Intramedullary Location Research Articles

Intramedullary Capillary Hemangioma of the Thoracic Spine: Case Report and Review of the Literature

Capillary hemangiomas are benign vascular neoplasms. When associated with the spine, these growths frequently involve the vertebral body, but rarely have they been reported to occur as intradural lesions, while even more rarely occurring in a true intramedullary location. We report a rare case of an intramedullary capillary hemangioma of the thoracic spinal cord and a review of the literature.

Spinal teratomas: a clinico-pathological study of 27 patients

Spinal cord teratomas are uncommon tumours. The diagnosis is rarely suspected before surgery and final diagnosis is invariably made at histopathological examination. We retrospectively analysed our neuropathology records for the last 20 years and all patients diagnosed as spinal teratomas were retrieved. The clinical and radiological features were noted. Slides were reviewed and the diagnosis was confirmed. During a period of 20 years, we found 27 examples of spinal teratomas. There was a wide age range and 37.3% presented in adulthood. Male predominance was observed both in children as well as adults. The dorso-lumbar location was common in children as well as in adults but an intramedullary location, in association with a myelomeningocele and split cord malformation, was more common in children. Twenty one patients had associated features of spinal dysraphism. Of the six who were not associated with this, five were adult patients. Radiologically mixed solid and cystic morphology, fat signal and areas of calcification were seen in one third of patients. All tumours except one showed benign (mature) components. Interestingly, two lesions contained mature renal tissue: one exhibited pulmonary differentiation and one had Wilm's tumour as a component of the teratoma. In five patients the initial sections showed epidermis with skin adenexal structures and a diagnosis of dermoid cyst was suspected but serial sectioning revealed other teratomatous components. All patients were treated with surgical excision. Although there are no specific features on imaging, mixed solid and cystic morphology, fat signal and areas of calcification are helpful in the pre-operative diagnosis. Most of these lesions are diagnosed on histopathological examination after surgery and therefore thorough sampling and serial sectioning are mandatory to identify all components of the teratoma in order to avoid misdiagnosis.

Pulmonary meningioma and neurinoma associated with multiple CNS tumours in a patient with neurofibromatosis type 2

Objective Neurofibromatosis type 2 (NF2) is a common neurocutaneous disorder that exhibits an autosomal dominant inheritance, with a mutation at chromosome 22q12.2. Two forms can be distinguished: the Wishart-phenotype with an early and aggressive course and the Feiling–Gardner-phenotype with a less dramatic presentation. In general, patients present bilateral vestibular schwannomas, meningiomas and neurinomas of the central and peripheral nervous system as well as neurofibromas and gliomas. There is no reported case of pulmonary meningiomas and neurinomas associated with NF2 until now. Patient and methods Here, we present a 16-year-old girl with NF-2 associated to CNS and pulmonary tumours and we discuss the case in the backlight of the literature. Results The reported patient presented a de novo NF2 germline mutation (R341X) and displayed the Wishart-type of NF-2 since she is 11 years old, with a huge anaplastic biparietal falx meningioma and a tentorium meningioma and a tumour-associated parietal mass as well as hypacusis starting at the infant age of 3 years. Multiple cranial and spinal tumours with extra- and intramedullary localization were also found. Moreover, recurrent pulmonary tumours developed and were classified as benign meningiomas and a single neurinoma. No direct evidence concerning a relationship between the pulmonary and cerebral tumours could be drawn. Conclusion This rare case extends our knowledge of NF2 and also raises interesting questions about the pathogenesis of meningiomas outside the CNS.

Intramedullary arachnoid cyst: 3-year follow up after bilateral dorsal root entry zone myelotomy

Spinal arachnoid cysts are relatively uncommon an intramedullary location is believed to be extremely rare. A 35 year old woman, admitted with progressive weakness in the lower limbs, was diagnosed as having a thoracic intramedullary arachnoid cyst. After bilateral dorsal root entry zone myelotomy procedures and wide fenestration was performed, there was a dramatic and immediate recovery. This is the first intramedullary arachnoid cyst reported to be treated by this approach and the long term outcome discussed.

Is total excision of spinal neurenteric cysts possible?

