Spinal Schwannomas Research Articles

Concurrent spinal schwannoma and meningioma without neurofibromatosis: case presentation and review of literature

Abstract Background Concurrent multiple tumors developing in the spinal cord are rare, except for in genetic disorders, such as neurofibromatosis and von Hippel–Lindau disease. The presence of multiple histologically different spinal tumors in the absence of NF-2 is extremely rare. Schwannoma and meningioma can be explained partly by their common origin from the same mesenchymal cell, there is currently no explanation for the simultaneous existence of different spinal tumors other than simple coincidence. Case presentation After review of literature, there are thirteen such cases reported in the literature in addition to the present case. The current patient presents with lower limb weakness, and the patient radiology revealed intradural mass opposite the fourth lumbar vertebra with contrast enhancement and intradural extramedullary mass opposite the tenth dorsal vertebra with contrast enhancement in MRI spine. The coexistence of these tumors may be coincidental or might be due to a common histological origin of the Schwann cells and the meningocytes. Conclusion Schwannoma and meningioma have different radiological characteristics and require different managing procedures such as dural base coagulation in meningiomas and rootlet sacrifice in schwannomas.

Excision of a supergiant spinal schwannoma: illustrative case.

The authors report on a patient who presented with an extremely large presacral schwannoma and subsequent mass effect-induced hydronephrosis and kidney failure. To the authors' knowledge, this case represents the largest radiographically verified spinal schwannoma in the medical literature. The tumor presented here was more than three times as large as a typical giant schwannoma. While smaller presacral schwannomas are usually uncomplicated surgical cases, the supergiant schwannomas described here create significant surgical challenges. The extremely large size of this nerve sheath tumor introduced a level of surgical complexity not seen in most spinal schwannoma cases. The authors hope that this case informs surgeons regarding the approach to excision of giant spinal schwannomas. The resection of giant spinal schwannomas is likely to involve one or more lengthy surgeries, and the resection volume can be limited by excessive bleeding. Physicians approaching these large, benign spinal tumors should be aware of the challenges of surgery duration and hemostasis before approaching a tumor of this kind. https://thejns.org/doi/10.3171/CASE24224.

Generation of a zebrafish neurofibromatosis model via inducible knockout of nf2.

Neurofibromatosis Type 2 (NF-2) is a dominantly inherited genetic disorder that results from mutations in the tumor suppressor gene, neurofibromin 2 (NF2) gene. Here, we report the generation of a conditional zebrafish model of neurofibromatosis established by an inducible genetic knockout of nf2a/b, the zebrafish homolog of human NF2. Analysis of nf2a and nf2b expression reveals ubiquitous expression of nf2b in the early embryo, with overlapping expression in the neural crest and its derivatives and in the cranial mesenchyme. In contrast, nf2a displays lower expression levels. Induction of nf2a/b knockout at early stages increases the proliferation of larval Schwann cells and meningeal fibroblasts. Subsequently, in adult zebrafish, nf2a/b knockout triggers the development of a spectrum of tumors, including vestibular Schwannomas, spinal Schwannomas, meningiomas, and retinal hamartomas, mirroring the tumor manifestations observed in patients with NF-2. Collectively, these findings highlight the generation of a novel zebrafish model that mimics the complexities of the human NF-2 disorder. Consequently, this model holds significant potential for facilitating therapeutic screening and elucidating key driver genes implicated in NF-2 onset.

