scholarly journals RARE-10. PRIMARY INTRACRANIAL EWING SARCOMA IN A 12 MONTH OLD MALE

2021 ◽  
Vol 23 (Supplement_1) ◽  
pp. i42-i43
Author(s):  
Clay Hoerig ◽  
Aaron Goldberg ◽  
Jody Pathare ◽  
Tuan Dao ◽  
Ali Nael Amzajerdi ◽  
...  
Keyword(s):  
Ewing Sarcoma ◽  
Pediatric Brain Tumor ◽  
Distant Metastases ◽  
Residual Tumor ◽  
Axial Skeleton ◽  
Rare Type ◽  
Beam Radiation ◽  
Pathological Evaluation ◽  
The Right ◽  
Round Blue Cell Tumor

Abstract Background Ewing sarcoma (EWS) is a rare type of pediatric bone and soft tissue tumor that accounts for approximately 1% of all pediatric malignancies. It most commonly occurs in the long bones or axial skeleton, and rarely includes extraosseous sites or intracranial involvement. Reports of primary intracranial EWS are minimal. Pediatric intracranial EWS is even more rare with less than 15 cases reported. Case Description We describe the case of primary intracranial EWS in a 12 month old male. The patient’s initial MRI showed a large heterogeneous supratentorial cystic and solid mass centered in the right parietal region measuring 9.1cm x10.3cm x 7.6cm. No distant metastases were detected. The patient underwent surgical resection and pathology was consistent with a small round blue cell tumor. Further pathological evaluation revealed presence of EWSR1-FLI1 fusion and was negative for CD99, GFAP, synaptophysin, Olig2, desmin, and CAM5.2. Stains for INI1 and BRG1 were retained. The patient was treated with adjuvant chemotherapy and focal proton beam radiation (as per AEWS0031). Conclusion Primary intracranial Ewing Sarcoma is a rare pediatric brain tumor and, to our knowledge, this would be the youngest reported case to date. This case demonstrates the successful application of a sarcoma-based regimen to a primary intracranial EWS tumor with no evidence of residual tumor on MRI at 8 months into treatment. Future studies should be directed at understanding the biology of these rare tumors and optimizing treatment approaches.

scholarly journals QOL-09. WHOLE-BRAIN WHITE MATTER NETWORK CONNECTIVITY IS DISRUPTED BY PEDIATRIC BRAIN TUMOR TREATMENT

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii432-iii432
Author(s):  
Adeoye Oyefiade ◽  
Kiran Beera ◽  
Iska Moxon-Emre ◽  
Jovanka Skocic ◽  
Ute Bartels ◽  
...  
Keyword(s):  
White Matter ◽  
Pediatric Brain Tumor ◽  
Pediatric Brain Tumors ◽  
Magnetic Resonance Images ◽  
Long Term Effects ◽  
Brain White Matter ◽  
The Right ◽  
Pediatric Brain ◽  
Betweeness Centrality

Abstract INTRODUCTION Treatments for pediatric brain tumors (PBT) are neurotoxic and lead to long-term deficits that are driven by the perturbation of underlying white matter (WM). It is unclear if and how treatment may impair WM connectivity across the entire brain. METHODS Magnetic resonance images from 41 PBT survivors (mean age: 13.19 years, 53% M) and 41 typically developing (TD) children (mean age: 13.32 years, 51% M) were analyzed. Image reconstruction, segmentation, and node parcellation were completed in FreeSurfer. DTI maps and probabilistic streamline generation were completed in MRtrix3. Connectivity matrices were based on the number of streamlines connecting two nodes and the mean DTI (FA) index across streamlines. We used graph theoretical analyses to define structural differences between groups, and random forest (RF) analyses to identify hubs that reliably classify PBT and TD children. RESULTS For survivors treated with radiation, betweeness centrality was greater in the left insular (p < 0.000) but smaller in the right pallidum (p < 0.05). For survivors treated without radiation (surgery-only), betweeness centrality was smaller in the right interparietal sulcus (p < 0.05). RF analyses showed that differences in WM connectivity from the right pallidum to other parts of the brain reliably classified PBT survivors from TD children (classification accuracy = 77%). CONCLUSIONS The left insular, right pallidum, and right inter-parietal sulcus are structurally perturbed hubs in PBT survivors. WM connectivity from the right pallidum is vulnerable to the long-term effects of treatment for PBT.

scholarly journals LINC-06. OBSERVATION ONLY IN A PATIENT WITH SUSPECTED LOW GRADE GLIOMA. SHOULD NEUROSURGERY ALWAYS BE THE FIRST STEP IN LOW AND MIDDLE INCOME COUNTRIES?