Spinal neurenteric cysts are intradural cystic lesions. These represent a part of the spectrum of developmental anomalies. These are rare lesions. Most of the data in literature is review of case reports. This made us evaluate our results of 23 cases and review the literature. This is a retrospective study of 23 patients managed at our institute over 20 years. The slides were retrieved and histopathological features studied. Twenty patients were less than 30 years old and 21 were males. Follow-up was available for 21 patients with mean duration of 71 months (range 2 months to 23 years). The typical presentation was backache with progressive neurological deficits pertaining to the level. Atypical presentations as acute onset, recurrent episodic events and aseptic meningitis were also noted. The cervicothoracic region was the most common site. 16 patients had cyst in intradural extramedullary plane and seven had intramedullary location. Associated vertebral anomalies as hemivertebrae, Klippel – Feil and spina bifida were noted in seven patients. There were two histological types of cysts with no correlation between the type of cysts and associated vertebral anomalies and extent of outcome. Partial excision though had higher risk of recurrence, was not associated with poorer outcome. Dorsal approach is an acceptable route with reasonably good results for this lesion. Spinal neurenteric cysts present at younger age with varied clinical presentations. These are commonly located intradurally ventral to the cord. Histological types have no effect on the outcome. Total excision is the choice of treatment. However, partial excision is a feasible option in intramedullary lesions and when significant adhesions occur. Although associated with higher risk of recurrence, the outcome is still good in these patients on re-excision.

Primary intramedullary primitive neuroectodermal tumor (PNET) – case report and review of the literature

Spinal primitive neuroectodermal tumors (PNET) are very rare tumors, and intramedullary localization is even less common. Indeed, amongst the 23 cases of the spinal PNET described in the literature, only eight cases had an intramedullary localization. Following the WHO 2000 classification, PNETs have been considered embryonal tumors composed of undifferentiated neuroepithelial cells with a capacity of differentiation into different cellular lines, such as astrocytic, ependymal, melanotic and muscular. They have been considered to arise from a neoplastic transformation of primitive neuroepithelial cells, thereby making their presence possible in any part of the central nervous system. The optimal treatment for these malignant tumors is not yet clear, although, over the years, radiotherapy has been considered the best treatment for spinal PNETs. The described case is that of a 38-year-old man with a primary intra-extramedullary PNET, treated by laminectomy, open biopsy and chemotherapy. The patient, 18 months after the onset of his symptomatology, died without cerebral tumor involvement.

A novel intramedullary spinal cord tumor model: functional, radiological, and histopathological characterization

Survival rates for high-grade intramedullary spinal cord tumors (IMSCTs) are approximately 30%, and optimal therapy has yet to be determined. Development of a satisfactory intramedullary tumor model is necessary for testing new therapeutic paradigms that may prolong survival. The authors report the technique, functional progression, radiological appearance, and histopathological features of a novel intramedullary model in rabbits. Ten New Zealand white rabbits were randomized to receive an intramedullary injection of either 25 microl of VX2 carcinoma cells (500,000 cells; six rabbits) or 25 microl of medium (Dulbecco modified Eagle medium; four rabbits) into the midthoracic spinal cord. Postoperatively the rabbits were evaluated twice daily for neurological deficits. High-resolution magnetic resonance (MR) images were acquired preoperatively and weekly postoperatively until onset of paraparesis, at which point the animals were killed, and the midthoracic spines were processed for histopathological examination. The VX2-carcinoma cells grew in 100% of animals injected and resulted in a statistically significant mean onset of paraparesis of 16.8 +/- 1.7 days (p = 0.0035, log-rank test), compared with animals in the control group in which neurological deficits were absent by Day 45. Contrast-enhanced T1-weighted MR imaging best demonstrated space-occupying intramedullary lesions and histopathological findings confirmed the intramedullary location of the tumor. Animals in the control group exhibited no functional, radiographic, or pathological signs of tumor. Progression to paraparesis was consistent in all the VX2-injected animals, with predictable onset of paraparesis occurring approximately 17 days postinjection. Histopathological and radiological characteristics of the VX2 intramedullary tumor are comparable with those of aggressive primary human IMSCTs. Establishment of this novel animal tumor model will facilitate the testing of new therapeutic paradigms for the treatment of IMSCTs.

