Melanotic nerve sheath tumors

1974 ◽  
Vol 41 (2) ◽  
pp. 187-192 ◽  
Author(s):  
Thaddeus I. Mandybur
Keyword(s):  
Normal Skin ◽  
Cord Compression ◽  
Radicular Pain ◽  
Nerve Sheath Tumor ◽  
Melanin Production ◽  
Content Type ◽  
Histological Features ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
History Of

✓ A solitary intraspinal, extradural melanotic tumor was subtotally resected in a 59-year-old man who had a 17-year history of radicular pain and later evidence of progressive spinal cord compression. The neoplasm revealed the histological features of a benign nerve sheath tumor with massive but uneven melanin production. In electron micrographs the tumor cells contained masses of melanosomes of the type seen in normal skin melanocytes and in B type melanocarcinomas. In the 16-month postoperative period there has been only minimal radiological indication of local recurrence and no metastases.

Primary malignant peripheral nerve sheath tumor of the spine with acute hydrocephalus: a rare clinical entity

2014 ◽  
Vol 21 (3) ◽  
pp. 367-371 ◽  
Author(s):  
Yaxiong Li ◽  
Fengshi Fan ◽  
Jianguo Xu ◽  
Jie An ◽  
Weining Zhang
Keyword(s):  
Peripheral Nerve ◽  
English Language ◽  
Standard Of Care ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
History Of ◽  
Brain Ct Scan ◽  
The Right

Primary malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare in patients without a history of neurofibromatosis; only 18 cases have been reported in the English-language literature to this point. The authors report their experience with 1 new case of a primary MPNST. A 33-year-old woman presented with low-back pain radiating to the right calf that progressed over 1 year. Magnetic resonance imaging of the spine revealed an intradural extramedullary lesion at the T12–L1 level. The patient was diagnosed with primary MPNST, underwent two surgical excisions and radiation therapy, and developed leptomeningeal metastases as well as brain metastases. The patient revisited the emergency room with sudden loss of consciousness. A brain CT scan displayed bilateral lateral ventricle enlargement, for which a ventriculoperitoneal shunt was inserted. These symptoms have not been described in any previous report. Primary spinal MPNST is an exceedingly rare entity, and the overall prognosis is very poor. To the authors' knowledge, no standard of care for primary spinal MPNSTs has yet been established. All 19 cases of primary spinal MPNSTs are reviewed, and the authors discuss their clinical, radiological, and therapeutic features and outcomes.

Spinal malignant nerve-sheath tumor or cellular schwannoma? A striking difference in prognosis

1993 ◽  
Vol 79 (4) ◽  
pp. 528-532 ◽  
Author(s):  
Matti T. Seppälä ◽  
Matti J. J. Haltia
Keyword(s):  
Malignant Tumors ◽  
Spinal Tumors ◽  
Striking Difference ◽  
Malignant Neoplasms ◽  
Nerve Sheath Tumor ◽  
Content Type ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Cellular Schwannoma ◽  
S 100

✓ Cellular schwannoma, a recently delineated entity, has a histological appearance mimicking that of malignant neoplasms. The aim of this study was to determine the outcome for patients treated for a spinal cellular schwannoma or malignant nerve-sheath tumor. A histological re-examination was conducted of 283 spinal tumors, considered to originate from a nerve root, that were treated in the Department of Neurosurgery between 1953 and 1985. After re-examination, 50 of these were determined to be other tumors or nonneoplastic lesions. The review yielded eight cellular schwannomas and six malignant nerve-sheath tumors out of 233 of nerve-sheath origin. Immunohistochemical staining with a commercially available polyclonal antibody against S-100 protein was positive in all cases of cellular schwannoma, but negative for the malignant tumors. Clinical outcome was favorable for patients with cellular schwannomas, but uniformly poor for those with the malignant tumors.

scholarly journals 1534 A rare operation: resection of a complex malignant peripheral nerve sheath tumour deemed inoperable by a national centre

2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
M. R Chowdhury ◽  
A Goel ◽  
P Sinha ◽  
R Chave-Cox ◽  
D Pal
Keyword(s):  
Peripheral Nerve ◽  
Cord Compression ◽  
Nerve Sheath Tumor ◽  
Rare Type ◽  
The Road ◽  
National Centre ◽  
Nerve Sheath ◽  
History Of ◽  
On The Road ◽  
Middle Aged Adults