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii379-iii379
Author(s):  
Carlos Leal - Cavazos ◽  
Jose Arenas-Ruiz ◽  
Oscar Vidal-Gutierrez
Keyword(s):  
Brain Mri ◽  
Internal Capsule ◽  
Pediatric Brain Tumor ◽  
Clinical History ◽  
Low Grade ◽  
Large Tumor ◽  
Middle Income ◽  
Dismal Prognosis ◽  
Close Observation ◽  
The Right

Abstract BACKGROUND Low grade gliomas (LGGs) are the most frequent pediatric brain tumor and they comprise a variety of histologies. Complete surgery is curative but sometimes its location makes it difficult. Recent publications highlight the excellent long-term outcomes of patients with LGGs with complete and incomplete resected tumors. Current strategies are focused on reducing risks of treatment related sequelae. METHOD We describe a patient with a suspected LGG managed by close observation. We describe the case of a 6 year old female with 5 months history of focal onset seizures. During this time a brain MRI was requested and tumor was evidenced. After “tumor diagnosis” was made family visited a handful of private neurosurgeons with a uniformly dismal prognosis and high risk morbidity from procedures offered. When first seen at our Hospital, the clinical history seemed compatible with a LGG and seizures well controlled with antiepileptic drugs. Neurological examination was completely normal. MRI showed a large tumor (7x5x5 cm) hypointense on T1, hyperintense on T2, without contrast enhancement, involving the right temporal lobe white matter, insula, internal capsule, hipoccampus, thalamus and mesencephalus with middle cerebral artery encasement. Interval imaging was proposed and after 4.5 years since diagnosis the tumor has been stable and patient clinically excellent. CONCLUSION Overall survival in pediatric LGGs is excellent and risk of sequelae should always be part of multidisciplinary team considerations. In centers with significant neurosurgical morbidity, biopsy of large tumors that are compatible with LGG may not be required in selected cases.

Rapid Deterioration of Latent HBV Hepatitis during Cushing Disease and Posttraumatic Stress Disorder after Earthquake

2017 ◽  
Vol 78 (04) ◽  
pp. 407-411 ◽  
Author(s):  
Ryosuke Tashiro ◽  
Teiji Tominaga ◽  
Yoshikazu Ogawa
Keyword(s):  
Hepatic Dysfunction ◽  
Residual Tumor ◽  
Optic Chiasm ◽  
Japanese Woman ◽  
Hbv Reactivation ◽  
Cushing Disease ◽  
Old Japanese ◽  
Threatening Condition ◽  
The Right ◽  
Subtotal Removal

AbstractReactivation of the hepatitis B virus (HBV) is a risk in the 350 million HBV carriers worldwide. HBV reactivation may cause hepatocellular carcinoma, cirrhosis, and fulminant hepatitis, and HBV reactivation accompanied with malignant tumor and/or chemotherapy is a critical problem for patients with chronic HBV infection. Multiple risk factors causing an immunosuppressive state can also induce HBV reactivation.We present a case of HBV reactivation during an immunosuppressive state caused by Cushing disease and physical and psychological stress after a disaster. A 47-year-old Japanese woman was an inactive HBV carrier until the Great East Japan Earthquake occurred and follow-up was discontinued. One year after the earthquake she had intractable hypertension, and her visual acuity gradually worsened. Head magnetic resonance imaging showed a sellar tumor compressing the optic chiasm, and hepatic dysfunction with HBV reactivation was identified. Endocrinologic examination established the diagnosis as Cushing disease. After normalization of hepatic dysfunction with antiviral therapy, transsphenoidal tumor removal was performed that resulted in subtotal removal except the right cavernous portion. Steroid hormone supplementation was discontinued after 3 days of administration, and gamma knife therapy was performed for the residual tumor. Eighteen months after the operation, adrenocorticotropic hormone and cortisol values returned to normal. The patient has been free from tumor regrowth and HBV reactivation throughout the postoperative course.Accomplishment of normalization with intrinsic steroid value with minimization of steroid supplementation should be established. Precise operative procedures and careful treatment planning are essential to avoid HBV reactivation in patients with this threatening condition.