Schwannome intramédullaire: À propos d’un cas

The intramedullary localization of schwannoma is rare, corresponding to 0.3% of all intraspinal tumors. We report a case of intramedullary schwannoma without symptoms suggestive of neurofibromatosis. This patient presented with symptoms of spinal compression. Total removal of the tumor was achieved. The literature is reviewed about of this rare localization of schwannoma.

Les tuberculomes intramédullaires: À propos de 5 cas

Tuberculosis of the central nervous system is uncommon. The intramedullary localization is exceptional. We report five cases of intramedullary tuberculosis observed in four women and one man with a mean age of 43 years. Two patients had a prior history of tuberculosis. Spinal cord compression was found clinically in all cases. The spinal MRI visualized the tuberculoma in all patients; two had a double tumor. Complete removal of the tuberculoma was possible in only one patient. Outcome was stationary for four patients.

Reconstruction of the Laminar Roof With Miniplates for a Posterior Approach in Intraspinal Surgery: Technical Considerations and Critical Evaluation of Follow-up Results

A retrospective clinical follow-up study of patients who underwent intraspinal surgery with reconstruction of the laminar roof using titanium miniplates. To evaluate the surgical technique of laminar roof reconstruction and to analyze follow-up results with regard to bony healing of the laminae and the development of spinal deformities. The reconstruction of the laminar roof was initially proposed to overcome adverse effects associated with extensive cervical laminectomy in children. Because technical evolutions such as the use of an air drill and miniplates have facilitated the procedure, it has gained more widespread use as a posterior approach for intraspinal surgery. Thus, with a sufficient number of patients treated and a longer period of follow up, it seems reasonable to critically evaluate the technique and its suitability as a standard approach for intraspinal surgery. The surgical procedures of 79 patient who underwent intraspinal surgery with osteotomy and reconstruction of a total of 323 spinal laminae using an air drill and miniplates were analyzed. In 59 patients, data of a complete clinical and radiologic follow-up examination were evaluated. Plain radiographs and computed tomography scans were analyzed for bony healing of the laminae and spinal alignment. Minor complications such as cerebrospinal fluid collections and disturbed wound healing occurred within normal ranges. The was no case of dural, nerve root, or spinal cord injury attributable to laminotomy or laminar reconstruction. Eight (14.3%) patients complained of moderate to severe local pain at the time of follow-up examination and 8 patients stated impaired mobility of their spine at the surgical site. Bony healing was confirmed radiologically in 86.1% of the laminae. In 12 patients, a preexisting spinal deformity worsened after surgery and five patients demonstrated a new spinal malalignment. No patient required additional surgery because of progressive spinal deformity. Intramedullary location of the lesion and cervical location of surgery were significantly associated with the development of spinal malalignment, whereas incomplete bony healing of the laminae was not. The reconstruction of the laminar roof using the technique described is safe, well suitable to serve as a standard posterior approach to intraspinal pathologies, and offers distinct advantages over laminectomy. However, some patients, particularly those with intramedullary cervical lesions, could develop spinal malalignment after surgery despite reconstruction of the laminar roof and sufficient bony healing of the laminae.

Spinal meningeal melanocytoma

The authors report on two patients with spinal meningeal melanocytoma and review the literature on this lesion. One case is particularly interesting because of the lesion's thoracic intramedullary localization. Meningeal melanocytoma is a benign but locally aggressive lesion and is very rarely associated with spinal localizations. This patient presented with paraparesis. Clinical and radiological examinations suggested the possibility of an intramedullary solid tumor. Thoracic laminectomy, posterior myelotomy, and tumor resection were performed; the mass was totally removed. The patient suffered no additional neurological deficit. During a 3-year follow-up period in which radiotherapy was not performed, the lesion did not recur. Total excision of the tumor is the best therapeutic option.

