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.

scholarly journals Malignant Peripheral Nerve Sheath Tumor of the Infraorbital Nerve

2016 ◽  
Vol 9 (2) ◽  
pp. 170-174
Author(s):  
JoséLuis D'Addino ◽  
Laura Piccoletti ◽  
MaríaMercedes Pigni ◽  
Maria José Rodriguez Arenas de Gordon
Keyword(s):  
Peripheral Nerve ◽  
Chronic Atrial Fibrillation ◽  
Radical Resection ◽  
Infraorbital Nerve ◽  
Malignant Schwannoma ◽  
Ocular Motility ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
History Of

The objective of this study is to report a large, rare, and ulcerative infiltrated skin lesion. Its diagnosis, therapeutic management, and progress are described. The patient is a 78-year-old white man, who presented with a 12-month ulcerative perforated lesion that had affected and infiltrated the skin, with easy bleeding. He had a history of hypertension, although controlled, was a 40-year smoker, had chronic atrial fibrillation, diabetes, and microangiopathy. During the consultation, the patient also presented with ocular obstruction due to an inability to open the eye. He mentioned having reduced vision. The computed tomography scan showed upper maxilla osteolysis without eye involvement. We underwent a radical resection in which upper maxilla and the anterior orbital margin were included. We used a Becker-type flap that allowed us to rebuild the cheek and to complete a modified neck dissection. Progress was favorable; the patient recovered ocular motility and his vision improved to 20/200. The final biopsy result was “malignant peripheral nerve sheath tumor, malignant schwannoma.” Malignant schwannoma of the peripheral nerve is extremely rare. The total resection and reconstruction being completed in one surgery represented a challenge due to the difficulty in obtaining tissues in addition to the necessity of an oncological resection.

scholarly journals Systemic Treatment for Advanced and Metastatic Malignant Peripheral Nerve Sheath Tumors—A Sarcoma Reference Center Experience

2020 ◽  
Vol 9 (10) ◽  
pp. 3157
Author(s):  
Paweł Sobczuk ◽  
Paweł Teterycz ◽  
Anna M. Czarnecka ◽  
Tomasz Świtaj ◽  
Hanna Koseła-Paterczyk ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Systemic Treatment ◽  
Treatment Options ◽  
Soft Tissue Sarcomas ◽  
Nerve Sheath Tumor ◽  
First Line ◽  
Rare Type ◽  
Reference Center ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Malignant peripheral nerve sheath tumor (MPNST) is a rare type of soft tissue sarcomas. The localized disease is usually treated with surgery along with perioperative chemo- or radiotherapy. However, up to 70% of patients can develop distant metastases. The study aimed to evaluate the modes and outcomes of systemic treatment of patients with diagnosed MPNST treated in a reference center. In total, 115 patients (56 female and 59 male) diagnosed with MPNST and treated due to unresectable or metastatic disease during 2000–2019 were included in the retrospective analysis. Schemes of systemic therapy and the outcomes—progression-free survival (PFS) and overall survival (OS)—were evaluated. The median PFS in the first line was 3.9 months (95% CI 2.5–5.4). Doxorubicin-based regimens were the most commonly used in the first line (50.4% of patients). There were no significant differences in PFS between chemotherapy regimens most commonly used in the first line (p = 0.111). The median OS was 15.0 months (95% CI 11.0–19.0) and the one-year OS rate was 63%. MPNST are resistant to the majority of systemic therapies, resulting in poor survival in advanced settings. Chemotherapy with doxorubicin and ifosfamide is associated with the best response and longest PFS. Future studies and the development of novel treatment options are necessary for the improvement of treatment outcomes.

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.

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.

scholarly journals Extradural malignant peripheral nerve sheath tumor of the thoracic spine: A rare case report

2021 ◽  
Vol 12 ◽  
pp. 560
Author(s):  
Ayu Yoniko Christi ◽  
Wisnu Baskoro ◽  
Bidari Kameswari ◽  
Irfaanstio Akbar Hakim ◽  
Vega Sola Gracia Pangaribuan ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Thoracic Spine ◽  
Soft Tissue Sarcomas ◽  
Cord Compression ◽  
Nerve Sheath Tumor ◽  
Total Excision ◽  
Peripheral Nerve Sheath Tumors ◽  
Immunohistochemistry Staining ◽  
Nerve Sheath ◽  
Progressive Paraparesis

Background: Malignant peripheral nerve sheath tumors (MPNSTs) typically found in the trunk, limbs, head, and neck represent 3–10% of all soft-tissue sarcomas. Although they typically originating from peripheral nerve Schwann cells, 2–3% arise from the spinal nerves and may be found within the spinal canal. Here, we present a 43-year-old male with an extradural thoracic MPNST contributing to marked cord compression and a progressive paraparesis. Case Description: A 43-year-old male presented with a progressive paraparesis of 16 months’ duration. The MRI showed a posterior T2-T4 extradural tumor in the thoracic spine resulting in significant cord compression. Following a T2-T4 laminectomy and gross total excision of the epidural mass, the patient regained modest neurological function. Immunohistochemistry staining supported the diagnosis of thoracic spinal MPNST. Conclusion: Rarely, spinal MPNST can be considered amongst the differential diagnoses of an extradural spinal tumor. In this case, gross total excision of a posterior T2-T4 epidural MPNST resulted in improvement in the patient’s original paraparesis. Notably, immunohistochemistry staining helped confirm the diagnosis of a MPNST.