Abstract We present this rare case which was led by spinal surgeons at Leeds after being deemed not suitable for operative input by a national centre. A 54-year-old lady who presented with 2-month history of worsening left flank pain on a background of four years. She was otherwise fit and well. CT confirmed aggressive left-sided paraspinal tumour with epidural encroachment, extending from T8 to T10 vertebral levels. Its deep surface was well-defined and extending superficially through the chest wall with destruction of ribs. The mass grew in size in course of days requiring urgent attention due to risk of cord compression. Biopsy proved high grade MPNST. Malignant peripheral nerve sheath tumor (MPNST) is a rare type of sarcoma, 5-10% of sarcoma cases. MPNST is most common in young adults and middle-aged adults. About 25% to 50% of people with MPNST have NF1. 8% to 13% of people with NF1 will get MPNST in their lifetime. An MDT approach involving spinal, thoracic, vascular and plastic surgeons opined it requiring extensive surgery due to its complexity. It was performed in stages – embolisation then aortic stent placement followed by final resection of the tumour with chest well reconstruction using an LD flap. A multidisciplinary and multispecialty approach has led to a positive prognosis for this patient who is on the road to recovery.

Extrathecal intraradicular nerve sheath tumor

2005 ◽  
Vol 3 (1) ◽  
pp. 1-11 ◽  
Author(s):  
Paolo Celli ◽  
Giuseppe Trillò ◽  
Luigi Ferrante
Keyword(s):  
Nerve Root ◽  
Spinal Nerve ◽  
Nerve Sheath Tumor ◽  
En Bloc ◽  
Content Type ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Radical Removal ◽  
Fine Print

Object. The purpose of this study was to analyze the clinical profile of patients harboring extrathecal and intraradicular nerve sheath tumors (NSTs), located inside the sleeve of an extrathecal nerve root and very often within the proximal portion of the spinal nerve, and to evaluate the incidence of long-term dysfunction of the tumor-affected roots if resected. These tumors have not received particular attention in the literature. Methods. A single-institution series of 16 patients who had undergone surgery for intraradicular NSTs during a 50-year period was selected retrospectively. Data pertaining to clinical features, tumor characteristics, and results of surgery were analyzed. Conclusions. Extrathecal and intraradicular neurofibromas or schwannomas more frequently affect the lumbar and S-1 nerve roots, often producing root pain only. Selective en bloc enucleation sparing at least part of the motor rootlets is possible for small schwannomas of the extrathecal—preganglion segment of the radix, whereas total resection of the affected root is generally required for radical removal of neurofibromas and large schwannomas. In the authors' experience, neither deafferentation pain nor severe radicular weakness occurs after division of the nerve root harboring the tumor.

scholarly journals Toward Understanding the Mechanisms of Malignant Peripheral Nerve Sheath Tumor Development

2021 ◽  
Vol 22 (16) ◽  
pp. 8620
Author(s):  
Teddy Mohamad ◽  
Camille Plante ◽  
Jean-Philippe Brosseau
Keyword(s):  
Peripheral Nerve ◽  
Tumor Development ◽  
Neurofibromatosis Type ◽  
Type I ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Research Perspectives ◽  
Nerve Sheath ◽  
History Of

Malignant peripheral nerve sheath tumors (MPNSTs) originate from the neural crest lineage and are associated with the neurofibromatosis type I syndrome. MPNST is an unmet clinical need. In this review article, we summarize the knowledge and discuss research perspectives related to (1) the natural history of MPNST development; (2) the mouse models recapitulating the progression from precursor lesions to MPNST; (3) the role of the tumor microenvironment in MPNST development, and (4) the signaling pathways linked to MPNST development.

scholarly journals Metastatic small cell carcinoma of the lung with prominent spindle cell morphology and hemangiopericytoma-like vascular pattern: A sarcoma mimicker