Solitary Thoracic Intramedullary Schwannoma Report of Two Cases

Schwannomas are the most common tumors of the spinal cord. Nevertheless, sporadic intramedullary location of this entity is a very rare condition. We present two cases of solitary thoracic intramedullary schwannoma, located at the T6 and T10 levels respectively, in patients without evidence of Von Recklinghausen's disease. Two males, 71 and 51 years old respectively, presented with signs and symptoms of progressive thoracic myelopathy. In both cases, magnetic resonance imaging disclosed a spinal cord tumor, located at T6 and T10 levels respectively. Both patients were treated surgically. Patient 1 underwent an operation via a posterolateral extrapleural approach. The second patient was submitted to surgical resection of the tumor via a posterior approach. In both patients, total excision of an intramedullary lesion was achieved and histological and immunohistochemical findings were consistent with a typical schwannoma. Postoperative magnetic resonance imaging demonstrated no evidence of residual tumor in either case. The first patient is currently symptom-free and neurologically intact, almost five years after surgery. About one year after operation, patient 2 presents nearly complete resolution of his motor and sensory disturbances. Solitary intramedullary schwannomas are rare. Controversy remains regarding the origin of these tumors. In the majority of the cases, intramedullary schwannomas are benign, non-infiltrating tumors, hence amenable to aggressive surgery. Radical excision remains the gold standard in the treatment of such lesions.

Intramedullary Neurenteric Cyst with a False Mural Nodule: Case Report

OBJECTIVE AND IMPORTANCE Spinal neurenteric cysts are rare congenital lesions that may occur either alone or in the context of a complex malformative disorder including typical vertebral and cutaneous abnormalities. The interest of the case of a spinal neurenteric cyst described here lies in its rare intramedullary location and in the false mural nodule image on the preoperative magnetic resonance imaging scan. A further distinctive feature is the association with a cleft spinal cord. CLINICAL PRESENTATION A 28-year-old woman presented with a 2-year history of progressive paraparesis and urinary retention. A magnetic resonance imaging study disclosed a T8–T9 intramedullary cystic lesion with a mural nodulelike formation on the posterior face. INTERVENTION A posterior midline myelotomy exposed a cystic lesion that had translucent walls and contained a milky fluid. No mural nodules were found. Once the cyst had been emptied, a collateral finding was a cleft that was clearly observed in the anterior spinal cord. The histological diagnosis was a neurenteric cyst. Retrospectively, the nodular lesion found on the preoperative scan was attributed to mucinous clots deposited at the bottom of the cyst. CONCLUSION This case report demonstrates that neurenteric cysts can vary widely in radiological appearance, depending on the contents of the cyst. These differences become especially important if the associated stigmata are lacking and the preoperative diagnosis rests on magnetic resonance imaging scan appearance alone.

Ganglioglioma da medula espinhal: relato de caso

Gangliogliomas are rare tumors of the CNS, representing only 2.7-3.8% of primary tumors of the CNS, and the intramedullary location accounts 7.6-14.3% of cases. The main goal of treatment is the total resection, preserving as much as possible the patients neurological function. Adjuvant therapy as radiotherapy is reserved to cases of progression of disease after surgery or in such lesion with more aggressive biological behavior. In this article we report the case of a patient with a intramedullary ganglioglioma involving spinal levels T5 to T10, who was operated in our service, and we review the literature analyzing various aspects, including the modalities of treatment which can be used in this kind of lesion.

Intramedullary spinal teratoma with spina bifida.

Spinal intramedullary teratoma is a rare tumor. We report a case of intramedullary teratoma of the conus medullaris with spina bifida. A 5-year-old boy was admitted to our hospital for removal of a lumbosacral tumor sited in an intramedullary location at the conus medullaris, which was totally resected with neuroendoscopic assistance. The pathological diagnosis was mature teratoma consisting of three germ cell layers. The pathogenesis of spinal intramedullary teratoma is discussed with special reference to spina bifida.

Hemangioblastomas and other uncommon intramedullary tumors.

