scholarly journals Solitary bone plasmacytoma as posterior fossa cranial neoplasia, presentation of two clinical cases

2022 ◽  
Vol 13 ◽  
pp. 7
Author(s):  
Luis David Molina Andaluz ◽  
Josué Alejandro Cervantes Gonzalez ◽  
Zita Elizabeth Salazar Ramírez ◽  
Nelly Ramírez ◽  
Luis Guillermo Castellanos ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Clinical Presentation ◽  
The Other ◽  
Occipital Region ◽  
Cerebellar Syndrome ◽  
Total Resection ◽  
Plasma Cell Neoplasm ◽  
Cell Neoplasm ◽  
Solitary Bone Plasmacytoma

Background: Solitary bone plasmacytoma is a plasmatic cell dyscrasia; its presentation in the posterior fossa is very rare. Case Description: We present two cases, a 59-year-old male and a 50-year-old female, both with heterogeneous clinical presentation. One had symptoms compatible with endocranial hypertension, and the other presented with a hemispheric cerebellar syndrome and ipsilateral trigeminal neuralgia. They were both related to an intraosseous tumor of the occipital region near the torcula with large extension to the posterior fossa. The diagnosis of a plasma cell neoplasm arising from the diploe of the squamous portion of the occipital bone was confirmed with immunohistochemistry. Conclusion: The treatment for a cranial tumor that is suspected to be a solitary bone plasmacytoma requires a multidisciplinary team to diagnose, plan a total resection, and after surgery continue with the follow-up of the patient. Solitary bone plasmacytoma should be considered as a differential diagnosis for a tumor that produces cancellous bone widening without sclerotic borders.

Aggressive Intramedullary Melanotic Schwannoma: Case Report

Neurosurgery ◽  
2004 ◽  
Vol 55 (6) ◽  
pp. E1430-E1434 ◽  
Author(s):  
Carlo Santaguida ◽  
Abdulrahman J. Sabbagh ◽  
Marie-Christine Guiot ◽  
Rolando F. Del Maestro
Keyword(s):  
Clinical Presentation ◽  
Magnetic Resonance Imaging Scan ◽  
Resonance Imaging ◽  
Total Resection ◽  
Neck Stiffness ◽  
Neck Extension ◽  
Melanotic Schwannoma ◽  
Pathological Characteristics ◽  
Intramedullary Lesion

Abstract OBJECTIVE AND IMPORTANCE: Intramedullary melanotic schwannomas are very rare lesions; only four cases have been reported previously. We describe a patient with an intramedullary melanotic schwannoma that had a more aggressive course than those reported in the literature, and we review the theories regarding the cause of these lesions. CLINICAL PRESENTATION: A 35-year-old man presented with neck stiffness and paraesthesia extending down his right arm upon neck extension. A magnetic resonance imaging scan revealed an intramedullary lesion extending from C4 to C5. INTERVENTION: Gross total resection of the mass was performed, and pathological characteristics were consistent with a melanotic schwannoma. Two years after resection the tumor recurred, and the patient was treated with radiation therapy. The tumor progressed 2 years after radiotherapy, and at repeat resection, multiple pigmented foci were present on the surface of the spinal cord and dura consistent with metastatic seeding. CONCLUSION: In a patient with intramedullary melanotic schwannoma with an unusually aggressive course, careful follow-up may be essential.

scholarly journals Meningiomas of pineal region in children

2007 ◽  
Vol 65 (4a) ◽  
pp. 1000-1006 ◽  
Author(s):  
Hamilton Matushita ◽  
Fernando Campos Pinto ◽  
José Píndaro Pereira Plese
Keyword(s):  
Clinical Presentation ◽  
Complete Removal ◽  
Pineal Region ◽  
Total Resection ◽  
Long Term Follow Up ◽  
One Year ◽  
Velum Interpositum ◽  
Transtentorial Approach

