Benign neural sheath tumours of major nerves: characteristics in 119 surgical cases

Peripheral benign nerve sheath tumours are infrequent tumours and affect major nerve trunks. Some authors have indicated a high and prohibitive incidence of neurological injury in resection of these lesions. The authors describe their findings in a retrospective study comprising 119 patients with spontaneous benign nerve sheath tumours of the peripheral nervous system. Seventy-three patients had a schwannoma, 41 had neurofibroma and 5 had plexiform neurofibroma; 25 of the 119 patients suffered from neurofibromatosis. All schwannomas were excised completely and the outcome of patients was 41.0% improved, 6.8% worsened, 52.0% unchanged. Twenty-eight neurofibromas were excised completely and 13 subtotally; the outcome for patients was 19.5% improved, 19.5% worsened and 61% unchanged. All plexiform neurofibromas were removed subtotally and the outcome for patients was 20% improved and 80% unchanged. The best surgical results at average follow-up of 6 years were observed in the patients with schwannoma, the worst in those with plexiform neurofibroma. Our results demonstrated that it is often possible to remove schwannomas as well as neurofibromas with an acceptable risk of injury to the nerve.     

Cutaneous-splanchnic neurofibromatosis

BACKGROUND: Segmental neurofibromatosis (NF V) is ten times less frequent than Recklinghausen disease. Would the risk of visceral involvement in this uncommon form of neurofibromatosis warrant systematic imaging procedures? CASE REPORT: A 31-year-old man consulted for a voluminous plexiform neurofibroma in the left lumbar area. More ventrally, on the left side, there was also a café au lait spot. There were no Lisch nodules. The chest and abdominopelvic computed tomography and magnetic resonance imaging showed intramuscular tumoral extension, two neurofibromas in the 9th intercostal space and a voluminous 5-cm tumor situated in the left adrenal area. After resection pathology examination of the surgical specimen confirmed the diagnosis of ganglioneuroma. DISCUSSION: In this patient, all the neurofibromas and the café of lait spot developed in the territories of the left T10 and adjacent spinal roots. This was also true for the ganglioneuroma which developed on the deep sympathetic ramus to the adrenal gland which originates essentially from roots T8 to T11. This would place this case in the second subgroup of NF V in Roth's classification. Only six other cases have been reported in the literature. Such deep localizations are very likely to be underestimated, raising the problem of their detection and the correct protocol to follow asymptomatic forms, especially to detect disease progression to malignant degeneration which has a poor prognosis. Patients with a NF V should receive genetic counselling with a search for a family history, other signs of neurofibromatosis and Lisch nodules. In young patients, the risk of deep asymptomatic spread underlines the importance of regional computed tomographic or magnetic resonance explorations.     

A case of congenital intraspinal neuroblastoma

A case of congenital intraspinal neuroblastoma with paraplegia in a newborn is described, and a review of previously reported cases is presented. Calcification within the spinal canal and abdominal mass was noted. At age 75 days, the patient underwent a resection of almost the entire retroperitonial tumor. A magnetic resonance scan showed hepatic metastasis, and chemotherapy was changed to a more intensive regimen. The liver metastasis disappeared; however, neurological signs persisted. A literature review of 38 cases showed 26 patients had a laminectomy and 12 patients did not. Treatment with or without laminectomy was associated with a poor prognosis for neurological recovery, suggesting cord compression must occur antenatally, and irreversible damage occurs before birth. Congenital intraspinal neuroblastoma has a relatively good prognosis (survival rate, 91.9%), although complete neurological recovery is highly unusual. Based on these findings, the authors conclude that a laminectomy is not indicated for those patients.     

