scholarly journals Failure to Thrive Revealing a Pilomyxoid Astrocytoma: An Uncommon Case Report with Literature Review

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Salma Benyakhlef ◽  
Abir Tahri ◽  
Asmaa Khlifi ◽  
Hajar Abdelouahab ◽  
Kamaoui Imane ◽  
...  

Pilomyxoid astrocytoma (PMA) is a freshly described figure of low-grade neoplasms encountered in early childhood. Nevertheless, its precise classification by the World Health Organization (WHO) is still debatable. Making an exact diagnosis relies on histological and immunohistochemical pathognomonic features with specific radiological findings. PMA behaves aggressively with a shorter progression-free survival, and its management is unfortunately still arguable. We describe a rare case of PMA involving the suprasellar region who displays symptoms consistent with diencephalic syndrome. The diagnosis was made by magnetic resonance imaging (MRI) focused on the hypothalamic-pituitary axis, and the patient underwent a subtotal tumor resection combined with chemotherapy. Diagnosis of brain tumors should be kept in mind in young children with generalized and severe unexplained loss of subcutaneous fat with failure to thrive after ruling out classical causes.

2021 ◽  
Vol 9 (F) ◽  
pp. 25-30
Author(s):  
Dirga Rachmad Aprianto ◽  
Rahadian Indarto Susilo ◽  
Joni Wahyuhadi ◽  
Irwan Barlian Immadoel Haq

BACKGROUND: Meningioma is mostly benign tumor (World Health Organization Grade 1) and surgery remains the best option in treating symptomatic or enlarging meningiomas where total removal of the tumor is the goal of surgery. Radiation therapy has shown to be effective to cease the growth of the tumor, but not in tumor regression. Adjuvant therapy may treat patients with recurrence or unresectable meningiomas yet the uses of hormone therapy, immunotherapy, or chemotherapy had many results and were not consistently effective. Hydroxyurea has promising results in patients with meningiomas. AIM: This study analyzed the efficacy and safety of hydroxyurea for the treatments of recurrence or unresectable meningiomas. MATERIALS AND METHODS: The study adapted PRISMA guidelines by searching electronic databases, PUBMED, Cochrane, and JNS in August 2020 and was full-text observational study or randomized control trial presented as PICO and assessed using the risk-of-bias assessment tool. RESULTS: A total of six articles (157 patients with meningioma) were reviewed from the total of 425. Hydroxyurea was administered orally for 28 days continuously and repeated every 28 days or after recovery with various dosages in six studies. DISCUSSION: Administration of hydroxyurea showed a varied stable disease rate ranging from 30 to 69% with a median progression-free survival med varying between 2 and 27.75 months. The studies performed oral hydroxyurea administration at a dose of 20–30 mg/kg body weight/day or 1000 mg/m2/day. However, the adverse events (AEs) that appear also, based on literature, are not much different from other chemotherapy administrations. CONCLUSION: Patients with unresected and recurrent meningiomas have limited treatment options due to difficulty for surgical management. However, this study offers another perspective addressing the efficacy and safety results with the use of hydroxyurea. Overall, hydroxyurea showed good outcomes, particularly in low-grade meningioma, with relatively low AEs. Further combination treatment may be used as a multimodal therapy.


Neurosurgery ◽  
2004 ◽  
Vol 55 (2) ◽  
pp. 358-371 ◽  
Author(s):  
Christopher Nimsky ◽  
Atsushi Fujita ◽  
Oliver Ganslandt ◽  
Boris von Keller ◽  
Rudolf Fahlbusch

Abstract OBJECTIVE: To investigate the contribution of high-field intraoperative magnetic resonance imaging (iMRI) for further reduction of tumor volume in glioma surgery. METHODS: From April 2002 to June 2003, 182 neurosurgical procedures were performed with a 1.5-T magnetic resonance system. Among patients who underwent these procedures, 47 patients with gliomas (14 with World Health Organization Grade I or II glioma, and 33 with World Health Organization Grade III or IV glioma) who underwent craniotomy were investigated retrospectively. Completeness of tumor resection and volumetric analysis were assessed with intraoperative imaging data. RESULTS: Surgical procedures were influenced by iMRI in 36.2% of operations, and surgery was continued to remove residual tumor. Additional further resection significantly reduced the percentage of final tumor volume compared with first iMRI scan (6.9% ± 10.3% versus 21.4% ± 13.8%; P < 0.001). Percentages of final tumor volume also were significantly reduced in both low-grade (10.3% ± 11.5% versus 25.8% ± 16.3%; P < 0.05) and high-grade gliomas (5.4% ± 9.9% versus 19.5% ± 13.0%; P < 0.001). Complete resection was achieved finally in 36.2% of all patients (low-grade, 57.1%; high-grade, 27.3%). Among the 17 patients in whom complete tumor resection was achieved, 7 complete resections (41.2%) were attributable to further tumor removal after iMRI. We did not encounter unexpected events attributable to high-field iMRI, and standard neurosurgical equipment could be used safely. CONCLUSION: Despite extended resections, introduction of high-field iMRI in conjunction with functional navigation did not translate into an increased risk of postoperative deficits. The use of high-field iMRI increased radicality in glioma surgery without additional morbidity.