Intracranial Peripheral Primitive Neuroectodermal Tumors of the Cavernous Sinus: A Diagnostic Peculiarity

2005 ◽  
Vol 129 (1) ◽  
pp. e11-e15 ◽  
Author(s):  
Muhammad Idrees ◽  
Chirag Gandhi ◽  
Simone Betchen ◽  
James Strauchen ◽  
Wesley King ◽  
...  
Keyword(s):  
Cavernous Sinus ◽  
Ewing Sarcoma ◽  
Moderate Amount ◽  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors ◽  
Male Preponderance ◽  
Poorly Differentiated ◽  
Immunohistochemical Techniques ◽  
The Right ◽  
Peripheral Primitive Neuroectodermal Tumors

Abstract Peripheral primitive neuroectodermal tumors (pPNETs) are aggressive, poorly differentiated neoplasms that occur in children and young adults. These tumors are associated with a peak incidence in the second decade and a slight male preponderance. Recently, Ewing sarcoma and pPNET tumors have been proven to carry identical translocations, the most common being t(11;22)(q24;q12). Intracranial Ewing sarcoma/pPNETs have rarely been described in the literature. We studied a case of intracranial pPNET arising in the right cavernous sinus of a 46-year-old man. On imaging, the tumor had both sellar and suprasellar components and was centered within the right parasellar region. Histologically, the tumor was composed of intermediate to large cells with round to oval hyperchromatic nuclei with distinct nucleoli. The cells contained a moderate amount of slightly basophilic cytoplasm. The tumor was markedly fibrotic and had collagen bands surrounding both individual and groups of cells. A large immunohistochemical panel was positive only for CD99 and vimentin. Fluorescence in situ hybridization did not show translocations associated with Ewing sarcoma/pPNET. However, a small percentage of these tumors can be negative for this translocation. In these cases, histology and immunohistochemical techniques in the absence of an alternative diagnosis are the only tools available to establish the diagnosis.

scholarly journals Orbital metastasis as primary clinical manifestation of thyroid carcinoma: case report and literature review

2008 ◽  
Vol 52 (9) ◽  
pp. 1497-1500 ◽  
Author(s):  
Francisco Dário Rocha Filho ◽  
Gabrielle Gurgel Lima ◽  
Francisco V. de Almeida Ferreira ◽  
Michelle Gurgel Lima ◽  
Miguel N. Hissa
Keyword(s):  
Thyroid Carcinoma ◽  
Clinical Manifestation ◽  
Palliative Chemotherapy ◽  
Distant Metastases ◽  
Histopathological Analysis ◽  
Free T4 ◽  
Follicular Variant ◽  
Orbital Metastasis ◽  
Uncommon Case ◽  
The Right

Capillary thyroid carcinoma (PTC) is the most common neoplasm of thyroid. It usually grows slowly and is clinically indolent, although rare, its aggressive forms with local invasion or distant metastases can occur. Metastatic thyroid carcinoma rarely involves the orbit. We reported an uncommon case of orbital metastasis of PTC. A 66-years-old woman presented proptosis of the right eye. The biopsy of the tumor in orbit revealed metastatic thyroid carcinoma. The ultrasensitive TSH level was 1,34 mUI/L and free T4 level was 1,65 ng/dL. A total thyroidectomy was performed and histopathological analysis of the nodule revealed follicular variant of papillary thyroid carcinoma. Currently, the patient has been receiving palliative chemotherapy with Clodronate Disodium. The importance of the case is due to its unusual presentation, which emerged as a primary clinical manifestation. Although rare, thyroid carcinoma should be suspected in orbit metastasis.

scholarly journals Splenic undifferentiated high grade pleomorphic sarcoma of a small size with fatal tumor rupture

1970 ◽  
Vol 1 (2) ◽  
pp. 151-153 ◽  
Author(s):  
BM Amatya ◽  
M Sawabe ◽  
T Arai ◽  
T Kumakawa ◽  
K Takubo ◽  
...  
Keyword(s):  
Past History ◽  
Distant Metastases ◽  
Tumor Rupture ◽  
Retroperitoneal Hemorrhage ◽  
Undifferentiated Pleomorphic Sarcoma ◽  
Bone Marrow Histology ◽  
Pleomorphic Sarcoma ◽  
Fatal Bleeding ◽  
History Of ◽  
The Right