6564 A Case of Severe Hypercalcemia in Carney's Complex

Abstract Disclosure: J. Chippior: None. M. Rayan: None. L. Wright: None. L.S. Kirschner: Grant Recipient; Self; Medpace, Sparrow, Corcept Therapeutics, Corcept, Spruce, CinCor, Crinetics, Adrenas. S.W. Ing: Grant Recipient; Self; Alexion Pharmaceuticals, Inc., Amgen Inc, Radius Health, Inc, Takeda, Ultragenyx, Amolyt, Bridge Bio. Carney complex is a rare syndrome with fewer than 750 reported cases, characterized by lentigniosis, myxomas, and the occurrence of both endocrine and non-endocrine tumors. An inactivating mutation PRKAR1A, which encodes for the type 1A regulatory subunit of Protein kinase A, is responsible for over 70% of familial cases and 37% of sporadic cases. A 31-year-old female with a history of Carney complex, Cushing syndrome due to primary pigmented nodular adrenocortical disease (PPNAD), facial lentigines, spinal Schwannomas, and recent ischemic stroke secondary to embolization of a cardiac atrial myxoma, was transferred to our medical center for hyperemesis and severe hypercalcemia. Albumin-corrected serum calcium measured 15.4 mg/dL (8.6-10.5 mg/dL), ionized calcium 7.8 mg/dL (4.6-5.3 mg/dL) associated with suppressed PTH, normal PTH-related protein, and elevated bone turnover markers, C-telopeptide 4,273 pg/ml (150-635) and Procollagen Type 1 N-terminal Propeptide 222 mcg/L (19-83). Evaluation for etiology of hypercalcemia revealed elevated 1,25 dihydroxy vitamin D at 107.0 pg/ml (20-79) and elevated interleukin-6 at 11 pg/ml (<6.4). IL-6 is expressed by cardiac myxomas. Serum angiotensin-converting enzyme level was 50 U/L (16-85) and infectious workup was negative for potential granulomatous causes of elevated calcitriol (including testing for tuberculosis, histoplasma, blastomyces, bartonella, and coccidioides). CT angiogram of the abdomen and pelvis (completed in preparation of atrial myxoma resection) revealed ground-glass opacities in bilateral posterior lung fields and multiple pulmonary nodules, with the largest measuring 8 mm. After treatment with aggressive intravenous fluid resuscitation, intramuscular calcitonin, and intravenous zoledronic acid, corrected calcium normalized to 9.4 mg/dL at discharge. Urinary cortisol was 6.2 mcg per 24-hr (3.5-45), for which she started hydrocortisone 20 mg daily to treat suspected adrenal insufficiency as excess cortisol secretion in PPNAD may be episodic. Thus remission of hypercortisolism may have unmasked an underlying hypercalcemia, and adrenal insufficiency itself may have contributed to this finding. She had excision of left atrial myxoma and has continued to exhibit normal calcium levels on steroid replacement therapy. Repeat chest CT to further characterize these pulmonary findings is planned. This case illustrates a unique constellation of endocrine features associated with severe hypercalcemia in Carney complex. Presentation: 6/1/2024

6564 A Case Of Severe Hypercalcemia In Carney's Complex

Abstract Disclosure: J. Chippior: None. M. Rayan: None. L. Wright: None. L.S. Kirschner: Grant Recipient; Self; Medpace, Sparrow, Corcept Therapeutics, Corcept, Spruce, CinCor, Crinetics, Adrenas. S.W. Ing: Grant Recipient; Self; Alexion Pharmaceuticals, Inc., Amgen Inc, Radius Health, Inc, Takeda, Ultragenyx, Amolyt, Bridge Bio. Carney complex is a rare syndrome with fewer than 750 reported cases, characterized by lentigniosis, myxomas, and the occurrence of both endocrine and non-endocrine tumors. An inactivating mutation PRKAR1A, which encodes for the type 1A regulatory subunit of Protein kinase A, is responsible for over 70% of familial cases and 37% of sporadic cases. A 31-year-old female with a history of Carney complex, Cushing syndrome due to primary pigmented nodular adrenocortical disease (PPNAD), facial lentigines, spinal Schwannomas, and recent ischemic stroke secondary to embolization of a cardiac atrial myxoma, was transferred to our medical center for hyperemesis and severe hypercalcemia. Albumin-corrected serum calcium measured 15.4 mg/dL (8.6-10.5 mg/dL), ionized calcium 7.8 mg/dL (4.6-5.3 mg/dL) associated with suppressed PTH, normal PTH-related protein, and elevated bone turnover markers, C-telopeptide 4,273 pg/ml (150-635) and Procollagen Type 1 N-terminal Propeptide 222 mcg/L (19-83). Evaluation for etiology of hypercalcemia revealed elevated 1,25 dihydroxy vitamin D at 107.0 pg/ml (20-79) and elevated interleukin-6 at 11 pg/ml (<6.4). IL-6 is expressed by cardiac myxomas. Serum angiotensin-converting enzyme level was 50 U/L (16-85) and infectious workup was negative for potential granulomatous causes of elevated calcitriol (including testing for tuberculosis, histoplasma, blastomyces, bartonella, and coccidioides). CT angiogram of the abdomen and pelvis (completed in preparation of atrial myxoma resection) revealed ground-glass opacities in bilateral posterior lung fields and multiple pulmonary nodules, with the largest measuring 8 mm. After treatment with aggressive intravenous fluid resuscitation, intramuscular calcitonin, and intravenous zoledronic acid, corrected calcium normalized to 9.4 mg/dL at discharge. Urinary cortisol was 6.2 mcg per 24-hr (3.5-45), for which she started hydrocortisone 20 mg daily to treat suspected adrenal insufficiency as excess cortisol secretion in PPNAD may be episodic. Thus remission of hypercortisolism may have unmasked an underlying hypercalcemia, and adrenal insufficiency itself may have contributed to this finding. She had excision of left atrial myxoma and has continued to exhibit normal calcium levels on steroid replacement therapy. Repeat chest CT to further characterize these pulmonary findings is planned. This case illustrates a unique constellation of endocrine features associated with severe hypercalcemia in Carney complex. Presentation: 6/1/2024