2020 ◽  
Author(s):  
Achiraya Teyateeti ◽  
Connie S Geno ◽  
Scott S Stafford ◽  
Anita Mahajan ◽  
Elizabeth S Yan ◽  
...  

Abstract Background Patterns of recurrence and survival with different surgical and radiotherapy (RT) techniques were evaluated to guide RT target volumes for patients with temporal lobe glioma. Methods and Materials This retrospective cohort study included patients with World Health Organization grades II to IV temporal lobe glioma treated with either partial (PTL) or complete temporal lobectomy (CTL) followed by RT covering both the parenchymal and dural resection bed (whole-cavity radiotherapy [WCRT]) or the parenchymal resection bed only (partial-cavity radiotherapy [PCRT]). Patterns of recurrence, progression-free survival (PFS) and overall survival (OS) were evaluated. Results Fifty-one patients were included and 84.3% of patients had high-grade glioma (HGG). CTL and PTL were performed for 11 (21.6%) and 40 (78.4%) patients, respectively. Median RT dose was 60 Gy (range, 40-76 Gy). There were 82.4% and 17.6% of patients who received WCRT and PCRT, respectively. Median follow-up time was 18.4 months (range, 4-161 months). Forty-six patients (90.2%) experienced disease recurrence, most commonly at the parenchymal resection bed (76.5%). No patients experienced an isolated dural recurrence. The median PFS and OS for the PCRT and WCRT cohorts were 8.6 vs 10.8 months (P = .979) and 19.9 vs 18.6 months (P = .859), respectively. PCRT was associated with a lower RT dose to the brainstem, optic, and ocular structures, hippocampus, and pituitary. Conclusion We identified no isolated dural recurrence and similar PFS and OS regardless of postoperative RT volume, whereas PCRT was associated with dose reduction to critical structures. Omission of dural RT may be considered a reasonable alternative approach. Further validation with larger comparative studies is warranted.


Author(s):  
Mizuho Inoue ◽  
Mohamed Labib ◽  
Alexander Yang ◽  
A. Samy Youssef

AbstractA case of a recurrent sphenocavernous meningioma is presented. The patient is a 42-year-old male who presented with an episode of transient right-sided numbness. A magnetic resonance imaging (MRI) revealed a large left sphenocavernous meningioma. The patient underwent a frontotemporal craniotomy for tumor resection. Near total resection was achieved with minimal residual in the left cavernous sinus (CS) and orbital apex. The pathology was consistent with meningioma, World Health Organization (WHO) grade I. A follow-up MRI was done 9 months after surgery and showed a growth of the residual tumor, which was treated with intensity modulated radiotherapy. Tumor growth was detected on serial imaging over a 4-year period. Surgical resection was offered. A left frontotemporal craniotomy with pretemporal transcavernous approach was performed. The bone flap was reopened and the dura was opened in a Y-shaped fashion. The roof of the optic canal was drilled off, and the falciform ligament was opened to decompress the optic nerve. The tumor was disconnected from the anterior clinoid region (the anterior clinoid process was eroded by the tumor) and reflected off the wall of the lateral CS. Tumor was adherent to the V2 fascicles (the lateral CS wall was resected in the first surgery) and was sharply dissected off. Gross total resection was achieved. The pathology was consistent with meningioma, WHO grade I. The patient had an unremarkable postoperative course without any new neurological deficits.The link to the video can be found at: https://youtu.be/KVBVw_86JqM.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii367-iii367
Author(s):  
Nongnuch Sirachainan ◽  
Attaporn Boongerd ◽  
Samart Pakakasama ◽  
Usanarat Anurathapan ◽  
Ake Hansasuta ◽  
...  