(The order of authors on this article was changed on 09/01/2012.)Primary undifferentiated pleomorphic sarcoma of the spleen is a rare and highly aggressive neoplasm that usually presents with splenomegaly, constitutional symptoms and frequent distant metastases. We report a case of 77-year old male patient with a past history of dissecting aortic aneurysm that developed acute hemorrhagic shock. Aneurysmal rupture was clinically suspected, but the postmortem examination revealed a 25 mm-sized tumor in an atrophic spleen weighing 65 gram with massive retroperitoneal bleeding. Metastases were found in the right renal hilus, the right adrenal gland and femoral bone marrow. Histology of the tumor showed undifferentiated pleomorphic sarcoma. Tumor rupture with fatal bleeding and systemic metastases had occurred despite the small size of the tumor. Tumor size is not a reliable predictor of systemic metastasis or tumor rupture for splenic undifferentiated pleomorphic sarcoma. Keywords: Undifferentiated pleomorphic sarcoma; Malignant fibrous histiocytoma; Splenic neoplasms; Retroperitoneal hemorrhage DOI: http://dx.doi.org/10.3126/jpn.v1i2.5411 JPN 2011; 1(2): 151-153

Foix-Chavany-Marie syndrome caused by a disconnection between the right pars opercularis of the inferior frontal gyrus and the supplementary motor area

2012 ◽  
Vol 117 (5) ◽  
pp. 844-850 ◽  
Author(s):  
Juan Martino ◽  
Enrique Marco de Lucas ◽  
Francisco Javier Ibáñez-Plágaro ◽  
José Manuel Valle-Folgueral ◽  
Alfonso Vázquez-Barquero
Keyword(s):  
Supplementary Motor Area ◽  
Diffusion Tensor ◽  
Anterior Part ◽  
Inferior Frontal Gyrus ◽  
Motor Area ◽  
Lower Limbs ◽  
Rare Type ◽  
Orofacial Movement ◽  
Pars Opercularis ◽  
The Right

Foix-Chavany-Marie syndrome (FCMS) is a rare type of suprabulbar palsy characterized by an automaticvoluntary dissociation of the orofacial musculature. Here, the authors report an original case of FCMS that occurred intraoperatively while resecting the pars opercularis of the inferior frontal gyrus. This 25-year-old right-handed man with an incidentally diagnosed right frontotemporoinsular tumor underwent surgery using an asleep-awake-asleep technique with direct cortical and subcortical electrical stimulation and a transopercular approach to the insula. While resecting the anterior part of the pars opercularis the patient suffered sudden anarthria and bilateral facial weakness. He was unable to speak or show his teeth on command, but he was able to voluntarily move his upper and lower limbs. This syndrome lasted for 8 days. Postoperative diffusion tensor imaging tractography revealed that connections of the pars opercularis of the right inferior frontal gyrus with the frontal aslant tract (FAT) and arcuate fasciculus (AF) were damaged. This case supplies evidence for localizing the structural substrate of FCMS. It was possible, for the first time in the literature, to accurately correlate the occurrence of FCMS to the resection of connections between the FAT and AF, and the right pars opercularis of the inferior frontal gyrus. The FAT has been recently described, but it may be an important connection to mediate supplementary motor area control of orofacial movement. The present case also contributes to our knowledge of complication avoidance in operculoinsular surgery. A transopercular approach to insuloopercular gliomas can generate FCMS, especially in cases of previous contralateral lesions. The prognosis is favorable, but the patient should be informed of this particular hazard, and the surgeon should anticipate the surgical strategy in case the syndrome occurs intraoperatively in an awake patient.

scholarly journals Cardiac Lipoma: An Uncharacteristically Large Intra-Atrial Mass Causing Symptoms

2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Fahad Syed Naseerullah ◽  
Hemangkumar Javaiya ◽  
Avinash Murthy
Keyword(s):  
Right Atrial ◽  
Rare Type ◽  
Atrial Mass ◽  
Persistent Symptoms ◽  
Coronary Syndrome ◽  
Cardiac Tumours ◽  
Large Size ◽  
Right Atrial Mass ◽  
Cardiac Lipoma ◽  
The Right