Peripheral Nerve Sheath Tumor of the Vagus Nerve in a Dog

2016 ◽  
Vol 52 (1) ◽  
pp. 57-62 ◽  
Author(s):  
Fui Yap ◽  
Kathryn Pratschke
Keyword(s):  
Peripheral Nerve ◽  
Vagus Nerve ◽  
Cervical Region ◽  
Nerve Sheath Tumor ◽  
Computed Tomography Scan ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
History Of ◽  
Tomography Scan ◽  
Exploratory Surgery

A peripheral nerve sheath tumor was diagnosed in a female, neutered Labrador retriever with a 6 mo history of coughing, retching, ptyalism, and left-sided Horner's syndrome. Computed tomography scan of the neck revealed a mass lesion between the carotid artery and esophagus in the mid-cervical region. Exploratory surgery was performed and an 18 cm section of thickened vagus nerve was excised. Histopathological findings and immunochemistry staining confirmed a malignant peripheral nerve sheath tumor. The tumor showed microscopic signs of malignancy, but there were no macroscopic signs of local extension or distant metastasis. This report documents a peripheral nerve sheath tumor of rare origin in dogs.

scholarly journals Intrathoracic malignant peripheral nerve sheath tumor with poor outcome: a case report

2010 ◽  
Vol 10 (4) ◽  
pp. 328-330 ◽  
Author(s):  
Violeta Kolarov ◽  
Jelena Stanić ◽  
Živka Eri ◽  
Biljana Zvezdin ◽  
Marija Kojičić ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Nerve Sheath Tumor ◽  
Lethal Outcome ◽  
Body Mass Loss ◽  
Large Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Progressive Dyspnea ◽  
History Of ◽  
The Right

We report a case of intrathoracic malignant peripheral nerve sheath tumor in a 65-year old woman revealed after a few-month history of progressive dyspnea, appetite and body mass loss. The chest magnetic resonance (MR) examination revealed the presence of a large tumor occupying the mediastinum and a major portion of the right hemithorax. The diagnostic tumor sample was obtained by parasternal biopsy in local anesthesia. The surgical resection of the tumor could not be performed due to its excessive size, intrathoracic involvement and bad respiratory reserves of a patient. The chemotherapy and irradiation were performed as palliative measures. The lethal outcome appeared 10 months after the diagnosis was established.

Recurrent Malignant Peripheral Nerve Sheath Tumor Presenting As an Asymptomatic Intravenous Thrombus Extending to the Heart: A Case Report

2021 ◽  
Author(s):  
Toru Hirozane ◽  
Robert Nakayama ◽  
Sayaka Yamaguchi ◽  
Tomoaki Mori ◽  
Naofumi Asano ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Radial Nerve ◽  
Subclavian Vein ◽  
Tumor Thrombus ◽  
Venous Invasion ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
History Of ◽  
The Right

Abstract Background: Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma mainly treated via surgical resection. Herein, we report a case of MPNST wherein a massive tumor thrombus extended to the major veins and heart.Case presentation: A 39-year-old female with a history of neurofibromatosis type 1 developed MPNST from the right radial nerve. In addition to adjuvant chemotherapy, she underwent wide tumor resection and concomitant radial nerve resection, followed by postoperative radiotherapy. Histological evaluation revealed marked venous invasion. The 2-year follow-up CT revealed an asymptomatic recurrent tumor thrombus extending from the right subclavian vein to the heart. An urgent life-saving operation was performed to ligate the base of the right subclavian vein and remove the entire intravenous thrombus that extended to the right ventricle. The remaining tumor in the right subclavian vein increased in size 3 months after thrombectomy. After confirming the absence of any metastatic lesions, the patient underwent extended forequarter amputation to achieve surgical remission. One year later, a new metastasis to the right diaphragm was safely resected. The patient remains alive without any evidence of disease 2 years after the extended forequarter amputation. Conclusions: In cases of a previous history of microscopic venous invasion, recurrence can occur as a massive tumor thrombus that extends to the great vessels.

scholarly journals Recurrent malignant peripheral nerve sheath tumor presenting as an asymptomatic intravenous thrombus extending to the heart: a case report

2022 ◽  
Vol 20 (1) ◽  
Author(s):  
Toru Hirozane ◽  
Robert Nakayama ◽  
Sayaka Yamaguchi ◽  
Tomoaki Mori ◽  
Naofumi Asano ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Radial Nerve ◽  
Subclavian Vein ◽  
Tumor Thrombus ◽  
Venous Invasion ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
History Of ◽  
The Right

Abstract Background Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma mainly treated via surgical resection. Herein, we report a case of MPNST wherein a massive tumor thrombus extended to the major veins and heart. Case presentation A 39-year-old female with a history of neurofibromatosis type 1 developed MPNST from the right radial nerve. In addition to adjuvant chemotherapy, she underwent wide tumor resection and concomitant radial nerve resection, followed by postoperative radiotherapy. Histological evaluation revealed marked venous invasion. The 2-year follow-up CT revealed an asymptomatic recurrent tumor thrombus extending from the right subclavian vein to the heart. An urgent life-saving operation was performed to ligate the base of the right subclavian vein and remove the entire intravenous thrombus that extended to the right ventricle. The remaining tumor in the right subclavian vein increased in size 3 months after thrombectomy. After confirming the absence of any metastatic lesions, the patient underwent extended forequarter amputation to achieve surgical remission. One year later, a new metastasis to the right diaphragm was safely resected. The patient remains alive without any evidence of disease 2 years after the extended forequarter amputation. Conclusions In cases of a previous history of microscopic venous invasion, recurrence can occur as a massive tumor thrombus that extends to the great vessels.

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.

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