Abstract INTRODUCTION Low grade glioma (LGG) is the most common central nervous system (CNS) tumor in children accounted for 30–50%. Regarding benign characteristic of disease, surgical management remains the mainstay of treatment. However, surgical approach is limited in some conditions such as location at brainstem or infiltrative tumor. Chemotherapy and radiation treatments have been included in order to control tumor progression. The 5-years survival rate is approach 90% especially in patients who receive complete resection. However, the outcome of children with LGG in low to middle income is limited. Therefore, the aim of the study was to determine long-term outcome of children with newly diagnosed LGG. METHODS A retrospective study enrolled children aged <18 years who were newly diagnosed LGG during January 2006- December 2019. Diagnosis of LGG was confirmed by histological findings of grade I and II according to WHO criteria. RESULTS A total of 40 patients, female to male ratio was 1:1.35 and mean (SD) for age was 6.7 (4.0) years. The most common location was optic chiasmatic pathway (42.5%), followed by suprasellar region (25.0%). Sixty percent of patients received at least partial tumor removal. Chemotherapy and radiation had been used in 70% and 10.0% respectively. The 10-year progression free survival was 74.1±11.4% and overall survival was 96.2±3.8%. SUMMARY: Treatment of Pediatric LGG mainly required surgical management, however, chemotherapy and radiation had been used in progressive disease. The outcome was excellent.


2009 ◽  
Vol 3 (6) ◽  
pp. 461-466 ◽  
Author(s):  
Mostafa El Khashab ◽  
Lynn Gargan ◽  
Linda Margraf ◽  
Korgun Koral ◽  
Farideh Nejat ◽  
...  

Object Few reports describe the outcome and prognostic factors for children with gangliogliomas. The objective of this report was to describe the progression-free survival (PFS) for children with low-grade gangliogliomas and identify risk factors for tumor progression. Methods A retrospective study was performed in children with low-grade gangliogliomas who were evaluated and treated in the neuro-oncology department between 1986 and 2006 to determine risk factors for subsequent tumor progression. Results A total of 38 children with newly diagnosed gangliogliomas were included in this report. Thirty-four children were treated with surgery alone, 3 with subtotal resection and radiation therapy, and 1 with subtotal resection and chemotherapy. The follow-up ranged from 4 months to 15.8 years (mean 5.7 ± 4.2 years [± SD]). Seven children have experienced tumor progression, and 1 child died after his tumor subsequently underwent malignant transformation. The 5-year PFS was calculated to be 81.2% using Kaplan-Meier survival analysis. Initial presentation with seizures (p = 0.004), tumor location in the cerebral hemisphere (p = 0.020), and complete tumor resection (p = 0.035) were associated with prolonged PFS. Further analysis of the above significant variables by a Cox regression model identified initial presentation with seizures as being associated with prolonged PFS (p = 0.028). Conclusions The PFS and overall survival of children with gangliogliomas are good. Tumors located in the cerebral hemispheres, the achievement of total resection, and seizures at presentation were associated with prolonged PFS. Cox regression analysis identified presenting symptoms including seizures as significant predictive factors of PFS. Prospective studies with larger numbers of children are needed to define the significant factors of tumor progression.


2013 ◽  
Vol 71 (1) ◽  
pp. 31-34 ◽  
Author(s):  
André de Macedo Bianco ◽  
Flavio Key Miura ◽  
Carlos Clara ◽  
Jose Reynaldo W. Almeida ◽  
Clemar Correa da Silva ◽  
...  

A retrospective study of 81 patients with low-grade astrocytoma (LGA) comparing the efficacy of aggressive versus less aggressive surgery in eloquent and non-eloquent brain areas was conducted. Extent of surgical resection was analyzed to assess overall survival (OS) and progression- free survival (PFS). Degree of tumor resection was classified as gross total resection (GTR), subtotal resection (STR) or biopsy. GTR, STR and biopsy in patients with tumors in non-eloquent areas were performed in 31, 48 and 21% subjects, whereas in patients with tumors in eloquent areas resections were 22.5, 35 and 42.5%. Overall survival was 4.7 and 1.9 years in patients with tumors in non-eloquent brain areas submitted to GTR/STR and biopsy (p=0.013), whereas overall survival among patients with tumors in eloquent area was 4.5 and 2.1 years (p=0.33). Improved outcome for adult patients with LGA is predicted by more aggressive surgery in both eloquent and non-eloquent brain areas.


