Extradural Spinal Schwannoma in 12 Year Old Child : A Case Report
Journal name: The Malaysian Journal of Medical Sciences
Original article title: Extradural Spinal Schwannoma in 12 Year Old Child : A Case Report
The Malaysian Journal of Medical Sciences (MJMS) is a peer-reviewed, open-access journal published online at least six times a year. It covers all aspects of medical sciences and prioritizes high-quality research.
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Toh Charng Jeng, Jafri Malin Abdullah, Jain George, John Tharakan KJ, Sharon Casilda, Mazira Mohamad Ghazali, Hasnan Jaafar, Win Mar Salmah
The Malaysian Journal of Medical Sciences:
(A peer-reviewed, open-access journal)
Full text available for: Extradural Spinal Schwannoma in 12 Year Old Child : A Case Report
Year: 2005
Copyright (license): CC BY 4.0
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Summary of article contents:
Introduction
This report discusses a case of a 12-year-old girl who presented with progressive bilateral lower limb weakness and was subsequently diagnosed with an extradural schwannoma located in the lower thoracic and upper lumbar regions of her spine. Spinal tumors in children are relatively rare, accounting for 5–10% of central nervous tumors in this age group, with common extradural tumors including sarcoma, neuroblastoma, and lymphoma. Schwannomas are infrequently observed in pediatric patients and usually present as intradural mass lesions, but in this instance, the tumor was found to be exclusively extradural. Furthermore, associated clinical features raised the suspicion of Neurofibromatosis Type I.
Clinical Significance of Neurofibromatosis Type I
The presence of multiple café-au-lait spots, aqueductal stenosis with arrested hydrocephalus, and neurological deficits in this patient suggested Neurofibromatosis Type I (NF1), even though she did not fully meet the diagnostic criteria. NF1 is characterized by a range of clinical manifestations, including skin lesions, neurological complications, and skeletal abnormalities. In this case, the existence of the extradural schwannoma, combined with other findings such as kyphoscoliosis and "unidentified bright objects" in the cerebral cortex, pointed to a potential NF1 diagnosis. Importantly, genetic studies can aid in distinguishing between NF1 and NF2, as treatment and prognostic implications differ significantly between the two conditions.
Conclusion
The case presented demonstrates that while schwannomas are rare in the pediatric population, their occurrence can be associated with genetic disorders such as Neurofibromatosis Type I. The patient underwent successful surgical excision of the extradural schwannoma and showed significant postoperative recovery. This highlights the importance of vigilance for neurological signs in patients with congenital anomalies and skin manifestations. Long-term follow-up is crucial for early detection of potential complications in patients with NF1, ensuring that any emerging neurological deficits can be addressed in a timely manner.
FAQ section (important questions/answers):
What symptoms did the 12-year-old girl experience related to her condition?
The girl experienced progressive bilateral lower limb weakness, radicular pain, and urinary hesitancy. She also had physical examination findings including café-au-lait spots, mild spasticity, and absent knee reflexes.
What were the imaging findings for the girl's spinal tumor?
An MRI showed an extradural lesion from T12 to L1, isointense to hypointense on T1, which caused cord compression. Additionally, multiple unidentified bright objects were observed in the brain.
How was the spinal tumor diagnosed and treated?
The tumor was diagnosed as a schwannoma after histopathological examination following surgical excision via laminectomy. It was noted to be well-capsulated and entirely confined to the extradural space.
What features suggested the possibility of Neurofibromatosis Type I in this case?
The presence of café-au-lait spots, aqueductal stenosis, kyphoscoliosis, and unexplained neurological symptoms indicated possible Neurofibromatosis Type I, though not all diagnostic criteria were fully met.
Glossary definitions and references:
Scientific and Ayurvedic Glossary list for “Extradural Spinal Schwannoma in 12 Year Old Child : A Case Report”. This list explains important keywords that occur in this article and links it to the glossary for a better understanding of that concept in the context of Ayurveda and other topics.
1) Disease:
Disease refers to a pathological condition affecting the body, often leading to dysfunction or distress. In the context of this case, Neurofibromatosis Type I is a genetic disorder discussed in relation to the spinal schwannoma and its clinical manifestations, which include café-au-lait spots and neurological issues.
