scholarly journals Pilocytic Midbrain Astrocytoma Presenting with Fresh Bleed after Twenty-one-years Survival Following First Surgery: A Unique Case of Longest Brainstem Glioma Survival

2016 ◽  
Vol 07 (S 01) ◽  
pp. S088-S090 ◽  
Author(s):  
Guru Dutta Satyarthee ◽  
M. D. Sudhan ◽  
V. S. Mehta

ABSTRACTBrainstem glioma usually carries a poor prognosis and prolonged survival is very infrequent. In a detailed Pubmed, Medline search for prolonged survival, authors could got a longest survival only up to seventeen years, reported by Umehara et al, who was subjected to gamma knife therapy and got symptomatic, MRI brain reveled large tumor growth during pregnancy necessitating emergency surgery and histopathological diagnosis was pilocytic astrocytoma. Authors report an interesting case of midbrain glioma diagnosed 21 years back, who underwent gross resection in the year 1993, histopathology was pilocytic astrocytoma, WHO grade I, and received gamma knife surgery for residual subsequently and he presented with sudden onset left sided hemiplegia on the current admission. The cranial MRI imaging revealed an infarct involving right hemi midbrain, contrast MRI brain revealed no residual glioma. To the best knowledge of authors such prolonged survival is not reported with a case of brainstem glioma survived twenty- one years with non residual tumor on the last imaging study represents first case of its kind in the western literature and probably developed hemiplegia due to bleed, highlighting bleed as delayed complication following gamma knife therapy for cranial tumors

2020 ◽  
Vol 11 ◽  
pp. 367
Author(s):  
Sarah Bin Abdulqader ◽  
Nasser Almujaiwel ◽  
Wafa Alshakweer ◽  
Gmaan Alzhrani

Background: Spheno-orbital meningiomas (SOMs) are often benign. The association of meningioma and systemic lupus erythematosus (SLE) is rarely discussed in the literature. Here, we report two patients with high-grade, SOMs with a prolonged history of SLE and review the literature. Case Description: The first case is a 52-year-old female patient with a 15-year history of SLE diagnosis who was referred to our center with a 1-year history of proptosis and excessive tearing of the left eye. This patient was operated for the left SOM with histopathological diagnosis of the World Health Organization (WHO) Grade III rhabdoid meningioma. The second case is a 36-year-old female patient with a 12-year history of SLE diagnosis who presented to our clinic with a 5-year-history of progressive right eye proptosis and occasional headaches. She was operated for the right SOM with histopathological diagnosis of the WHO Grade II chordoid meningioma. Conclusion: Rhabdoid and chordoid SOMs are uncommon and no previous report discussed their occurrence in patients with SLE. The association of high-grade meningiomas and SLE deserves further exploration.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii425-iii425
Author(s):  
Nobuyoshi Sasaki ◽  
Tomohiro Chiba ◽  
Kuniaki Saito ◽  
Keiichi Kobayashi ◽  
Yoshiaki Shiokawa ◽  
...  

Abstract Pilocytic astrocytomas rarely present with oligodendroglioma-like morphological features, which gives rise to a diagnostic challenge. In this report we present a case of pilocytic astrocytoma harboring the FGFR1 mutation, accompanied with a predominant oligodendroglioma-like component, thus initially diagnosed as oligodendroglioma. A 14-year-old female presented with syncope and simple partial seizure involving her right upper limb. Contrast-enhanced MRI revealed an enhancing lesion with substantial cystic portion and perifocal edema in the left parietal lobe. Open surgery was performed and a gross total resection of the tumor was achieved. On initial histopathological diagnosis, tumor cells with monotonous round nuclei and perinuclear halo predominated with branching capillaries, which were strongly suggestive for oligodendroglioma. Immunohistochemically, IDH1 R132H was negative, and Ki-67 index was around 5%. The patient was thus initially diagnosed as oligodendroglioma, WHO grade II, based on the 2007 WHO classification criteria. However, histopathological re-review revealed a minor astrocytic component with Rosenthal fibers and rare eosinophilic granular bodies, thus the diagnosis was changed as pilocytic astrocytoma. FGFR1 K654E mutation was confirmed by Sanger sequencing. Although she postoperatively developed mild sensory disturbance in her right hands, finger agnosia, and left-right disorientation, her symptoms had gradually improved, and she was discharged on day 17 with a Karnofsky performance status (KPS) of 90 and no cognitive decline. Without any adjuvant therapies, she has remained recurrence-free for 85 months. While the diagnosis of pilocytic astrocytoma with predominant oligodendroglioma-like component can be challenging, analysis of IDH1 and FGFR1 mutations can be beneficial in certain cases.