Recurrence of spinal schwannomas in a patient with neurofibromatosis type 1: a case report

Introduction and importance: Spinal schwannomas are benign tumors usually attached to peripheral nerves, consisting of a clonal population of Schwann cells. Neurofibromatosis type 1 is an autosomal dominant neurocutaneous disorder that predominantly affects the skin, bone and nervous system. Neurofibromatosis type 1 is a clinically and genetically distinct from neurofibromatosis type 2. This case report highlights the rare association between spinal schwannoma and neurofibromatosis type 1. Case presentation: The patient with a past medical history of spinal schwannoma, operated 1 year back, presented with back pain, weakness of lower limbs and urge incontinence. On examination, she had cutaneous features suggestive of neurofibromatosis type 1 and there was impairment of all sensory modalities below hip region. MRI revealed spinal schwannoma at D9 level for which laminectomy with removal of schwannoma was performed. Clinical discussion: The occurrence and recurrence of spinal schwannoma in neurofibromatosis type 2 is a common finding. But such an association has not been established between spinal schwannoma and neurofibromatosis type 1. In this case, the recurrence of spinal schwannoma has been linked to neurofibromatosis type 1 in the absence of other well-defined etiologies. Conclusion: The occurrence of spinal schwannoma can be genetic or sporadic. The recurrence is usually associated with familial tumor syndrome. The available literature has not established association between neurofibromatosis type 1 and spinal schwannoma, thus, emphasizing the need of more focused studies.

Epidemiology, tumour characteristics, treatment and outcomes associated with spinal nerve sheath tumours: a systematic review protocol

IntroductionNerve sheath tumours arise from both the central and peripheral nervous systems. In particular, cases of spinal or paraspinal origins are scarce and poorly covered in the literature. This systematic...

Spinal Schwannoma Classification Based on the Presumed Origin With Preoperative Magnetic Resonance Images.

Classification guides the surgical approach and predicts prognosis. However, existing classifications of spinal schwannomas often result in a high 'unclassified' rate. Here, we aim to develop a new comprehensive classification for spinal schwannomas based on their presumed origin. We compared the new classification with the existing classifications regarding the rate of 'unclassified'. Finally, we assessed the surgical strategies, outcomes, and complications according to each type of the new classification. A new classification with 9 types was created by analyzing the anatomy of spinal nerves and the origin of significant tumor portions and cystic components in preoperative magnetic resonance images. A total of 482 patients with spinal schwannomas were analyzed to compare our new classification with the existing classifications. We defined 'unclassified' as the inability to classify a patient with spinal schwannoma using the classification criteria. Surgical approaches and outcomes were also aligned with our new classification. Our classification uniquely reported no 'unclassified' cases, indicating full applicability. Also, the classification has demonstrated usefulness in predicting the surgical outcome with the approach planned. Gross total removal rates reached 88.0% overall, with type 1 and type 2 tumors at 95.3% and 96.0% respectively. The approach varied with tumor type, with laminectomy predominantly used for types 1, 2, and 9, and facetectomy with posterior fixation used for type 3 tumors. The new classification for spinal schwannomas based on presumed origin is applicable to all spinal schwannomas. It could help plan a surgical approach and predict its outcome, compared with existing classifications.