Surgery of spinal nerve sheath tumors with special reference to neurofibromatosis

OBJECTIVE: We conducted a retrospective study of 87 patients with spinal nerve sheath tumors to determine the overall clinical outcome and specific features in 20 patients affected with neurofibromatosis Types 1 and 2 (NF-1 and NF-2, respectively). METHODS: Case records, operation notes, outpatient files, and radiological examinations were analyzed for all patients treated between September 1977 and August 1994. Additional follow-up data were obtained using outpatient examinations, questionnaires, and telephone calls. RESULTS: During the study period, 128 spinal neuromas (i.e., schwannomas) and 6 neurofibromas in 87 patients were treated. Fifty-seven neuromas were associated with NF-2 in 17 patients and six neurofibromas with NF-1 in 3 patients. Patients with NF-2 and symptomatic neuromas presented with more severe neurological deficits compared to patients without NF-2. Eighty-six percent of the neuromas were removed completely. On average, most preoperative deficits or symptoms improved in patients without NF-2, whereas neurological symptoms remained unchanged in patients with NF-2. Multiple regression analysis revealed that partial removal, surgery of a recurrent tumor, NF-2, and old age predisposed for tumor recurrence. No increased risk of recurrence was observed for patients with NF-1. For patients without NF-2, we observed overall recurrence rates of 10.7% after 5 years and 28.2% after 10 and 15 years, respectively, as determined by Kaplan-Meier analysis. For NF-2, the recurrence rate at 5 years was 39.2%, and all tumors had recurred by 9 years. CONCLUSION: Spinal nerve sheath tumors carry an excellent prognosis in patients with NF-1 and in patients without neurofibromatosis. Symptomatic neuromas occurring in association with NF-2 present with more severe neurological deficits, demonstrate little postoperative improvement, and have a very high recurrence rate.     

Spinal subdural enhancement after suboccipital craniectomy

PURPOSE: To characterize transient intraspinal subdural enhancement (potentially mimicking the subarachnoid spread of tumor) seen on MR images in some children after suboccipital craniectomy for posterior fossa tumor resection. METHODS: Radiologic and medical records of 10 consecutive children who had MR imaging for spinal staging after resection of posterior fossa tumor during a 9-month period were reviewed retrospectively. In addition, one case with similar findings of intraspinal enhancement on spinal staging MR images obtained at another institution was included in the review. RESULTS: Intraspinal enhancement thought to be subdural was seen in four of 10 patients undergoing spinal staging MR imaging 6 to 12 days after surgery. In these four patients, MR studies 50 to 18 days later, without intervening treatment, showed resolution of the abnormal enhancement. A fifth patient (from another institution) with similar intraspinal enhancement underwent CT myelography 4 days later, which showed no subarachnoid lesions. No metastases have developed in any of these five patients during the 2.5- to 3.5-year follow-up period. conclusions: From analysis of the MR appearance and on the basis of prior myelographic experience, we suggest an extraarachnoid, probably subdural, location of this enhancement. Awareness of this phenomenon will reduce the rate of false-positive diagnoses of metastatic disease. Preoperative spinal staging should be considered for patients undergoing suboccipital craniectomy.     

Oral, facial and cranial manifestations of von Recklinghausen neurofibromatosis (NF)

19 patients with neurofibromatosis (NF) type 1 were evaluated retrospectively. Many patients presented not only with orocraniofacial neurofibromas but also with cranial skeletal deformities--involving skullbase, skull, orbit, midface and mandibula--and ophthalmological and dental symptoms, as well as neurological disorders. Malignant transformation of NF was observed in 1 case. Severe manifestations of NF were associated with the plexiform growth type. Due to the nature of NF surgical treatment most often is only palliative.     

Cutaneous manifestations of type 1 neurofibromatosis and their treatment

After recalling the frequency and pathophysiology of type 1 neurofibromatosis, or Von Recklinghausen disease, the authors report the various cutaneous manifestations: café-au-lait spots, lentigines, cutaneous neurofibromas, plexiform neurofibromas. Treatment must be multidisciplinary, as surgery is difficult and often gives disappointing results. The various surgical treatments are considered according to the type and site of the lesions to be treated, with particular emphasis on the treatment plan, which must be discussed with the patient.     