Primary tumours of the heart are often encountered in clinical practice. Different autopsy series estimate the incidence to be anywhere from 0.001% to 0.19%. Cardiac lipoma is a rare type of tumour of the heart and pericardium. It comprises approximately 10–19% of all cardiac tumours. We present a case of a large cardiac lipoma in a fifty-year-old female. She presented with sharp chest pains, palpitations, and dizziness. Acute coronary syndrome was ruled out. A transthoracic echocardiogram showed an abnormal, large, fixed right atrial mass. The mass was noted to be occupying most of the right atrium. It was excised due to its large size and persistent symptoms. On pathophysiology, the mass was definitively diagnosed to be an 80 mm × 70 mm cardiac lipoma. Postoperatively, the patient did well with resolution of her symptoms. This case provides evidence that even large, invasive, symptomatic cardiac lipomas can be successfully resected with good outcomes.

Hemangiopericytoma of the Sphenoid Sinus

1981 ◽  
Vol 89 (5) ◽  
pp. 713-716 ◽  
Author(s):  
Charles P. Kimmelman
Keyword(s):  
Radiation Therapy ◽  
Sphenoid Sinus ◽  
Vascular Tumor ◽  
External Beam Radiation ◽  
Orbital Apex ◽  
Tumor Nodule ◽  
Beam Radiation ◽  
The Right ◽  
Sinus Ostium ◽  
Ethmoid Labyrinth

A 51-year-old woman complained of intermittent nasal obstruction and rhinorrhea. Sinus roentgenograms and polytomograms revealed a small mucocele of the right sphenoid sinus. There was no encroachment of the orbital apex or intracranial contents. The mucocele was marsupialized via an external ethmoidectomy, and the scant tissue present inside the sinus was examined histologically. The pathologic diagnosis was hemangiopericytoma. The small tumor had apparently originated at the sinus ostium, thus creating the mucocele. Several months postoperatively, a small, vascular tumor nodule was noted in the incision. No recurrence was evident intranasally. A course of 5,040 rads of external beam radiation therapy was given to the right ethmoid labyrinth and right sphenoid. Since completing her radiation therapy, there has been no recurrence. This case is of interest because the tumor occurred as a mucocele of the sphenoid sinus.

scholarly journals Prognostic value of subclassification (pT2 stage) of pathologically organ-confined prostate cancer: Confirmation of the changes introduced in the 8th edition of the American Joint Committee on Cancer (AJCC) staging system

2018 ◽  
Vol 90 (3) ◽  
pp. 191-194 ◽  
Author(s):  
Hugo Pontes Antunes ◽  
Belmiro Parada ◽  
João Carvalho ◽  
Miguel Eliseu ◽  
Roberto Jarimba ◽  
...  
Keyword(s):  
Prostate Cancer ◽  
Specific Antigen ◽  
Staging System ◽  
Distant Metastases ◽  
Grade Group ◽  
Pathological Evaluation ◽  
Ajcc Staging System ◽  
Oncological Results ◽  
Ajcc Staging ◽  
8Th Edition

Objective: The last edition of the AJCC staging system eliminated the pT2 subclassification of prostate cancer (PCa). Our objective was to evaluate the association of pT2 subclassification with the oncological results of patients with PCa who underwent radical prostatectomy (RP). Material and methods: We evaluated 367 patients who underwent RP between 2009 and 2016, with pT2 disease in the final pathological evaluation. We assessed differences in rates of biochemical recurrence (BCR), metastasis and mortality between T2 substages (pT2a/b vs pT2c). Results: Fifty-three (14.4%) patients presented pT2a/b disease and 314 (85.6%) pT2c disease. The mean follow-up time was 4.9 ± 2.6 years. Grade group scores (p = 0.1) and prostate specific antigen (PSA) (p = 0.2) did not differed between pT2 substages. The rate of BCR in pT2a/b and pT2c patients was 11.3% and 18.2%, respectively (p = 0.2). Five (9.4%) patients with pT2a/b and 45 (14.3%) with pT2c substage underwent salvage radiotherapy (p = 0.3). The rate of positive surgical margins did not differ between groups (p = 0.2). Seven (2.2%) patients with pT2c had lymph nodes or distant metastases. The overall survival was 92.5% and 93.6% in pT2a/b and pT2c, respectively (p = 0.2). Conclusion: Our results are in accordance with the changes introduced in the 8th edition of the AJCC staging system in which the pT2 subclassification was eliminated.

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