2018 ◽  
Vol 129 (3) ◽  
pp. 593-597 ◽  
Author(s):  
Shuhei Morita ◽  
Masayuki Nitta ◽  
Yoshihiro Muragaki ◽  
Takashi Komori ◽  
Kenta Masui ◽  
...  

In this report, the authors present the first case of adult brainstem pilocytic astrocytoma (PA) with the H3 K27M mutation. A 53-year-old man was incidentally found to have a 2.5-cm partially enhanced tumor in the tectum on MRI. The enhancement in the lesion increased over 3 years, and gross-total removal was performed via the occipital transtentorial approach. The resected tissue indicated PA, WHO Grade I, and genetic analysis revealed the H3 K27M mutation. However, although the radiological, surgical, and pathological findings all corresponded to PA, this entity can easily be misdiagnosed as diffuse midline glioma with the H3 K27M mutation, which is classified as a WHO Grade IV tumor according to the updated classification. This case highlights the phenotypic spectrum of PA, as well as the biology of the H3 K27M–mutated gliomas, and may prove to be an exception to the rule that diffuse midline gliomas with the H3 K27M mutation behave in an aggressive manner. Based on the findings of this case, the authors conclude that, in addition to detecting the existence of the H3 K27M mutation, an integrated approach in which a combination of clinical, pathological, and genetic information is used should be applied for accurate diagnosis and determination of the appropriate treatment for diffuse midline gliomas.


2016 ◽  
Vol 17 (4) ◽  
pp. 446-452 ◽  
Author(s):  
Mitchell P. Wilson ◽  
Edward S. Johnson ◽  
Cynthia Hawkins ◽  
Kerry Atkins ◽  
Wael Alshaya ◽  
...  

Acute hemorrhagic presentation in pilocytic astrocytomas (PAs) has become increasingly recognized. This type of presentation poses a clinically emergent situation in those hemorrhages arising in PAs of the cerebellum, the most frequent site, because of the limited capacity of the posterior fossa to compensate for mass effect, predisposing to rapid neurological deterioration. As examples, we describe two cases of fatal hemorrhagic cerebellar PAs: one of a child with a slowly growing stereotypical WHO Grade I PA with a 1-year period of symptomatology that preceded a rapid clinical deterioration, and another of an asymptomatic child having a PA variant, presenting with progressive obtundation following a presumed Valsalva event. These two scenarios parallel previous reports in the literature of either a setting of progressive expression of cerebellar dysfunction and transient episodes of raised intracranial pressure (ICP), or abrupt onset of features of increased ICP in a previously well child. The literature is further reviewed for a current understanding of the factors that predispose, initiate and propagate bleeding, with specific reference to the role of vascular endothelial growth factor and other angiogenic agents in the genesis and stability of the vasculature in PAs. In this context, we propose that obliterative vascular mural hyalinization with associated altered flow dynamics and microaneurysm formation was the pathogenesis of the hemorrhage in our first case. In the second case, large tumor size, increased growth rate, looseness of the background myxoid matrix, and thinness of the tumor blood vessels with calcospherite deposition predisposed to vascular leakage and bleeding concurrent with sudden increases in intravascular hydrostatic pressure. In that cerebellar PAs are common, this report underscores the importance of considering in the differential diagnosis the possibility of a spontaneous hemorrhage in a posterior fossa PA in a child presenting with a sudden neurological ictus and raised ICP.