2020 ◽  
Vol 8 ◽  
pp. 2050313X2098117
Author(s):  
Esra Nsour ◽  
Ali Al Khader ◽  
Bushra Al-Tarawneh
Keyword(s):  
Cell Carcinoma ◽  
Small Cell Carcinoma ◽  
Spindle Cell ◽  
Small Cell ◽  
Nerve Sheath Tumor ◽  
Vascular Pattern ◽  
Nerve Sheath ◽  
History Of ◽  
Wall Mass ◽  
Aggressive Clinical Behavior

Small cell carcinoma is a malignant neuroendocrine tumor with aggressive clinical behavior. Histologically, the tumor is characterized by the proliferation of small, round, blue cells. Here, we present the case of a 50-year-old man with a 1-month history of enlarging chest wall mass. Microscopic examination of the lesion revealed a highly cellular neoplasm composed of closely packed, atypical spindle cells with scant cytoplasm, inconspicuous nucleoli, and brisk mitotic activity. The hemangiopericytoma-like vascular pattern was prominent. Areas showing a fibrosarcoma-like fascicular pattern were also observed. The tumor was immunohistochemically positive for TTF1, synaptophysin, and chromogranin, confirming small cell carcinoma. Further investigations revealed a lung origin and widespread metastases. The tumor in this case closely mimicked synovial sarcoma or malignant peripheral nerve sheath tumor. Small cell carcinoma demonstrates a hemangiopericytoma-like pattern that can mimic sarcoma histologically. This is a serious pitfall that can significantly affect the speed of diagnosis and management.

Malignant Nerve Sheath Tumor of the Right Cerebral Peduncle: Case Report

Neurosurgery ◽  
2004 ◽  
Vol 54 (2) ◽  
pp. 500-504 ◽  
Author(s):  
Patrick Beauchesne ◽  
Jean-François Mosnier ◽  
Thierry Schmitt ◽  
Jacques Brunon
Keyword(s):  
Stereotactic Biopsy ◽  
Clinical Presentation ◽  
Positive Response ◽  
Cerebral Peduncle ◽  
Nerve Sheath Tumor ◽  
Chemotherapy Treatment ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
The Right ◽  
First Time

Abstract OBJECTIVE AND IMPORTANCE Schwannomas occurring in the neuraxis are very rare. Usually, these tumors are benign. Primary malignant intracerebral nerve sheath tumors are extremely rare, with only five documented cases in the international literature. We report one case of a primary malignant intracerebral nerve sheath tumor occurring in the right cerebral peduncle of a 35-year-old man. CLINICAL PRESENTATION Magnetic resonance imaging revealed a heterogeneous peripherally enhancing mass of the right cerebral peduncle, surrounded by a small edema. INTERVENTION Unlike the five cases previously reported, this is the first time a stereotactic biopsy has been performed, and this is the only patient who responded to cranial radiation therapy for approximately 2 years. When the tumor recurred, a systemic chemotherapy treatment was prescribed. No positive response was seen, and the patient died 29 months after the initial diagnosis. CONCLUSION An accurate diagnosis and planned aggressive treatment seem to be the key elements in the management of the disease.

Nerve sheath tumors involving the sacrum

2003 ◽  
Vol 15 (2) ◽  
pp. 1-6 ◽  
Author(s):  
Paul Klimo ◽  
Ganesh Rao ◽  
Richard H. Schmidt ◽  
Meic H. Schmidt
Keyword(s):  
Type I ◽  
Nerve Sheath Tumor ◽  
Delayed Presentation ◽  
Type Ii ◽  
Complete Excision ◽  
Posterior Surgery ◽  
Sacral Nerve ◽  
Type Iii ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath

Nerve sheath tumors that involve the sacrum are rare. Delayed presentation is common because of their slow-growing nature, the permissive surrounding anatomical environment, and nonspecific symptoms. Consequently, these tumors are usually of considerable size at the time of diagnosis. The authors discuss a case of a sacral nerve sheath tumor. They also propose a classification scheme for these tumors based on their location with respect to the sacrum into three types (Types I–III). Type I tumors are confined to the sacrum; Type II originate within the sacrum but then locally metastasize through the anterior and posterior sacral walls into the presacral and subcutaneous spaces, respectively; and Type III are located primarily in the presacral/retroperitoneal area. The overwhelming majority of sacral nerve sheath tumors are schwannomas. Neurofibromas and malignant nerve sheath tumors are exceedingly rare. Regardless of their histological features, the goal of treatment is complete excision. Adjuvant radiotherapy may be used in patients in whom resection was subtotal. Approaches to the sacrum can generally be classified as anterior or posterior. Type I tumors may be resected via a posterior approach alone, Type III may require an anterior approach, and Type II tumors usually require combined anterior–posterior surgery.