2) Mutation:
Mutation signifies a change in the genetic material of an organism. The analysis for mutations in the NF2 gene was mentioned as part of the diagnostic workup for the patient, helping to determine the presence of Neurofibromatosis. Mutations can affect disease severity and treatment strategies.
3) Arrow:
Arrow represents visual markers used in imaging studies, such as MRI. In this case, arrows are used to indicate the location of the extradural schwannoma and its effect on the spinal cord, highlighting the significance of precise imaging in diagnosis and surgical planning.
4) Post:
Post refers to the outcomes that occur following a medical intervention. The term is used to describe the patient's progress after the surgical excision of the tumor, emphasizing recovery times and the importance of monitoring in pediatric patients after such procedures.
5) Young girl:
Young girl identifies the demographic context of the case study, being a 12-year-old with significant neurological symptoms and a diagnosis of schwannoma. Her age and sex can affect the clinical presentation and management of spinal tumors in children, influencing treatment decisions.
6) Medicine:
Medicine is the science and practice of diagnosing, treating, and preventing illness. In this report, it encompasses the surgical and medical management of the 12-year-old girl's spinal tumor, reflecting the broader application of medical principles to address complex health problems.
7) Activity:
Activity refers to the functional capabilities of the patient. It highlights the girl's ability to carry out daily living tasks post-surgery, demonstrating the impact of neurological conditions on quality of life, and underscoring the importance of rehabilitation in pediatric cases.
8) Family:
Family pertains to the genetic aspect of conditions like Neurofibromatosis where familial patterns can influence diagnosis. In this case, the lack of family history of Neurofibromatosis is significant, suggesting a sporadic occurrence of the condition rather than inherited.
9) Annal (Annaḷ, Aṇṇal, Aṉṉal, Annāḷ):
Annal refers to a record or report, particularly in the context of medical literature. The term points to the citation of previous research and case studies relevant to neurofibromatosis and schwannomas, providing a foundation for understanding the rarity and characteristics of these tumors.
10) Study (Studying):
Study implies a systematic investigation into a medical condition. In this context, it references both the case at hand and the larger body of clinical knowledge regarding schwannomas and their presentation in pediatric patients, contributing to ongoing research and treatment methods.
11) Sign:
Sign refers to the clinical manifestations observed in patients, which aid in diagnosis. In this report, signs include neurological deficits and physical findings, providing crucial evidence for clinicians in identifying and managing the underlying pathology affecting the patient.
12) Bell:
Bell can refer to a 'dumb-bell shaped tumor,' a typical presentation of schwannomas when they occur intradural. The mention of this shape provides insight into the nature of the tumor's development, although in this report, a pure extradural formation was highlighted.
13) Pain (Paiṇ):
Pain is a subjective experience indicating discomfort or distress often associated with medical conditions. In this case, radicular pain experienced by the girl is an important symptom indicating spinal cord involvement, which plays a critical role in the assessment and urgency of treatment.
14) Hand:
Hand can relate to the examination of motor function in terms of limb strength and neurological evaluation. Physical examination demonstrates the impact of the tumor on motor skills, emphasizing the importance of thorough assessments in determining the extent of neurological compromise.
15) Pur (Pūr):
Poor typically indicates a deficiency in quality or function. Here, it refers to the mild mental subnormality of the patient, reflecting the associated cognitive impacts of her condition and the importance of neurological assessments in understanding the broader effects of neurofibromatosis.
Other Science Concepts:
Discover the significance of concepts within the article: ‘Extradural Spinal Schwannoma in 12 Year Old Child : A Case Report’. Further sources in the context of Science might help you critically compare this page with similair documents:
Surgical excision, Early detection, Long-term follow-up, Laminectomy, Neurological Deficit, Muscle power, Micturation, Histopathological examination, Radicular pain, Magnetic resonance imaging, Biochemical investigation, Spinal canal, Paediatric age group, Motor system examination, MRI of spine, MRI of brain, Kyphoscoliosis, Lower limb weakness, Past medical history, Genetic studies, Spindle cells, Bilateral lower limb weakness, Activity of daily living, Intracranial Pressure, Vestibular schwannoma, Post Operatively, Cafe au lait spots, Vascular anomalies, Primary bone tumors.