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Background: Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas. Their resection may lead to serious morbidity. Incidence of postoperative motor and sensory deficits is unknown, and reconstruction aimed at restoring such deficits is infrequently carried out., Objective: To identify the incidence and risk factors of postoperative morbidity in MPNST as well as the use and outcomes of functional reconstructions in these patients., Methods: Postoperative function and treatment of MPNSTs diagnosed from 1988 to 2019 in 10 cancer centers was obtained. Two models were constructed evaluating factors independently associated with postoperative motor (

Objective and Importance: The present article describes a rare presentation of Type I neurofibromatosis (NFI) involving adjoining intercostal plexiform neurofibromas, as well as the novel use of thoracoscopy to guide surgical resection. This presentation highlights the manner in which NFI may affect selective regions of the body disproportionately through genetic mosaicism., Clinical Presentation: A 40-year-old man had intractable neuropathic anterior right chest wall pain in the distribution of T2 and T3. Magnetic resonance imaging and computed tomography of the chest revealed masses deep to the second and third ribs in the axilla. Biopsy of a subcutaneous nodule in the right chest wall revealed a small neurofibroma. An extensive workup revealed no masses outside the region of the right chest wall and no stigmata of NFI., Intervention: The patient underwent right-sided thoracoscopy for identification of the intrathoracic neurofibromas and placement of spinal needles to localize the anterior and posterior extent of the masses. These landmarks were used to guide a transaxillary approach to third rib resection and nerve-sparing neurofibroma resection., Conclusion: Isolated adjoining intercostal plexiform neurofibroma is a unique presentation of mosaic NFI. Because of its limited penetrance, this variant may present as a regional pain syndrome. Thoracoscopy can be used effectively to guide intercostal nerve sheath tumor resection.

Objective: Plexiform neurofibromas with sizable intraspinal extensions and resultant spinal cord compromise pose challenging management problems, because these lesions may involve multiple nerves and engulf adjacent vascular and visceral structures. In this report, we review our experience with the surgical treatment of these lesions., Methods: Patients were identified by a detailed review of hospital medical records and the database of our multidisciplinary neurofibromatosis clinic. Ten patients had large plexiform neurofibromas that extended intraspinally, producing a combination of myelopathy and radiculopathy. Two patients exhibited single-level intraspinal growth, and eight showed multilevel involvement. Four patients showed bilateral plexiform neurofibromatous growth intraspinally, with "hourglass" compression of the spinal cord. Operative approaches and outcomes were reviewed in detail., Results: Gross total resection of the symptomatic intraspinal tumor component was achieved for nine patients. The management of the extraspinal component was individualized, depending on the pattern and extent of involvement of the surrounding structures. Nine patients experienced complete recovery of neurological function postoperatively; the remaining patient demonstrated significant functional improvement. With a median follow-up period of 4 years, only one patient has developed recurrent intraspinal compression, in this case from tumor involvement by the same plexiform lesion at a lower spinal level. Two patients treated early in the series using standard laminectomy approaches developed significant kyphotic deformities, necessitating subsequent fusion. Based on these initial results, osteoplastic laminotomy techniques were used in the last five cases, allowing anatomic reconstruction of the involved levels; none of these latter patients has developed significant kyphosis, with a median follow-up period of 3 years., Conclusion: Radical resection of intraspinal tumor components in patients with neurofibromatosis 1 and large plexiform neurofibromas can help to preserve excellent neurological function. Technical factors in the management of these lesions are presented.

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.