Microsurgery of intramedullary cervical cord tumor

OBJECTIVE: To evaluate whether intramedullary tumor of the cervical spinal cord is amenable to aggressive surgery and to clarify surgical timing and important points for tumor removal. PATIENTS AND METHODS: Fifty-eight patients with intramedullary tumor of the cervical spinal cord were examined by magnetic resonance imaging and treated by microsurgery in Huashan Hospital between May 1988 and December 1994. The results were analysed by F or Chi square tests. RESULTS: Tumors were totally resected in 50 cases (86.2%), subtotally resected in 7 and partially resected in 1. Forty-five (77.6%) patients had their neurological status improved postoperatively. Most patients with moderate neurological deficit can recover remarkably after total tumor removal. Laser surgery is especially helpful for treating lipoma. CONCLUSIONS: Intramedullary tumor of the cervical spinal cord is amenable to total tumor removal. Operation is suitable when a patient presents moderate neurological deficit. Proficient surgical technique and standards for total tumor resection are essential for good results. Preoperative radiotherapy contributes to difficult surgery and poor prognosis, and is not recommended.     

Multiple epidural metastases and development of secondary metastatic spinal cord compression]

The purpose was to examine the frequency of initial multiple epidural metastases, and the occurrence of secondary spinal cord compression (SCC). One hundred and seven patients with SCC from a histologically verified solid tumour were followed prospectively with regular neurological examinations until death. Multiple metastases were demonstrated in 37 (35%). Eight (7.5%) patients developed a second occurrence of SCC all in a new location within the spinal canal. The second occurrence of SCC was found with the same frequency in patients with single metastases (7.1%) compared to patients with multiple metastases (8.1%). The median survival time after the diagnosis of spinal cord compression was 3.4 months, while in the group of patients who developed a second occurrence of SCC the median survival time was 9.2 months. We concluded that only symptomatic epidural metastases should be irradiated, and that all patients treated for SCC should be followed regularly and observed for development of a second SCC.     

CT imaging in adults with neurofibromatosis-1: frequent asymptomatic plexiform lesions

OBJECTIVE: The authors examined the incidence and radiologic characteristics of plexiform neurofibromas in neurofibromatosis-1 (NF-1) to define a cohort at greatest risk for malignant nerve-sheath tumors. BACKGROUND: Plexiform neurofibromas are a frequent complication of NF-1. They can impair function, produce disfigurement, and be the site for the development of malignant nerve-sheath tumors. The incidence and natural history of plexiform neurofibromas is unknown. METHODS: CT imaging of the chest, abdomen, and pelvis was performed in 91 of 125 consecutive adults (age, > or = 16 years) with NF-1. RESULTS: Twenty percent of patients had plexiform neurofibromas of the chest in the paraspinal, mediastinal, or supraclavicular area. Approximately 40% of patients had abnormal abdominal/pelvic scans. The paraspinal, sacral plexus, sciatic notch, and perirectal regions were the most common sites. Most plexiform neurofibromas were asymptomatic. Imaging also revealed a number of tumors, including malignant nerve-sheath tumors, adrenal tumors, carcinoids, and schwannomas. CONCLUSIONS: The frequency of plexiform lesions and other tumors in NF-1 indicates that clinicians should monitor young adults carefully; however, imaging characteristics alone cannot reliably distinguish benign from malignant lesions.     