Meningiomas are uncommon tumors in children and either more rarely encountered in the pineal region. We report two cases of meningioma of the pineal region in children. One of these cases was a five years-old girl and the other a one year-old boy. No specific clinical presentation or tomographic examinations findings was identified before treatment, suggestive of a diagnosis of menigioma. The clinical and laboratory features were very similar to the most common tumors of the pineal region. Prior to the surgery, the histology of these tumors was not suspected. Both patients underwent direct surgery and complete removal was achieved by a suboccipital transtentorial approach. The tumors originated from velum interpositum in both cases. At the follow up, one case presented with recurrence six years later, and she underwent a reoperation with total resection without morbidity. Long-term follow up presented no other recurrences.

scholarly journals Ependymomas in infancy: underlying genetic alterations, histological features, and clinical outcome

2020 ◽  
Vol 36 (11) ◽  
pp. 2693-2700
Author(s):  
Stephanie T. Jünger ◽  
Felipe Andreiuolo ◽  
Martin Mynarek ◽  
Evelyn Dörner ◽  
Anja zur Mühlen ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Genetic Alterations ◽  
Gross Total Resection ◽  
Older Children ◽  
Total Resection ◽  
Who Grade ◽  
Chromosome 1Q ◽  
Stable Genome ◽  
1Q Gain

Abstract Introduction Young age is an adverse prognostic factor in children with ependymomas. Treatment of these infants is challenging since beneficial therapeutic options are limited. As ependymomas are considered a biologically heterogeneous group, we aimed to characterize infant ependymomas with regard to their histological and genetic features. Materials and methods We analyzed 28 ependymomas occurring in children younger than 18 months at diagnosis enrolled into the HIT2000-E protocols with the aim to postpone irradiation until the age of 18 months if possible. All cases underwent neuropathological review, including immunohistochemical characterization. Genome-wide copy number alterations (CNA) were assessed by molecular inversion probe assays, and RELA and YAP1 fusions were detected by RT-PCR and sequencing. Results All infant ependymomas were anaplastic (WHO grade III). Twenty-one (75%) cases were located in the posterior fossa. Gross total resection was accomplished in 12 (57%) of these cases. All posterior fossa tumors showed loss of H3-K27me3 characteristic of PFA ependymomas. CNA analysis showed a stable genome in all cases with lack of chromosome 1q gain, an adverse prognostic marker in PFA ependymomas of older children. However, after a median follow-up of 5.4 years, 15 (71%) relapsed, and 9 (43%) died. Seven ependymomas (25%) occurred in the supratentorial region. Gross total resection could be achieved in only two of these cases. Four tumors carried C11orf95-RELA fusions, and two cases had typical YAP1-MAMLD1 fusions (one case was not analyzable). The RELA-fused cases did not display CDKN2A loss as an adverse indicator of prognosis in this disease entity. Although three infants (43%) with supratentorial ependymomas relapsed, all patients survived (median follow-up, 8.0 years). Conclusion Infant ependymomas seem to fall into three biological entities, with supratentorial tumors carrying RELA or YAP fusions and PFA posterior fossa ependymomas. The latter showed a poor outcome even though chromosome 1q gain was absent.

JUVENILE INTRADURAL CHORDOMA

Neurosurgery ◽  
2008 ◽  
Vol 62 (2) ◽  
pp. E525-E527 ◽  
Author(s):  
Steven W. Chang ◽  
Pankaj A. Gore ◽  
Peter Nakaji ◽  
Harold L. Rekate
Keyword(s):  
Clinical Presentation ◽  
Radiation Treatment ◽  
Complete Resection ◽  
Magnetic Resonance Imaging Scan ◽  
Resonance Imaging ◽  
Total Resection ◽  
Surgical Cure ◽  
Neurologically Intact ◽  
Resonance Imaging Scan