Analysis of the current status and hot topics in spinal schwannoma imaging research based on bibliometrics.

This study aims to explore the current hot topics and future research trends in spinal schwannoma imaging research, providing a reference for related studies and promoting the development of spinal schwannoma imaging. We conducted a literature search in the Web of Science database using the search terms (((TS = (Spinal schwannoma)) AND TS = (Imaging)) OR TS = (Spinal schwannoma)) AND TS = (image) to retrieve relevant articles. The collected data, including authors, keywords, journals, countries, institutions, and references, were subjected to visual analysis using the visualization software CiteSpace 6.4.2R and VOSviewer 1.6.19. A total of 310 relevant articles were identified. After further screening based on time limits, inclusion, and exclusion criteria, 179 articles were included in the study, consisting of 132 original articles and 42 reviews. These articles were authored by 1,034 authors from 35 countries and 324 institutions and were published in 82 different journals. The included articles cited a total of 6,583 references from 1,314 journals. Although the field of spinal schwannoma imaging research is not a popular research area in the medical community, there has been an increasing international interest in this field in recent years. While China ranks high in terms of the number of published articles, there is still a gap in terms of the quality and research level compared to developed countries in Europe and America. MRI, as the gold standard for diagnosing spinal schwannomas, is expected to be a research hotspot in terms of feature analysis, enhancement characteristics, and quantitative analysis. It is also hoped that China can increase its investment in research and contribute to the field by publishing high-quality articles in the future.

P82. The clinical significance of tumor location in surgical management of nonsyndromic spinal schwannomas

P82. The clinical significance of tumor location in surgical management of nonsyndromic spinal schwannomas

Functional outcome after introduction of hemilaminectomy in management of spinal schwannomas and meningiomas

BackgroundSchwannomas and meningiomas are intradural extramedullary spinal tumors which are regularly encountered in the neurosurgical clinic. These tumors cause neurological deficit by compression on the spinal cord and commonly pain when affecting the cauda equina. The traditional treatment with standard laminectomy (SL) can cause instability to the dorsal segments of the spinal column, and the less invasive option of hemilaminectomy (HL) has therefore been developed. We aim in this study to investigate transition from SL to HL in a population-based cohort. MethodsAdult patients (18 years and older) undergoing primary surgery due to spinal meningioma or schwannoma between 2007 and 2022 at the neurosurgical clinic were included. Data related to clinical, surgical and outcome variables were retrospectively collected. ResultsA total of 187 patients were identified: 155 in the SL group, 26 in the HL group and in 6 patients a combination of SL and HL. The mean age of the SL group was 62.7 years (SD14.2) compared to 58.0 (SD15.7) in the HL group (p = 0.16). Preoperative motor deficit was more common in SL group compared to HL group (76.8 % and 61.5 %, respectively, p = 0.14). Thoracal location was most common for both groups (SL 65.8 % and HL 61.5 %). Postoperative change in McCormick grades and early complications were similar between groups. ConclusionOutcome after hemilaminectomy due to intradural extramedullary schwannoma or meningioma is comparable to standard laminectomy with regards to postoperative complications and neurological improvement. Our findings support the transition to hemilaminectomy in selected cases.

A separate-dural-incision method of extradural dumbbell spinal schwannoma resection: cumulative experience at a single center

ObjectiveTo present our experience in the surgical management of completely extradural dumbbell spinal schwannomas with a new surgical strategy.MethodThis study is a case series of patients treated at the Neurosurgery Department of the First Affiliated Hospital of USTC, between January 2018 and June 2021.Results24 patients met the inclusion criteria, with cervical and lumbar spines being the most frequent locations. All patients underwent surgical treatment. Total gross resection was accomplished in all patients. Two cases had numbness and no case exhibited motor deficit. There was no postoperative CSF leakage or wound infection.ConclusionBased on a limited number of observations, we conclude that our technique was feasible and effective for the treatment of extradural dumbbell spinal schwannomas.Clinical trialhttp://www.chictr.org.cn/, No. ChiCTR2400086171.