Penicillin vs. erythromycin in the treatment of diphtheria

PURPOSE: To review the University of Florida experience in treating ependymomas, analyze prognostic factors, and provide treatment recommendations. METHODS AND MATERIALS: Forty-one patients with ependymoma and no metastases outside the central nervous system received postoperative radiotherapy with curative intent between 1966 and 1989. Ten patients had supratentorial lesions, 22 had infratentorial lesions, and 9 had spinal cord lesions. All patients had surgery (stereotactic biopsy, subtotal resection, or gross total resection). Most patients with high-grade lesions received radiotherapy to the craniospinal axis. Low-grade intracranial lesions received more limited treatment. Spinal cord lesions were treated using either partial spine or whole spine fields. RESULTS: Of 32 intracranial tumors, 21 recurred, all at the primary site; no spinal cord tumors recurred. Overall 10-year survival rates were 51% (absolute) and 46% (relapse-free); by tumor site: spinal cord, 100%; infratentorial, 45%; supratentorial, 20% (p = 0.002). On multivariate analysis, tumor site was the only factor that influenced absolute survival (p = 0.0004); other factors evaluated included grade, gender, age, duration of symptoms, resection extent, primary tumor dose, treatment field extent, surgery-to-radiotherapy interval, and days under radiotherapy treatment. CONCLUSIONS: Patients with supratentorial or infratentorial tumors receive irradiation, regardless of grade. Craniospinal-axis fields are used when spinal seeding is radiographically or pathologically evident. Spinal cord tumors are treated using localized fields to the primary site if not completely resected. Failure to control disease at the primary site remains the main impediment to cure.     

MR of spinal cord ganglioglioma

PURPOSE: Our purpose was to describe the MR imaging features in a series of spinal intramedullary gangliogliomas and to compare these findings with the MR features of intramedullary astrocytomas and ependymomas. METHODS: A retrospective analysis was performed of 76 MR examinations in 27 patients with histologically proved spinal ganglioglioma; these were then compared with imaging findings in a representative sample of histologically proved spinal cord astrocytomas and ependymomas. RESULTS: Statistically significant observations regarding spinal gangliogliomas included young age of the patients (mean, 12 years), long tumor length, presence of tumoral cyst, presence of bone erosion and scoliosis, absence of edema, presence of mixed signal intensity on T1-weighted images, and presence of patchy enhancement and cord surface enhancement. A trend (not statistically significant) was noted for holocord involvement and lack of magnetic susceptibility. CONCLUSION: Spinal ganglioglioma can be strongly suspected if MR images reflect the above criteria; however, the ultimate diagnosis still depends on radical resection and appropriate histopathologic investigation.     

Extra osseous tumors of the spine in children and adolescents. Spinal complications

PURPOSE OF THE STUDY: The delay in diagnosis of spinal tumors is not rare. The chief complaint may include pain, walking disability, and spinal or limb deformities. The purpose of our study is to analyze the spinal deformities associated with non osseous intraspinal tumors, to assess the complications of treatment, and to set out a preventive protocol. MATERIALS: We reviewed retrospectively 40 consecutive cases of non osseous intraspinal tumors treated between 1972 and 1991. There were 32 intradural, 2 extradural, and 6 intra and extradural combined tumors. At the first consultation, the age ranged between 4 months and 15 years, and only 16 patients showed neurologic deficit. Spinal deformity existed in 9 patients, 7 of which had no neurologic deficit. All the tumors were treated surgically. Laminectomy was done in 28 cases, and osteoplastic laminotomy in 12 cases. The number of levels included in the laminectomy ranged from 3 to 18. Bilateral arthrectomy at least at one level was undertaken in 15 cases. A postoperative brace was worn in all cases for an average period of 4 months. An adjuvant radiotherapy was undertaken in 12 cases for an incomplete resection. METHODS: The incidence and pattern of spinal deformity was assessed before tumor treatment and ultimately after laminectomy or osteoplastic laminotomy (or laminoplasty). RESULTS: Among the 9 cases with preexisting spinal deformity, the curve magnitude increased after laminectomy in 4. A kyphotic, kyphosoliotic or scoliotic deformity developed in 18 cases after surgery for tumor resection. Among these 18 patients, only one had had an adequate osteoplastic laminotomy. The treatment of spinal deformities was surgical in 12 cases, and done by either posterior or anterior and posterior combined arthrodesis. DISCUSSION: Spinal deformity may be the main complaint of a patient who has intraspinal tumor. Prevention of postlaminectomy spinal deformity is mandatory, and could be done by osteoplastic laminotomy and the use of a brace during a minimum period of 4 to 6 months after surgery. CONCLUSION: Diagnosis of intraspinal tumors in children and adolescents should be done early, and laminoarthrectomy should be replaced by osteoplastic laminotomy.     