Abstract OBJECTIVE We report the youngest known case of a prepontine intradural chordoma. These tumors are exceedingly rare. Unlike their more common extradural counterparts, no recurrence of an intradural chordoma has been reported. CLINICAL PRESENTATION A 9-year-old boy underwent diagnostic imaging for evaluation of headaches. Although neurologically intact, a magnetic resonance imaging scan revealed a large prepontine mass with focal enhancement. INTERVENTION Endoscopic-assisted gross total resection was attained with staged bilateral retrosigmoid approaches. There were no additional adjuvant therapies. At the time of the 1-year follow-up evaluation, the patient had no recurrence. CONCLUSION By using an endoscopic-assisted procedure, we achieved complete resection of an intradural chordoma offering a potential for surgical cure. Resection is particularly advantageous because it spares the young child the need for radiation treatment. Close follow-up is warranted because we postulate that this tumor exists in a biological continuum between benign notochordal hamartomatous remnants and typical invasive chordomas.

scholarly journals Cyst Formation Following Radiosurgery for AVMs: Report of 3 Cases

2011 ◽  
Vol 38 (5) ◽  
pp. 734-740 ◽  
Author(s):  
Q. Al Hinai ◽  
D. Tampieri ◽  
L. Souhami ◽  
A. Sadikot ◽  
D. Sinclair ◽  
...  
Keyword(s):  
Arteriovenous Malformations ◽  
Radiation Dosage ◽  
Clinical Presentation ◽  
Late Complication ◽  
Cyst Formation ◽  
The Other ◽  
Treatment Site ◽  
Long Term Follow Up

ABSTRACT:Background:Stereotactically-focused radiosurgery (SRS) for the treatment of arteriovenous malformations (AVM) has been in widespread use for over two decades. Over this timeframe the indications for treatment, standardization of radiation dosage, and the results expected from treatment have been elaborated. Less well known are the long-term complications associated with SRS. We report three patients who had SRS for the treatment of AVM who developed a cyst at the site of treatment as a late complication.Methods:From 201 patients treated by SRS for an AVM, three developed a cyst at the treatment site. Their clinical presentation, the characteristics of the AVMs and the treatment were reviewed, as well as similar cases gleaned from the literature.Results:Three women, aged 28-43 years, had an AVM treated by: craniotomy and clipping of arterial feeders followed by SRS, by craniotomy for resection followed by SRS or by endo vascular embolization and SRS. The patients did well following treatment but two of them developed a symptomatic and the other an asymptomatic cyst at the treatment site 3-19 years later. The symptomatic patients underwent marsupialization of the cyst and the other is under observation.Conclusion:Stereotactic radiosurgery is an established and safe treatment for patients with AVMs. Delayed cyst formation can occur many years after treatment and long term follow-up is indicated in patients whose AVM has been treated with SRS.

scholarly journals Spinal angiolipoma: a report of two cases and review of the literature

2020 ◽  
Vol 48 (9) ◽  
pp. 030006052095469
Author(s):  
RuiDeng Wang ◽  
Hai Tang
Keyword(s):  
Standard Treatment ◽  
Clinical Presentation ◽  
Cord Compression ◽  
Benign Tumors ◽  
Fatty Tissue ◽  
Resonance Imaging ◽  
Total Resection ◽  
Vascular Channels ◽  
Spinal Angiolipomas

Spinal angiolipomas (SALs) are extremely rare benign tumors composed of both mature fatty tissue and anomalous vascular channels. We present two cases of SALs and review the clinical presentation, radiological appearance, pathological aspects, and treatment of this distinct clinicopathological mass. The patients’ neurologic symptoms improved postoperatively and follow-up revealed no signs of tumor recurrence or neurological deficit. SAL should be considered as a differential diagnosis in patients with spinal cord compression. Magnetic resonance imaging is important for detecting and characterizing SALs. The gold standard treatment modality should be total resection.

scholarly journals The European Registry on Cushing's syndrome: 2-year experience. Baseline demographic and clinical characteristics

2011 ◽  
Vol 165 (3) ◽  
pp. 383-392 ◽  
Author(s):  
Elena Valassi ◽  
Alicia Santos ◽  
Maria Yaneva ◽  
Miklós Tóth ◽  
Christian J Strasburger ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Clinical Presentation ◽  
Cushing's Syndrome ◽  
The Other ◽  
European Level ◽  
Scale Scores ◽  
Design And Methods ◽  
European Registry ◽  
Baseline Demographic