scholarly journals Hybrid nerve sheath tumor in the orbit: A case report and review of literature

2019 ◽  
Vol 10 ◽  
pp. 250 ◽  
Author(s):  
Sukwoo Hong ◽  
Takayuki Hara
Keyword(s):  
Case Report ◽  
Nerve Sheath Tumor ◽  
Modified Rankin Scale ◽  
Case Description ◽  
Rare Tumor ◽  
Review Of Literature ◽  
Tumor Mass ◽  
First Report ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath

Background: In neurosurgical practice, we rarely encounter hybrid nerve sheath tumors (HNST) in the orbit. We recently had a patient of this rare tumor. We believe that this is the first report where we resected the tumor transcranially. Case Description: A 54-year-old male presented with the left proptosis and intraconal tumor of 43 mm. We performed fronto-orbital craniotomy to resect the tumor mass. His proptosis completely improved and discharged home with a modified Rankin Scale of 1. Conclusion: Transcranial resection of orbital HNST was a safe and effective way to treat. Since we do not have much data regarding this rare tumor, we need to accumulate more cases.

FORAMINAL NERVE SHEATH TUMORS

Neurosurgery ◽  
2009 ◽  
Vol 64 (suppl_2) ◽  
pp. A33-A43 ◽  
Author(s):  
Judith A. Murovic ◽  
Iris C. Gibbs ◽  
Steven D. Chang ◽  
Bret C. Mobley ◽  
Jon Park ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Medical Center ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Central Necrosis ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Neurological Findings ◽  
Nerve Sheath ◽  
Asymptomatic Case

Abstract OBJECTIVE To conduct a retrospective review of outcomes in 15 patients with 18 foraminal tumors, including 17 benign peripheral nerve sheath tumors and 1 malignant peripheral nerve sheath tumor, who underwent CyberKnife (Accuray, Inc., Sunnyvale, CA) radiosurgery at Stanford University Medical Center from 1999 to 2006. METHODS Symptoms and findings, neurofibromatosis (NF) association, previous radiation, imaging, dosimetry, tumor volume, central necrosis, and the relation of these factors to outcomes were evaluated. RESULTS Before treatment, 1 asymptomatic patient had radiculopathic findings, 3 patients experienced local pain with intact neurological examinations, and 7 patients had radiculopathic complaints with intact (1 patient), radiculopathic (4 patients), or radiculomyelopathic examinations (2 patients). Five patients had myelopathic complaints and findings. Three patients had NF1-associated neurofibromas, 1 patient with NF2 had a schwannoma, and 1 patient had a schwannomatosis-related lesion. Two likely radiation-induced lesions, a neurofibroma and a malignant peripheral nerve sheath tumor, were observed. Prescribed doses ranging from 16 to 24 Gy, delivered in 1 to 3 fractions of 6 to 20 Gy, resulted in maximum tumor doses ranging from 20.9 to 30 Gy. Target volumes ranged from 1.36 to 16.9 mL. After radiosurgery, the asymptomatic case remained asymptomatic, and neurological findings improved. Thirteen of 15 symptomatic patients with (12 patients) or without (3 patients) neurological findings improved (3 cases after resection) or remained stable, and 2 patients worsened. Symptoms and examinations remained stable or improved in 8 (80%) of 10 patients with schwannomas and 3 (60%) of 5 patients with neurofibromas. Tumor volumes decreased in 12 (67%) of 18 tumors and increased in 3 tumors. Tumor volumes decreased in 8 of 10 schwannomas and 3 of 7 neurofibromas. Central necrosis developed in 8 (44%) of 18 tumors. CONCLUSION CyberKnife radiosurgery resulted in pain relief and functional preservation in selected foraminal peripheral nerve sheath tumors and a malignant peripheral nerve sheath tumor. Symptomatic and neurological improvements were more noticeable with schwannomas. Myelopathic symptoms may necessitate surgical debulking before radiosurgery.

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