Pediatric spinal pilomyxoid astrocytoma

2013 ◽  
Vol 12 (5) ◽  
pp. 511-516 ◽  
Author(s):  
Sarah T. Garber ◽  
Robert J. Bollo ◽  
Jay K. Riva-Cambrin

Pediatric spinal pilomyxoid astrocytoma (PMA) is an extremely rare tumor that merits recognition as a specific, unique entity. The authors present the case of an intramedullary PMA in the thoracic spinal cord of an 11-year-old boy who presented with back pain, scoliosis, and multiple lung nodules. The patient underwent T5–11 laminoplasty and near-total resection of the spinal tumor. The final pathological diagnosis was WHO Grade II PMA. The patient did well for 14 months until the tumor progressed both clinically and radiographically. A literature review focusing on the clinical characteristics, histology, and treatment of PMAs provides a better understanding of these rare lesions. Because of the small number of cases optimal treatment guidelines have not been established, but gross-total resection and adjuvant chemotherapy with alkylating agents appear to confer a better long-term prognosis. Pediatric patients with PMAs can remain recurrence free at least 5 years after surgery, although these tumors may disseminate or dedifferentiate into more malignant gliomas. Recognition of intramedullary PMA as a unique entity in children is vital to the development of specific surgical and adjuvant treatment regimens.

2019 ◽  
Vol 8 (1) ◽  
pp. 5
Author(s):  
Dimitrios Panagopoulos

Background: Meningeal melanocytoma is a rare benign tumor, most frequently located in the posterior fossa and spinal canal. Our objective is to illustrate a case of this tumor that originated in the thoracolumbar area of the spine and had an uneventful clinical course after total resection. Case description: We present the case of a 59 years old woman who presented with a medical history of ongoing neurological deterioration due to spastic paresis of the lower extremities. MRI of the thoracolumbar region identified a melanocytic melanoma as the underlying cause. Conclusions: Melanocytic tumors of the central nervous system have a typical appearance on MRI scans, varying with the content and distribution of melanin. However, the differential diagnosis between malignant melanoma and melanocytoma still depends on pathological criteria. Spinal meningeal melanocytoma has a benign course, and it is amenable for gross total resection. The outcome is favorable following complete resection.


2020 ◽  
Vol 11 ◽  
pp. 423
Author(s):  
Jesus Rocha-Maguey ◽  
Jesus Salvador Velarde-Felix ◽  
Myriam Cabrera-Lopez ◽  
Jaime Moya-Nuñez ◽  
Edgar Fragosa-Sanchez

Background: Clear cell ependymomas (CCEs) are a rare variant of tumors of the nervous system, the main location is the intracranial compartment. Special differential diagnosis should be done with oligodendrogliomas, neurocytoma, glioneurocytoma, astrocytoma, or metastatic renal cell carcinoma, lesions that somehow share cells with clear cytoplasm. Most of these lesions are benign but differential diagnosis is essential to decide further treatment. Few case reports of intramedullary CCEs have being published and there is no strict consensus on the diagnostic criteria. Case Description: We hereby describe a new case of an intramedullary clear CCE with very few neurological symptoms, surgical treatment is satisfactory, histological and immunohistochemical analysis was confirmatory. After gross total resection and 3-year follow-up no recurrence of the lesion is evident. Conclusion: After this case presentation and review of the limited literature, it is evident that methodical clinical suspicion, radiological imaging combined with histological, and modern immunohistochemical techniques are essential for the diagnosis. Surgical options with gross total resection remain the cornerstone of its treatment. Neurophysiological monitoring is extremely useful to avoid postoperative morbidity.


2019 ◽  
Vol 11 ◽  
pp. 175883591987555 ◽  
Author(s):  
Shih-Jung Liu ◽  
Shun-Tai Yang ◽  
Shu-Mei Chen ◽  
Yin-Chen Huang ◽  
Wei-Hwa Lee ◽  
...  

Background: Malignant gliomas (MGs) are highly chemotherapy-resistant. Temozolomide (TMZ) and carmustine (BiCNU) are alkylating agents clinically used for treating MGs. However, their effectiveness is restrained by overexpression of the DNA repair protein O6-methylguanine-DNA methyltransferase (MGMT) in tumors. O6-benzylguanine (O6-BG) is a nonreversible inhibitor of MGMT, it promotes the cytotoxicity of alkylating chemotherapy. The authors have developed a hybrid-structured nanofibrous membrane (HSNM) that sequentially delivers high concentrations of O6-BG, BiCNU, and TMZ in an attempt to provide an alternative to the current therapeutic options for MGs. Methods: The HSNMs were implanted onto the cerebral surface of pathogen-free rats following surgical craniectomy, while the in vivo release behaviors of O6-BG, TMZ, and BiCNU from the HSNMs were explored. Subsequently, the HSNMs were surgically implanted onto the brain surface of two types of tumor-bearing rats. The survival rate, tumor volume, malignancy of tumor, and apoptotic cell death were evaluated and compared with other treatment regimens. Results: The biodegradable HSNMs sequentially and sustainably delivered high concentrations of O6-BG, BiCNU, and TMZ for more than 14 weeks. The tumor-bearing rats treated with HSNMs demonstrated therapeutic advantages in terms of retarded and restricted tumor growth, prolonged survival time, and attenuated malignancy. Conclusion: The results demonstrated that O6-BG potentiates the effects of interstitially transported BiCNU and TMZ. Therefore, O6-BG may be required for alkylating agents to offer maximum therapeutic benefits for the treatment of MGMT-expressing tumors. In addition, the HSNM-supported chemoprotective gene therapy enhanced chemotherapy tolerance and efficacy. It can, therefore, potentially provide an improved therapeutic alternative for MGs.