ObjectiveThe European Registry on Cushing's syndrome (ERCUSYN) is designed to collect prospective and follow-up data at EU level on Cushing's syndrome (CS).Design and methodsBaseline data on 481 CS patients (390 females, 91 males; mean age (±s.d.): 44±14 years) collected from 36 centres in 23 countries, including new patients from 2008 and retrospective cases since 2000. Patients were divided into four major aetiologic groups: pituitary-dependent CS (PIT-CS) (66%), adrenal-dependent CS (ADR-CS) (27%), CS from an ectopic source (ECT-CS) (5%) and CS from other aetiologies (2%).ResultsProportion of men in the ECT-CS group was higher than in the other groups (P<0.05). The ADR-CS group was older than the PIT-CS (P<0.05). Prevalence of hirsutism (92%) and diabetes (74%) in ECT-CS was higher than in the other groups (P<0.05 and P<0.01 respectively). PIT-CS had more skin alterations, menstrual irregularities and hirsutism than ADR-CS (P<0.01). Reduced libido was more prevalent in men than women (P<0.01). Prevalence of spine osteoporosis was higher in men than women (P<0.05), and males had more vertebral and rib fractures than females (52 vs 18% for vertebrae; P<0.001 and 34 vs 23% for ribs; P<0.05). ECT-CS consulted a diabetologist more frequently than ADR-CS (P<0.05), while a gynaecologist was consulted more often by women with PIT-CS or ADR-CS than with ECT-CS (P<0.05). Overall, weight gain was more common in women than men (P<0.01). CushingQoL and EuroQoL visual analogue scale scores did not differ between the groups.ConclusionsThe ERCUSYN project demonstrates a heterogeneous clinical presentation of CS at a European level, depending on gender and aetiology.

scholarly journals GCT-12. INTRACRANIAL GROWING TERATOMA SYNDROME: CLINICAL IMPLICATION FROM SINGLE UNIVERSITY EXPERIENCES

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii330-iii330
Author(s):  
Chae-Yong Kim ◽  
Ji-Yeon Kwon ◽  
Kyeong-O Go ◽  
Kihwan Hwang ◽  
Jung Ho Han ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Germ Cell Tumors ◽  
Clinical Information ◽  
Immature Teratoma ◽  
The Other ◽  
Adjuvant Radiation ◽  
Total Resection ◽  
Growing Teratoma Syndrome ◽  
Diagnostic Characteristics

Abstract In general, intracranial germ cell tumors (GCT) are sensitive to chemotherapy, radiation therapy, and have favorable outcomes. However, a rare chemotherapeutic retro conversion phenomenon, known as intracranial growing teratoma syndrome (iGTS), shown a poorer prognosis. We analyze the diagnostic characteristics and the result of treatment response for the patients with iGTS treated in our institutes (SNUH and SNUBH, from 1997 to 2019). The electronic medical records and PACS were used for reviewing the clinical information, follow-up MRI images, tumor markers (alpha-fetoprotein, human chorionic gonadotropin, in serum or cerebrospinal fluids), and pathological findings. Out of 328 intracranial GCT patients, seventeen were finally identified as iGTS. Sixteen out of 17 patients were non-germinomatous GCTs, and 1 were germinomas. Initial pathology was common in order of immature teratoma (26.7%), other than immature teratoma (11.5%), and germinoma (0.5%). All of the tumors showed typical ‘honeycomb appearance’ in their follow-up MRI images. Sixteen out of 17 tumors were surgically resected as 2nd look surgery. Among them, 13 tumors were gross totally resected. Twelve were alive without evidence of recurrences during follow-up periods, and the other was dead from the progression of the disease. Among the other than the gross total resection group (n=4), two patients were dead, one recurred the tumor, and the other is following up with stable disease after adjuvant radiation therapy. Early detection and total resection of the tumor as possible might be meaningful for favor prognosis, especially in non-germinomatous GCTs patients.

scholarly journals Posterior fossa ependymomas in children: still a challenge for pediatric neurosurgeons and oncologists.