Comments on "Double-blind comparison of olanzapine versus risperidone in the treatment of schizophrenia and other psychotic disorders" by Tran and Associates

PURPOSE: An unusual case of infantile myofibromatosis with spinal canal involvement is reported and the literature is reviewed. PATIENT AND METHODS: A female neonate had bladder and bowel dysfunction and paresis of the lower extremities. RESULTS: Intrapelvic and paravertebral masses with extension into the spinal canal were detected by imaging studies. In addition, radiologic examination showed multiple metaphyseal radiolucent lesions of the long bones and pathologic fracture of the left femur. The histopathologic diagnosis of the paravertebral tumor and bone lesions was infantile myofibromatosis. Surgical resection of the paravertebral and intrapelvic masses was performed to improve her neurologic impairments. Paresis of the lower limbs gradually improved, whereas bladder and bowel dysfunction remained unchanged. CONCLUSIONS: Only six cases of infantile myofibromatosis associated with spinal canal involvement have been reported. Three patients with flaccid paresis of extremities and respiratory distress died in the newborn period. The other three patients showed improvement of the paresis. The prognosis of infantile myofibromatosis without visceral complication is generally good, but neurologic impairment may occur at birth if the spinal cord is compressed.     

Extrinsic spinal cord mass lesions

Tumors that arise outside the substance of the spinal cord can be divided into intradural but extramedullary lesions and extradural and extramedullary lesions. Different groups of tumors appear at different sites and behave quite differently. The presentation, however, can be quite similar and is distinct from that of an intraparenchymal spinal cord mass. The prognosis depends on the histopathology. In cases of cord compression, surgery is indicated, but for many of the other tumors, radiation therapy and chemotherapy are appropriate, particularly if the lesion is a metastatic process. Both treatment approaches have obvious inherent problems in children. Extensive laminectomies can induce secondary spinal deformity in children, particularly kyphosis, and adverse effects of both chemotherapy and radiation therapy can cause significant stunting or asymmetry of growth or induce endocrine or other problems. It is critical for the pediatrician to recognize the patient who may have an intraspinal mass, to obtain an MRI scan quickly in those suspected of having an intraspinal mass, and to entertain the diagnosis of a psychiatric illness or social problem only after performing a full diagnostic evaluation. With this approach, many children who previously presented very late with diffusely spread disease may have their condition better controlled and possibly cured.     

Role of hormone substitution treatment following breast cancer

We describe a 20 year old man, who presented with an acute onset of transverse myelopathy evolving over 24 h at T6 spinal level; as yet, an unreported presenting symptom from a midthoracic intraspinal lipoma. The C.S.F. findings suggested a spinal block. MRI, imaging was not practical. Urgent myelography revealed extradural compression at the T5-6 vertebral level. The patient underwent surgical debulking of the tumor which on histopathology was found to be a lipoma, but there was no neurological improvement even at 3 months follow up. We believe that patients with intraspinal lipomas are at high risk of developing irreversible neurological dysfunction.     

A combined impression technique for a partial implant-supported fixed-detachable restoration

INTRODUCTION: The neuroectodermal tumor of bone constitutes a recently isolated neoplasm, which morphologically resembles the peripheral neuroepithelioma of soft tissue. The diagnosis is made by excluding readily classified small round cell neoplasms and then demonstrating the neural origin of the tumor by means of ultrastructural or immunocytochemical studies. CLINICAL CASE: We report a case of a primary neuroectodermal tumor (PNET) with atypical findings (primary cervical location, massive vertebral body infiltration and isolated neurological symptomatology). Total tumor removal was achieved by means of a one-stage three-level laminectomy (partial C5, total C6-C7), C6 somatectomy, and combined anterior and posterior cervical spinal instrumentation and fusion. CONCLUSIONS: Our case presents three peculiar features: 1) isolated myelopathic symptomatology (first case described); 2) primary isolated massive vertebral cervical involvement with intraspinal epidural extension (first case described); 3) one-stage tumor resection, fusion and instrumentation. Since radiation therapy causes tumor shrinkage but is not curative, radical removal is mandatory even with complex instrumentation procedures.     