Astrocytoma and ependymoma make up 90% of intramedullary tumors between them. However, a host of less common tumors form the remaining 10%: these include hemangioblastomas, subependymomas, gangliogliomas and other neuronal variants, metastases from extraneural cancers, and a host of other lesions that typically occur intracranially but which present on rare occasions in the intramedullary location. Most neurosurgeons will encounter the unusual tumors of the spinal cord described in this review only a few times during their professional careers, but it is nevertheless important to recognize the distinct radiological and intraoperative features of those for which significant series of patients have been accumulated. Metastases and germinomas aside, the other neoplasms described here are relatively benign in their clinical and histological behavior, and can be meaningfully resected by careful microsurgical technique.

Primary leiomyosarcoma of bone: a clinicopathologic, immunohistochemical, and ultrastructural study of 33 patients and a literature review.

Leiomyosarcoma of bone is a rare tumor in an unusual location. Previous analysis of this entity mostly involved small numbers of cases with limited follow-up. Thirty-three patients with leiomyosarcoma of bone between 1977 and 1996 were studied, and the histologic appearance and grade were correlated with subsequent treatment and clinical behavior. To be included in this study the tumor had to be intraosseous, with other primary sites of origin clinically excluded. Also, most of the sarcomatous tissue (> or =70%) had to be of intramedullary location with only limited extraosseous extension. The patient's age at diagnosis ranged from 13 to 77 years (average 44.4). The gender distribution was equal. The long bones were preferentially affected (64%), with the lower extremity, around the knee joint, predominantly involved. Five patients (15%) developed postradiation leiomyosarcomas. The histologic analysis showed that the osseous leiomyosarcomas are most commonly of the classic type, followed by the epithelioid, myxoid, and pleomorphic variants. Immunoreactivity for smooth muscle markers (smooth muscle actin, common muscle actin, desmin) was positive in all tumors, and ultrastructural confirmation was obtained in 21% of cases. All sarcomas were histologically graded, which accurately reflected the subsequent prognosis. Seventy-five percent of the lesions were high-grade and the rest low-grade. The histologic grade of the tumors correlated with both the recurrence as well as the metastatic rates and together with the clinicopathologic stage of disease represented the cornerstone on which prudent therapy should be based.

Cavernous angiomas of the spinal cord clinical presentation, surgical strategy, and postoperative results.

Nine consecutive cases of surgically treated spinal cavernous angiomas are presented. Our series consists of 6 men and 3 women with the following intramedullary spinal location of the cavernomas: 4 cervical, 4 thoracic and 1 thoraco-lumbar. All 9 patients were symptomatic with signs of myelopathy and senorimotor deficits corresponding to the level of the lesion. Six patients underwent laminectomy and in three patients a hemilaminectomy was performed to approach the lesion. A complete resection of the cavernoma was achieved in each case. Five patients showed transient neurological deterioration, in three cases the neurological status remained unchanged, and one patient experienced a slight improvement of symptoms during the early postoperative period. At follow-up examination (mean 14 months postoperative), a clear improvement of the clinical signs was demonstrable in 6 patients, and a complete resolution of the pre-existing symptoms and signs was achieved in two individuals. In one case the clinical state remained unchanged. It is concluded that microsurgical resection is the treatment of choice in cavernomas of the spinal cord.

Intramedullary Epidermoid Cyst Evaluated by Computed Tomographic Scan and Magnetic Resonance Imaging: Case Report

Abstract The authors have treated a 20-year-old man with a dorsal intramedullary epidermoid cyst in whom magnetic resonance imaging was performed both before evacuation of the cyst and 3 months later. Intraspinal epidermoid tumors are rare, and the intramedullary location is quite uncommon. To our knowledge, this is the first description of magnetic resonance imaging of an intramedullary epidermoid cyst. The frequency, possibilities, and limits of surgical treatment of such intraspinal benign tumors are reviewed.

Spontaneous spinal haematomas.

A series of 10 surgically treated "spontaneous" spinal haematomas (7 cases with epidural, 3 with intramedullary location), is presented. Symptomatology was rarely acute. Clinical onset was mostly dominated by spinal or radicular pain, followed by severe motor deficit. Surgical outcome was satisfactory in the majority of cases. Age, duration of symptoms, haematoma site and size appeared to have no influence on final outcome. This was significantly correlated only with the preoperative neurological condition.