points:  Citation Format


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