Giant Lumbosacral Extradural Intraosseous Schwannoma in an Adolescent Male – A Rare Lesion

Abstract Schwannoma are benign slow growing tumor arising from Schwann cells of the nerve sheaths of peripheral nerves. Giant spinal schwannomas are defined as lesions that extend over more than two vertebral levels or have extraspinal extension of more than 2.5 cm. They are usually intradural, Extradural giant spinal schwannomas in the adolescent age group is of rare. Only 5 cases of purely extradural giant spinal schwanomma have been reported in literature. We report a purely extradural giant schwanomma and its management.

Malignant melanotic schwannoma of the cervical spinal cord: a case report

Spinal cord malignant melanotic schwannoma (MMNST) is a rare central nervous system tumor that originates from the spinal cord or spinal myelin sheath cells and can produce melanin. This type of tumor is usually highly aggressive and malignant, with a poor prognosis. The clinical manifestations of spinal cord MMNST are mainly pain, paresthesia, muscle weakness, muscle atrophy, etc., and symptoms of spinal cord compression, such as intestinal and bladder dysfunction, paraplegia, etc. Early detection of tumor lesions can facilitate tumor removal, improve patients’ quality of life, and prolong patients’ survival. In this case report, a 27-year-old young woman was diagnosed with MMNST of the cervical spinal cord due to weakness of her limbs in our hospital, and underwent surgical resection. The patient’s limbs returned to normal after surgery. It is worth mentioning that the patient visited our hospital 7 months ago for “right upper limb pain for 3 days” and was diagnosed with a cervical spine space-occupying lesion at the same position this time, but the pathology report was “hemosiderosis”. This case report aims to raise awareness of the problem of spinal cord MMNST and contribute to greater knowledge of this rare tumor.

Unilateral hemilaminectomy vs. laminoplasty for the resection of spinal schwannomas: an analysis of 100 patients.

Spinal schwannomas are the most common intradural extramedullary tumors, and their complete removal is recommended to avoid tumor recurrence. Although laminoplasty provides a sufficient window for tumor resection, this approach may increase tissue trauma and cause postoperative instability compared with unilateral hemilaminectomy. This study aimed to compare the efficacy and clinical outcomes of the two approaches. We included 100 consecutive patients who underwent unilateral hemilaminectomy or laminoplasty for resection of spinal schwannomas between January 2015 and February 2023. The patients' baseline characteristics, including sex, age, tumor location, percentage of tumor occupying the intradural space, operative time, postoperative length of hospital stay, intraoperative bleeding volume, visual analog scale score, and neurologic results, were retrospectively analyzed. Hemilaminectomy patients who underwent unilateral hemilaminectomy had smaller intraoperative bleeding (p = 0.020) volume, shorter operative time (p = 0.012), and shorter postoperative length of hospital stay (p = 0.044). The mean VAS scores at the last follow-up were similar between the two groups (p = 0.658). Although the postoperative McCormick and Karnofsky Performance scores were not significantly different between the laminoplasty and unilateral hemilaminectomy groups (p = 0.687 and p = 0.649, respectively), there was a statistically significant improvement based on postoperative neurological results compared to preoperative neurological results for both groups. The incidence of postoperative complications was 5% and 11.7% in the unilateral hemilaminectomy and laminoplasty groups, respectively (p = 0.308). For spinal schwannoma resection, unilateral hemilaminectomy has more advantages than laminoplasty, including a shorter postoperative hospital stay, faster procedure, and less intraoperative blood loss while achieving the same desired result.