Significance of elevated IgG anticardiolipin antibody levels in patients with Budd-Chiari syndrome

OBJECT: Congenital spinal hamartomas are defined as tumors of well-differentiated mature elements situated in an abnormal location. In this report, the authors document the clinical and pathological features of spinal hamartomas in 10 patients. METHODS: Ten patients presented with midline dorsal malformations at birth, initially diagnosed as teratomas or myelomeningoceles. The locations of the masses were variable: two were located in the thoracic region, four at the thoracolumbar junction, two in the lumbar region, one at the lumbosacral junction, and one in the sacral region. The results of the neurological examination were normal in nine patients. All but one mass had intact skin and seven had palpable bone components. Neuroimaging studies revealed widening of the spinal canal, heterotopic bone located dorsally in some patients, and varying degrees of involvement of the intraspinal contents. During surgery, six patients were found to have involvement of the spinal cord or cauda equina. The pathological characteristics of the masses included three or more of the following: bone, cartilage, synovial membrane, urinary tract tissue, cyst wall, yellow or brown fat, and nerves. The well-differentiated cellular elements, which formed mature structures, along with the absence of primitive cellular components and neoplastic characteristics are more consistent with a diagnosis of hamartoma than teratoma. CONCLUSIONS: In this series, the authors describe a lesion that is overt on physical examination, yet can have occult spinal canal involvement. Complete neurosurgical evaluation is essential to provide appropriate treatment and prognosis.     

Premorbid hair growth over the trunk and severity of alcohol-related liver disease

OBJECTIVE: To evaluate the clinicopathologic features of malignant pleural effusions secondary to pulmonary adenocarcinoma in patients who have undergone surgical resection of the primary tumor. STUDY DESIGN: Clinical, pathologic and cytologic material from 19 patients who developed malignant pleural effusions after resection of pulmonary adenocarcinoma was reviewed. RESULTS: Malignant effusions developed only in patients with either lymph node or pleural involvement by neoplasm. Time to development of the effusion after resection and overall survival correlated with histologic findings. Malignant effusions in patients who survived > 24 months were secondary to another primary tumor (either breast or a new pulmonary carcinoma). Malignant effusions developed significantly sooner after resection (mean 5.0 +/- 2.0 months, median 5) in patients with lymph nodal metastases than in those with pleural involvement by neoplasm (mean 11.2 +/- 2.5 months, median 12) (Student's t test P = .01, Mann-Whitney U test .04). Nevertheless, survival after resection for patients with lymph node involvement (mean 9.0 +/- 3.6 months, median 8) and those with pleural involvement (mean 12.3 +/- 2.5 months, median 12) was not significantly different. CONCLUSION: Malignant effusions developing in patients more than two years following resection of a pulmonary adenocarcinoma are likely to be secondary to another primary neoplasm. Lymph node and pleural involvement at the time of resection are risk factors for the development of a malignant effusion. Patients with lymph node involvement develop malignant effusions sooner than those with pleural involvement, but the overall survival is not significantly different.     

Lymphoma of the spinal extradural space

Ninety-four patients with lymphoma involving the extradural space with spinal cord compression proven at the time of laminectomy were reviewed. There were about three times as many patients with non-Hodgkin's lymphoma than with Hodgkin's disease. The majority of those with Hodgkin's disease had a proven histologic diagnosis before the onset of the spinal cord compression syndrome, whereas only 15% of those with non-Hodgkin's lymphoma had previously been so diagnosed. Plain roentgenograms of the spine were suggestive of tumor involvement in less than one-third of the patients, whereas myelograms were invariably abnormal. As noted by others, the outlook for functional recovery and extended life expectancy is relatively good for patients with this type of cancer, in contrast to reports in the literature regarding prognosis for patients who have metastatic carcinoma with extradural spinal cord compression.