2018 ◽  
Vol 6 (4) ◽  
pp. 85 ◽  
Author(s):  
Ugo Testa ◽  
Germana Castelli ◽  
Elvira Pelosi

Brain tumors are highly heterogeneous and have been classified by the World Health Organization in various histological and molecular subtypes. Gliomas have been classified as ranging from low-grade astrocytomas and oligodendrogliomas to high-grade astrocytomas or glioblastomas. These tumors are characterized by a peculiar pattern of genetic alterations. Pediatric high-grade gliomas are histologically indistinguishable from adult glioblastomas, but they are considered distinct from adult glioblastomas because they possess a different spectrum of driver mutations (genes encoding histones H3.3 and H3.1). Medulloblastomas, the most frequent pediatric brain tumors, are considered to be of embryonic derivation and are currently subdivided into distinct subgroups depending on histological features and genetic profiling. There is emerging evidence that brain tumors are maintained by a special neural or glial stem cell-like population that self-renews and gives rise to differentiated progeny. In many instances, the prognosis of the majority of brain tumors remains negative and there is hope that the new acquisition of information on the molecular and cellular bases of these tumors will be translated in the development of new, more active treatments.


2018 ◽  
Vol 79 (S 05) ◽  
pp. S399-S401
Author(s):  
Sima Sayyahmelli ◽  
Adi Ahmetspahic ◽  
Mustafa Baskaya

Meningiomas are the second most common neoplasm in the cerebellopontine angle (CPA), and are challenging lesions to treat surgically. With significant refinements in surgical techniques, operative morbidity, and mortality have been substantially reduced. Total or near-total surgical resection can be accomplished in the majority of cases via appropriately selected approaches, and with acceptable morbidity. In this video, we present a 51-year-old woman, who had a 2-year history of vertigo with symptoms that progressed over time. She presented with blurry vision, sensorineural hearing loss, tinnitus, left-sided facial numbness, and double vision. Magnetic resonance imaging (MRI) showed a left-sided homogeneously enhancing mass at CPA with a supratentorial extension. MRI appearance was consistent with a CPA meningioma with supratentorial extension. The patient underwent surgical resection via a retrosigmoid approach. Suprameatal drilling and tentorial sectioning were necessary to achieve gross total resection. The surgery and postoperative course were uneventful. The histopathology was a WHO (world health organization) grade I meningioma. MRI showed gross total resection of the tumor. After a 1.5-year follow-up, the patient is continuing to do well with no residual or recurrent disease. In this video, microsurgical techniques and important steps for the resection of this challenging meningioma of the cerebellopontine angle are demonstrated.The link to the video can be found at: https://youtu.be/CDto52GxrG4.


2018 ◽  
Vol 6 (4) ◽  
pp. 249-258 ◽  
Author(s):  
Timothy J Brown ◽  
Daniela A Bota ◽  
Martin J van Den Bent ◽  
Paul D Brown ◽  
Elizabeth Maher ◽  
...  

Abstract Background Optimum management of low-grade gliomas remains controversial, and widespread practice variation exists. This evidence-based meta-analysis evaluates the association of extent of resection, radiation, and chemotherapy with mortality and progression-free survival at 2, 5, and 10 years in patients with low-grade glioma. Methods A quantitative systematic review was performed. Inclusion criteria included controlled trials of newly diagnosed low-grade (World Health Organization Grades I and II) gliomas in adults. Eligible studies were identified, assigned a level of evidence for every endpoint considered, and analyzed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The relative risk of mortality and of progression at 2, 5, and 10 years was calculated for patients undergoing resection (gross total, subtotal, or biopsy), radiation, or chemotherapy. Results Gross total resection was significantly associated with decreased mortality and likelihood of progression at all time points compared to subtotal resection. Early radiation was not associated with decreased mortality; however, progression-free survival was better at 5 years compared to patients receiving delayed or no radiation. Chemotherapy was associated with decreased mortality at 5 and 10 years in the high-quality literature. Progression-free survival was better at 5 and 10 years compared to patients who did not receive chemotherapy. In patients with isocitrate dehydrogenase 1 gene (IDH1) R132H mutations receiving chemotherapy, progression-free survival was better at 2 and 5 years than in patients with IDH1 wild-type gliomas. Conclusions Results from this review, the first to quantify differences in outcome associated with surgery, radiation, and chemotherapy in patients with low-grade gliomas, can be used to inform evidence-based management and future clinical trials.


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