Differentiation of the Intradural Extramedullary Spinal Tumors, Schwannomas, and Meningiomas Utilizing the Contrast Ratio as a Quantitative Magnetic Resonance Imaging Method

Schwannomas and meningiomas are the most common intradural extramedullary spinal tumors; however, differentiating between them using magnetic resonance imaging (MRI) is a frequent challenge. In this study, we aimed to investigate the use of the contrast ratio (CR) as a quantitative MRI method in the differentiation of schwannomas and meningiomas. We analyzed the data of patients with intradural extramedullary spinal tumors who underwent surgery and were diagnosed with either schwannomas or meningiomas by histopathological analysis. Regions of interest were set for the entire spinal tumor on T2-weighted sagittal MRI. To obtain the CR values of spinal tumors (CRtumor), we used the signal intensity (SI) values of the tumor (SItumor) and spinal cord (SIcord) according to the following formula: [CRtumor= (SItumor-SIcord)/(SItumor+SIcord)]. The study included 50 patients (23 males and 27 females) with a mean age of 61.5 years old (11-85 years old). Histopathological analysis revealed that 33 and 17 patients were diagnosed with schwannomas and meningiomas, respectively. The mean CR values of the schwannomas and meningiomas were 0.3040±0.1386 and 0.0173±0.1929, respectively. The CR value of the schwannomas was statistically significantly higher than that of meningiomas (P<0.01). The cutoff CR value obtained from the receiver operating characteristic curve was 0.143, with a specificity and sensitivity of 90.9% and 88.2%, respectively. Furthermore, the value for the area under the receiver operating characteristic curve was 0.925 (95% confidence interval: 0.852-0.998). The evaluation of CRs by using MRI to distinguish between schwannomas and meningiomas is a beneficial quantitative tool.

Health-Related Quality of Life and Return to Work after Surgery for Spinal Schwannoma: A Population-Based Cohort Study.

Spinal schwannomas are the second most common primary intradural spinal tumor. This study aimed to assess health-related quality of life (HRQoL) and the frequency of return to work after the surgical treatment of spinal schwannomas. HRQoL was compared to a sample of the general population. Patients operated for spinal schwannomas between 2006 and 2020 were identified in a previous study and those alive at follow-up (171 of 180) were asked to participate. Ninety-four (56%) responded and were included in this study. Data were compared to the Stockholm Public Health Survey 2006, a cross-sectional survey of a representative sample of the general population. An analysis for any potential non-response bias was performed and showed no significant differences between the groups. HRQoL was equal between the spinal schwannoma sample and the general population sample in all but one dimension; men in the spinal schwannoma sample reported more moderate problems in the usual activities dimension than men in the general population (p = 0.020). In the schwannoma sample, there were no significant differences between men and women in either of the dimensions EQ-5Dindex or EQVAS. Before surgery, a total of 71 (76%) were working full-time and after surgery almost all (94%) returned to work, most of them within 3 months of surgery. Eighty-nine (95%) of the patients responded that they would accept the surgery for their spinal schwannoma if asked again today. To conclude, surgical treatment of spinal schwannomas is associated with good HRQoL and with a high frequency of return to work.

Multiple spinal schwannomas of multiple intradural extramedullary tumors: a case report

We describe the case of a 30-year-old male patient who was brought to the hospital's general surgery department with a four-month history of low back pain, numbness, and paraesthesia in both legs, history of weakness in both legs is also present. Magnetic resonance imaging revealed multiple Intradural extramedullary tumors at the C3/C4, D1-D2, D4, and D8 levels- likely nerve sheath tumors (schwannomas). The patient underwent surgery for the removal of tumors. The diagnosis was validated by histopathological examination. Post-operative recovery was uneventful and the patient improved gradually and recovered fully in 10-15 days.

Spinal Schwannoma: A review of the literature

Spinal Schwannomas are rare, benign tumors originating from Schwann cells around peripheral nerves in the spinal canal, with significant clinical implications due to their potential to cause life-threatening neurological impairments. This comprehensive literature review integrates insights from pathology, anatomy, genetics, physiology, and radiology to offer a holistic understanding of spinal schwannomas, covering their clinical characteristics, diagnosis, management, and recent research developments. The aim is to enhance understanding, facilitate accurate diagnosis, develop targeted therapies, and improve clinical management, thereby contributing to better patient care and expanding knowledge on spinal schwannomas.

Clinical outcomes of spinal schwannoma microsurgery

Clinical outcomes of spinal schwannoma microsurgery