2021 ◽  
Author(s):  
Rel Gerald Boukaka kala ◽  
pierre-aurélien beuriat ◽  
federico di rocco ◽  
Pierre Leblond ◽  
Cecile Faure-Conter ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Facial Nerve Palsy ◽  
Surgical Removal ◽  
Cerebellar Syndrome ◽  
Metastatic Recurrence ◽  
Operative Complications ◽  
Spinal Mri ◽  
Aggressive Tumors

Introduction: Posterior fossa ependymomas in children are associated with a poor prognosis. This retrospective study tries to determine prognostic factors for the outcomes and the quality of life. Material and Methods: Thirty-three patients with posterior fossa ependymomas were treated from 2002 to 2018. All patients had a cranio-spinal MRI and 3.12% were metastatic. Removal was complete in 29 patients (90.62%). All patients received a complementary treatment: 11patients chemotherapy, radiotherapy in 35 patients, 24 cases of neoadjuvant type and for recurrence in 11. Results: Average age was 5.8 years with a range from 9 months to 18 years. The sex ratio was 2.3 (M/F: 23/10). Sixteen patients had one recurrence (3 metastatic). 90% of the EpPCF were of the PFA group. Overall Survival was 65% with a mean follow-up of 8.4 and a median of 9 years. Twenty-one patients were alive (63.63%) at last follow-up. All were tumour-free according the MRI except 2 patients, 1 with a stable residue for 5 years and 1 in palliative treatment for metastatic recurrence for 5 years. Post-operative complications were as follow, facial nerve palsy in four cases (12.12%), swallowing disorders in three cases (9.09%), transient cerebellar syndrome in 4 cases (12.12%). Fifteen patients had normal schooling. Conclusion: Posterior fossa ependymomas are aggressive tumors. Complete surgical removal remains the most important prognostic factor even if responsible of sequels. Despite molecular studies and the expression of different genes, no obvious therapeutic target has yet emerged.

Fully Endoscopic Resection of an Epidermoid Cyst of the Cerebellopontine Angle: Bilateral Resection via a Unilateral Approach

2020 ◽  
Author(s):  
Sricharan Gopakumar ◽  
Visish M Srinivasan ◽  
Himanshu Sharma ◽  
Jacob Cherian ◽  
Akash J Patel
Keyword(s):  
Epidermoid Cyst ◽  
Cerebellopontine Angle ◽  
Clinical Presentation ◽  
Contralateral Side ◽  
Radiographic Imaging ◽  
Total Resection ◽  
Surgical Video ◽  
Unilateral Approach ◽  
Neurovascular Structures

ABSTRACT BACKGROUND AND IMPORTANCE Epidermoid cysts are rare, benign intracranial neoplasms that typically arise at the cerebellopontine angle (CPA) and can be extensive lesions that intricately involve many critical neurovascular structures. We describe the case of a patient who presents with the classic picture of CPA epidermoid cyst and describe the value of the 4K endoscope for resection, which is illustrated in our accompanying surgical video. CLINICAL PRESENTATION The patient presents with headache, nausea, and vomiting accompanied by dizziness and balance issues. Radiographic imaging demonstrated a large lesion highly consistent with epidermoid cyst which involved the left CPA, encircled the basilar artery, and extended to the opposite side. Surgery was planned with a small left-sided retrosigmoid craniotomy with use of a 2-dimensional 4K endoscope to aid in resection, particularly of the contralateral side. This approach was successful with gross total resection apparent at 14-mo follow-up. CONCLUSION We describe the use of a fully endoscopic technique from a unilateral approach for resection of a lesion that extended in the CPA bilaterally. Additionally, we highlight the relevant neuroanatomical and neurovascular structures in this highly critical intracranial region which is well-visualized through endoscopy in the associated surgical video.

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