2021 ◽  
Vol 9 (7) ◽  
Author(s):  
Mohamed A. Almzeogi ◽  
Zeyad A. Abousabie ◽  
Jelena Kostic ◽  
Aleksandar M. Janicijevic ◽  
Goran Tasic

2014 ◽  
Vol 36 (2) ◽  
pp. E11 ◽  
Author(s):  
Matthias Millesi ◽  
Barbara Kiesel ◽  
Adelheid Woehrer ◽  
Johannes A. Hainfellner ◽  
Klaus Novak ◽  
...  

Object Subtotal resection (STR) of spinal tumors can result in tumor recurrence. Currently, no clinically reliable marker is available for intraoperative visualization of spinal tumor tissue. Protoporphyrin IX (PpIX) fluorescence induced by 5-aminolevulinic acid (5-ALA) is capable of visualizing malignant gliomas. Fluorescence-guided resections of malignant cerebral gliomas using 5-ALA have resulted in an increased rate of complete tumor removal. Recently, the application of 5-ALA has also been described in the first cases of spinal tumors. Therefore, the aim of this observational study was to systematically investigate 5-ALA–induced fluorescence characteristics in different spinal tumor entities. Methods Three hours before the induction of anesthesia, 5-ALA was administered to patients with different intra- and extradural spinal tumors. In all patients a neurosurgical resection or biopsy of the spinal tumor was performed under conventional white-light microscopy. During each surgery, the presence of PpIX fluorescence was additionally assessed using a modified neurosurgical microscope. At the end of an assumed gross-total resection (GTR) under white-light microscopy, a final inspection of the surgical cavity of fluorescing intramedullary tumors was performed to look for any remaining fluorescing foci. Histopathological tumor diagnosis was established according to the current WHO classification. Results Fifty-two patients with 55 spinal tumors were included in this study. Resection was performed in 50 of 55 cases, whereas 5 of 55 cases underwent biopsy. Gross-total resection was achieved in 37 cases, STR in 5, and partial resection in 8 cases. Protoporphyrin IX fluorescence was visible in 30 (55%) of 55 cases, but not in 25 (45%) of 55 cases. Positive PpIX fluorescence was mainly detected in ependymomas (12 of 12), meningiomas (12 of 12), hemangiopericytomas (3 of 3), and in drop metastases of primary CNS tumors (2 of 2). In contrast, none of the neurinomas (8 of 8), carcinoma metastases (5 of 5), and primary spinal gliomas (3 of 3; 1 pilocytic astrocytoma, 1 WHO Grade II astrocytoma, 1 WHO Grade III anaplastic oligoastrocytoma) revealed PpIX fluorescence. It is notable that residual fluorescing tumor foci were detected and subsequently resected in 4 of 8 intramedullary ependymomas despite assumed GTR under white-light microscopy. Conclusions In this study, 5-ALA–PpIX fluorescence was observed in spinal tumors, especially ependymomas, meningiomas, hemangiopericytomas, and drop metastases of primary CNS tumors. In cases of intramedullary tumors, 5-ALA–induced PpIX fluorescence is a useful tool for the detection of potential residual tumor foci.


2020 ◽  
Vol 13 ◽  
pp. 300-303
Author(s):  
Kelsey Knobbe ◽  
Melissa Gaines

Intramedullary tuberculoma (IMT) is a form of spinal tuberculosis which provides a challenge to the internist because there lacks clear diagnostic and treatment guidelines to date. Although rare, it should be included on the differential diagnosis of any patient who presents with progressively worsening symptoms of radiculopathy and a new spinal lesion on imaging. Traditional methods for detection of tuberculosis including tuberculosis spot test and the Ziehl-Neelsen stain have limited utility in diagnosing IMT. In this report, we describe the clinical course, diagnosis and treatment regimen of an adult male with intramedullary tuberculoma of the thoracic spinal cord


Author(s):  
Zeyad Abousabie ◽  
Mohamed Almzeogi ◽  
aleksandar janicijevic ◽  
Goran Tasic

Case summary: The MRI of a 73 year old male patient with paraparesis, showed an intramedullary mass at the thoracic spinal cord extending from T6 to T8. Partial surgical removal was preformed and a biopsy was taken, followed by postoperative radiotherapy.


2001 ◽  
Vol 45 (4) ◽  
pp. 353 ◽  
Author(s):  
Sung Chan Jin ◽  
Seoung Ro Lee ◽  
Dong Woo Park ◽  
Kyung Bin Joo

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