2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 262-265
Author(s):  
C. P. Yu ◽  
Joel Y. C. Cheung ◽  
Josie F. K. Chan ◽  
Samuel C. L. Leung ◽  
Robert T. K. Ho

Object. The authors analyzed the factors involved in determining prolonged survival (≥ 24 months) in patients with brain metastases treated by gamma knife surgery (GKS). Methods. Between 1995 and 2003, a total of 116 patients underwent 167 GKS procedures for brain metastases. There was no special case selection. Smaller and larger lesions were treated with different protocols. The mean patient age was 56.9 years, the mean number of initial lesions was 3.15, and the mean lesion volume was 10.45 cm.3 The mean follow-up time was 9.2 months. The median patient survival was 8.68 months. One-, 2-, 3-, 4-, and 5-year actuarial survival rates were 31.8%, 19.8%, 14.6%, 7.7%, and 6.9%, respectively. Patient age, number of lesions at presentation, and lesion volume had no influence on patient survival. Twenty-three (19.8%) patients survived for 24 months or more. Certain factors were associated with increased survival time. These were stable primary disease (21 of 23 patients), a long latency between diagnosis of the primary tumor and the occurrence of brain metastases (mean 28.4 months, median 16 months), absence of third-organ involvement, and repeated local procedures. Ten patients underwent repeated GKS (mean 3.4 per patient). Seven patients required open surgery for local treatment failures (recurrence or radiation necrosis). Two patients had both. Fifteen patients underwent repeated procedures. Conclusions. Aggressive local therapy with GKS, repeated GKS, and GKS plus surgery can achieve increased survival in a subgroup of patients with stable primary disease, no third-organ involvement, and long primary-brain secondary intervals.


2021 ◽  
pp. 112067212110307
Author(s):  
Raquel María Moral ◽  
Carlos Monteagudo ◽  
Javier Muriel ◽  
Lucía Moreno ◽  
Ana María Peiró

Introduction: Conjunctival melanoma is extremely rare in children and has low rates of resolution. Definitive histopathological diagnosis based exclusively on microscopic findings is sometimes difficult. Thus, early diagnosis and adequate treatment are essential to improve clinical outcomes. Clinical case: We present the first case in which the fluorescent in situ hybridization (FISH) diagnostic technique was applied to a 10-year-old boy initially suspected of having amelanotic nevi in his right eye. Based on the 65% of tumor cells with 11q13 (CCND1) copy number gain and 33% with 6p25 (RREB1) gain as measured by the FISH analysis, and on supporting histopathological findings, the diagnosis of conjunctival melanoma could be made. Following a larger re-excision, adjuvant therapy with Mitomycin C (MMC), cryotherapy and an amniotic membrane graft, the patient has remained disease-free during 9 years of long-term follow-up. Case discussion: Every ophthalmologist should remember to consider and not forget the possibility of using FISH analyses during the differential diagnosis of any suspicious conjunctival lesions. Genetic techniques, such as FISH, have led to great advances in the classification of ambiguous lesions. Evidence-based guidelines for diagnosing conjunctival melanoma in the pediatric population are needed to determine the most appropriate strategy for this age group.


2015 ◽  
Vol 61 (4) ◽  
pp. 275-278
Author(s):  
C. Tuleasca ◽  
L. Negretti ◽  
V. Magaddino ◽  
P. Maeder ◽  
B. Lhermitte ◽  
...  

