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

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.

Neurosurgery ◽  
2008 ◽  
Vol 62 (2) ◽  
pp. E525-E527 ◽  
Author(s):  
Steven W. Chang ◽  
Pankaj A. Gore ◽  
Peter Nakaji ◽  
Harold L. Rekate

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.


2020 ◽  
Vol 48 (9) ◽  
pp. 030006052095469
Author(s):  
RuiDeng Wang ◽  
Hai Tang

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.


Author(s):  
A.M. Parr ◽  
D. Fewer

AbstractObjective:To report a case of spinal intramedullary blastomycosis causing myelopathy. This is the first published case of a pediatric patient with intramedullary blastomycosis and compromised function.Clinical presentation:An otherwise healthy 13-year-old patient was diagnosed with respiratory North American blastomycosis. She subsequently received a five-month course of itraconazole with presumed resolution of the infection. The patient presented again at 14 years of age with a lumbar myelopathy. Magnetic resonance imaging revealed an intramedullary lesion of 1 cm diameter at the level of T12-L1.Intervention:AT12-L1 laminectomy was performed with a gross total resection of the lesion. Pathological examination and microbiological culture of the specimen was consistent with blastomycosis. Postoperatively, the patient was placed on a five week course of amphotericin B. The patient showed substantial improvement in neurological function.Conclusion:Blastomycosis can present as an isolated intramedullary lesion causing compromised function. It should be considered in the differential diagnosis of a patient with a myelopathy and previously recognized blastomycosis. The prognosis is good with surgical resection.


Neurosurgery ◽  
2011 ◽  
Vol 70 (6) ◽  
pp. E1608-E1612
Author(s):  
Mark J. Winder ◽  
Florin J. Tanase ◽  
Steven Rostad ◽  
Marc R. Mayberg

Abstract BACKGROUND AND IMPORTANCE: Sebaceous neoplasms range from hyperplastic hamartomas to malignant tumors and are most commonly cutaneous lesions. We describe the first reported case of an intracranial sebaceous neoplasm, discussing the differential diagnosis and possible pathogenesis in relation to the current literature. CLINICAL PRESENTATION: A 58-year-old man presented with evolving neck stiffness, facial pain, and progressively worsening diplopia. Magnetic resonance imaging identified a moderate-sized lesion intimately related to the left cavernous sinus, which had extended into the posterior fossa. The patient underwent endoscopic, transnasal subtotal resection of the neoplasm with significant improvement. Histologically, the tumor was identified as a sebaceous neoplasm previously unreported intracranially. Follow-up imaging at 6 months revealed no further recurrence. CONCLUSION: This is the first reported case of an intracranial sebaceous neoplasm. Careful follow-up is required to help elucidate the biology of this tumor in an effort to determine the role of adjuvant therapy.


2006 ◽  
Vol 21 (6) ◽  
pp. 1-4 ◽  
Author(s):  
Ahmet Şengöz ◽  
Erol Taşdemiroğlu ◽  
Halit Togay

✓The authors present a case of clear cell sarcoma (CCS) in which the tumor originated in the S-1 nerve root and had been previously diagnosed as psammomatous melanotic schwannoma (PMS). This is the third case of a spinal nerve root origin for CCS reported in the English-language literature. The similar histogenesis of CCS and malignant melanoma supports the hypothesis that biological agents or immunotherapy are potentially important areas of investigation. The patient underwent S1–3 laminectomy and gross-total resection of the mass lesion. The border of the resection was extended 1 cm distal to the tumor margin. The postoperative period was uneventful. The new histopathological diagnosis was CCS (malignant melanoma of soft tissue). Despite total resection, the patient returned with disseminated disease at the 18-month follow-up visit. His follow-up magnetic resonance image of the lumbar spine revealed sacral L5–S3 involvement of the vertebral bodies along with disseminated cauda equina seeding. A CCS originating from peripheral nerves is quite rare. The histopathological and immunohistochemical appearance of CCSs resembles those of PMSs. Surgery should be the first choice of treatment.


2007 ◽  
Vol 65 (4a) ◽  
pp. 1000-1006 ◽  
Author(s):  
Hamilton Matushita ◽  
Fernando Campos Pinto ◽  
José Píndaro Pereira Plese

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.