Although the association between optic glioma and neurofibromatosis is well recognized, few studies have systematically compared the outcomes of patients with optic gliomas and neurofibromatosis and patients with optic gliomas without neurofibromatosis. In the present study, patients with optic gliomas and Type 1 neurofibromatosis (NF-1) were compared with patients with optic gliomas without NF-1, with respect to survival, time to tumor progression, and tumor location. Forty-four patients with optic gliomas who were evaluated between 1949 and 1991 were studied retrospectively. Sixteen of 44 patients (36%) met the National Institutes of Health criteria for NF-1. The medical records of all patients were examined, and letters of inquiry were sent to every living patient to ascertain current health statuses. Death certificates were obtained to determine causes of death. Follow-up averaged 7.2 years (10.2 yr for patients with NF-1, 5.4 yr for patients without NF-1). The 5- and 10-year survival rates for patients with optic gliomas and NF-1 were 93 and 81%, respectively. For those patients with optic gliomas who did not have NF-1, 5- and 10-year survival rates were 83 and 76%, respectively. Seventeen patients experienced tumor progression (5 with NF-1, 12 without NF-1). A difference was observed in the mean time to tumor progression (first relapse) between the two groups (mean time with NF-1, 8.37 yr; without NF-1, 2.39 yr [P < 0.01]). However, no significant difference in overall survival, as evaluated by a log-rank test of the respective Kaplan-Meier survival curves, was observed between the two groups. A significant difference in distribution of tumor location between the group with NF-1 and the group without NF-1 was also noted (Fisher's exact test, P = 0.0338), although the number of patients evaluated in this series was too small to determine whether this difference in tumor location influenced relapse rate. We conclude that optic gliomas in patients with neurofibromatosis have a different distribution of location as opposed to those in patients without neurofibromatosis, and, for first relapse, the presence of neurofibromatosis is a significant favorable factor.

Neurofibromatosis (NF) describes the most commonly inherited disorders affecting the nervous system. These autosomal dominant, neurocutaneous syndromes are associated with multiple tumors of the nervous system, including neurofibromas, schwannomas, meningiomas, and intracranial gliomas. Spinal cord involvement in NF is typically from extramedullary growth of spinal nerve root tumors. Intramedullary spinal cord tumors in NF have been reported as scattered, single cases in literature. However, this association has not been clearly defined as have other nervous system neoplasms that are typically linked with NF. We present a series of nine patients with NF with intramedullary spinal cord tumors who were managed at our institution from 1984 to 1994. The patients' ages ranged from 4 to 31 years. There were seven male patients and two female patients. Three patients had NF-1 (von Recklinghausen's), five patients had NF-2, and one patient had NF, type uncertain. There were three cervicomedullary tumors, two cervical tumors, three cervicothoracic tumors, and one thoracic tumor. A histological examination revealed five ependymomas, three astrocytomas, and one intramedullary schwannoma. Two patients with malignant intramedullary spinal ocrd tumors have died with progressive disease. One other patient has required a subsequent operation for recurrent tumor. The other six patients are doing well, and their prognosis is expected to be related to their systemic disease. We conclude that there is a clinical entity of intramedullary spinal cord tumors associated with NF.

A case of rare segmental neurofibromatosis is presented. Multiple neurofibromas along the right sciatic nerve were found. Other typical manifestations of neurofibromatosis were absent. By microsurgical dissection, it was possible to remove the neurofibromas from the nerve trunks and to preserve all motor and sensory functions. Classification of the different neurofibromatosis types is briefly reviewed. The problems of resection of benign major nerve trunk tumors in neurofibromatosis are discussed.

The authors report a case of spinal subarachnoid hemorrhage caused by neurofibromas of the cauda equina. An examination of the literature revealing 13 similar cases and an analysis of this case showing three neurofibromas of the cauda equina prompted an hypothesis emphasizing the origin of bleeding and the difficulties associated with a correct diagnosis.

A series of 66 spinal cord neurofibromas was analyzed for history, signs, surgical approach, and outcome. The tumors presented primarily with sensory symptoms. Plain films were abnormal in 1/2 of cases and 1/2 had a complete block. They were primarily intradural, and primarily thoracic. A conservative exam system was used for follow-up and 85% with pain had complete relief; 50% with motor loss had normal motor function, and 88% had normal sensation who had prior sensory loss. In comparison to meningiomas, the principal differences were that neurofibromas had an even sex distribution, a lower incidence of cord signs and symptoms, more frequent findings on plain x-rays, and higher cerebrospinal fluid protein. Surgical outcome was similar. Sacrifice of the involved root during removal usually did not produce a deficit. The series is compared with a similar series of meningiomas from the same institution over the same time period.

After incisional biopsy, an opticochiasmatic astrocytoma in a 2-year-old child with neurofibromatosis was seen to regress by computed tomographic scanning. This case illustrates the need for expectant therapy in this entity.

Of the various forms of neurofibromatosis, the best known are peripheral neurofibromatosis (von Recklinghausen's disease) and central neurofibromatosis. There is a rare form called segmental neurofibromatosis, in which the cutaneous and neural changes are limited to one sector of the body and with which there is usually no family history of neurofibromatosis. We report four patients with this disorder and stress the value of repetitive, limited excision of peripheral nerve tumors in their management.