2021 ◽  
Vol 8 (3) ◽  
pp. 395-411
Author(s):  
Dewa Kartika ◽  
Baskoro Nurdopo

Pendahuluan Butterfly Glioma adalah high grade astrocytoma, biasanya glioblastoma (WHO grade IV), yang melintasi garis tengah melalui corpus callosum. Komissura white matter lainnya kadang juga terlibat. Istilah kupu-kupu mengacu pada ekstensi yang melewati garis tengah seperti sayap. Butterfly Glioma paling sering terjadi di lobus frontal, melintasi garis tengah melalui genu corpus callosum, namun butterfly glioma posterior kadang juga ditemui. Laporan kasus Seorang pasien laki-laki usia 24 tahun dengan keluhan utama 9 bulan, yang lalu. Penglihatan kabur, konsentrasi menurun. Kejang(-). Kemudian 3 bulan yang lalu mata tidak bisa melihat. Dan 1 bulan yang lalu tubuh lemas susah digerakkan Pemeriksaan patologi anatomi menunjukkan Pylocytic Astrocytoma. Pemeriksaan CT scan kepala menunjukkan  Massa solid inhomogen intraxial ( ukuran ± AP 7,6 x 8,9 x CC 6,2 cm ) disertai kalsifikasi di dalamnya pada corpus callosum yang tampak cross mid line ( sisi kiri lebih dominan ) membentuk gambaran butterfly sign dengan perifocal edema à curiga gambaran glioblastoma multiformis.   Pembahasan Hasil pemeriksaan anamnesis dan pemeriksaan fisik pasien ini menunjukkan kecurigaan adanya SOL. Pemeriksaan CT scan kepala menunjukkan  Massa solid inhomogen intraxial disertai kalsifikasi di dalamnya pada corpus callosum yang tampak cross mid line ( sisi kiri lebih dominan ) membentuk gambaran butterfly sign dengan perifocal edema à curiga gambaran glioblastoma multiformis. Dari PA didapatkan hasil Pilocytic astrocytoma. Sedangkan gambaran radiologi Pilocytic astrocytoma berupa lesi kistik dengan nodul mural yang enhanced. Kasus ini secara radiologis lebih mengarah ke Butterfly Glioblastoma dengan adanya lesi yang melewati garis tengah, serta ada komponen nekrotik dan perdarahan.. Modalitas imejing pilihan yang dapat dilakukan pada kasus Butterfly Glioblastoma adalah CT scan dan MRI. Kesimpulan Kasus ini secara radiologis lebih mengarah ke Butterfly Glioblastoma dengan adanya lesi yang melewati garis tengah, serta ada komponen nekrotik dan perdarahan. Dan pemeriksaan radiologis yang dapat digunakan pada Butterfly Glioblastoma adalah CT scan dan MRI.


Author(s):  
Glória França ◽  
◽  
Dáurea Sena ◽  
Juliana Pinheiro ◽  
Rodrigo Rodrigues ◽  
...  

A calcifying odontogenic cyst may be associated with odontogenic tumors, particularly odontomas. However, the association between calcifying odontogenic cysts and odontogenic cysts is rare. This study aims to report the first case of a calcifying odontogenic cyst associated with an antral pseudocyst. A male patient presented a tumor lesion in his right maxillary alveolar ridge with 6 months of evolution and painful symptoms . An excisional biopsy was performed, and a histopathological diagnosis of calcifying odontogenic cyst associated with an antral pseudocyst was issued. The treatment of choice was lesion enucleation and curettage. The patient has been under follow-up for about 3 years without lesion recurrence, which is typical indolent calcifying odontogenic cyst behavior.


2021 ◽  
Vol 8 (1) ◽  
pp. 10
Author(s):  
Jared F. Sweeney ◽  
Vaibhav Chumbalkar ◽  
Michael D. Staudt ◽  
Pouya Entezami ◽  
Jiang Qian ◽  
...  

Due to its rarity, a complete understanding of the clinical behavior, pathogenesis, and diagnostic definition of anaplastic pilocytic astrocytoma (APA) is currently lacking. The optimal clinical management and use of adjuvant therapies has yet to be defined. We present a 64 year-old-female with progressive headaches, dysarthria, and ataxia, who was found to have right cerebellar mass. A gross total resection was achieved through two staged operations. Pathology demonstrated focal areas of necrosis, tumor infiltration, and increased mitotic activity most consistent with APA. Adjuvant chemotherapy and stereotactic radiosurgery were administered. Approximately two years later, the patient presented with symptoms of cauda equina syndrome, and lumbar spine imaging demonstrated a large intradural mass at the conus medullaris with diffuse leptomeningeal enhancement. A biopsy was performed and was consistent with metastatic APA. APA may rarely progress to metastatic disease, most frequently involving the leptomeninges of the posterior fossa and cervical spine. This report represents the first case of metastases distal to the cervicomedullary junction.


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