2015 ◽  
Vol 14 (2) ◽  
pp. 134-137
Author(s):  
Pedro Luis Bazán

<sec><title>OBJECTIVE:</title><p> Recognizing the importance of SCIWORA in adult age; analyze the usefulness of complementary studies; evaluating therapeutic options; learn about the evolution of the treated patients.</p></sec><sec><title>METHODS:</title><p> A prospective evaluation with a minimum follow-up of 5 years, eight elderly patients with cervical arthrosis and diagnosis of SCIWORA. The Japanese Orthopaedic Association (JOA) scale and ASIA were used on admission and at 6, 12, 24, 36, 48 and 60 months.</p></sec><sec><title>RESULTS:</title><p> The central cord syndrome (CCS) was the neurological condition at admission. One patient recovered after corticosteroid therapy, but later, his disability worsened, and he was operated at 18 months, another patient recovered and a third died. The other patients underwent laminoplasty in the first 72 hours; patients with partial severity condition had a minimum improvement of five points in JAO scale and those with severe conditions died.</p></sec><sec><title>CONCLUSIONS:</title><p> The low-energy trauma can decompensate the relationship between container and content in the spine with asymptomatic arthrosis, and can be devastating to the patient. The diagnosis of intramedullary lesion is made by magnetic resonance imaging. Patients with incomplete deficit undergoing laminoplasty reached at least one level in ASIA score. The potential postoperative complications can be serious.</p></sec>


Neurosurgery ◽  
2009 ◽  
Vol 64 (2) ◽  
pp. E387-E388 ◽  
Author(s):  
Riccardo Ciarpaglini ◽  
Ernesto Pasquini ◽  
Diego Mazzatenta ◽  
Andrea Ambrosini-Spaltro ◽  
Vittorio Sciarretta ◽  
...  

Abstract OBJECTIVE Purely intradural clival chordomas are rare neoplasms, and only a few cases have been reported. The reported cases present features similar to ecchordosis physaliphora, which is a notochordal remnant. We describe these 2 entities and their differential diagnoses, clinical courses, and management. This is the first reported case to be treated using a neuroendoscopic technique. CLINICAL PRESENTATION A 60-year-old man presenting with memory loss underwent magnetic resonance imaging, which revealed an intradural retroclival mass without bone involvement. INTERVENTION The patient underwent an endoscopic transsphenoidal-transclival procedure with subtotal removal of the tumor. Histological findings confirmed the diagnosis of a chordoma. CONCLUSION Even if some parameters exist for a differential diagnosis, ecchordosis physaliphora and intradural chordoma may represent different aspects of the spectrum of the same pathology. Intradural clival chordomas have a better prognosis with respect to classic chordomas. Therefore, in subtotal removal such as that performed in our case, postoperative radiation therapy should be performed only if a regrowth of the remnant is seen during neuroradiological follow-up.


Neurosurgery ◽  
2003 ◽  
Vol 52 (3) ◽  
pp. 706-710 ◽  
Author(s):  
Robert D. Ecker ◽  
John L. Atkinson ◽  
Douglas A. Nichols

Abstract OBJECTIVE AND IMPORTANCE A unique case of delayed ischemic deficit after resection of a large intracranial dermoid is presented. CLINICAL PRESENTATION A 23-year-old woman, 36 hours after the uneventful gross total resection of a large intracranial dermoid cyst, slowly developed a progressive mixed aphasia and right hemiparesis. Magnetic resonance imaging and magnetic resonance angiography revealed small infarcts of the left putamen and temporal-occipital junction and a vasospastic tapering of the left M1 segment. INTERVENTION Angiography confirmed severe vasospastic tapering of the left M1 and M2 segments. Endovascular treatment successfully restored flow in the left superior division. However, the initial attempt at low-pressure dilation of the inferior division led to vessel rupture. Seven months after reoperation for emergent trapping of the M1 segment, the patient made an excellent recovery, with only mild right-hand incoordination. CONCLUSION Ruptured dermoid cysts are a risk for early and delayed cerebral ischemia, and endovascular treatment of dermoid-encased vessels may carry a higher risk for rupture.


2017 ◽  
Vol 36 (04) ◽  
pp. 256-259 ◽  
Author(s):  
Saleh Baeesa ◽  
Abdalrahman Aljameely

AbstractIntramedullary arachnoid cysts of the spinal cord are extremely rare benign lesions of unclear pathogenesis. To our knowledge, only 21 cases were reported in the literature, 10 of which involved the cervical spine. We report the case of a 47-year-old female who presented with a symptomatic spinal intramedullary arachnoid cyst (SIAC). Magnetic resonance imaging scan of the cervical spine demonstrated an intramedullary arachnoid cyst at C3-C5 level. The patient had a cervical laminectomy and cysto-subarachnoid shunt with rapid and excellent clinical recovery and no recurrence at 2-year follow-up.Intramedullary arachnoid cysts should be considered in the differential diagnosis of intramedullary cystic lesions of the spinal cord. Their pathogenesis and natural history are not well defined in the literature. However, a cysto-subarachnoid shunt can be performed with excellent long-term clinical and radiological results.


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