Cerebral Malignant Nerve Sheath Tumor, Triton Tumor Variant: Case Report

2003 ◽  
Vol 6 (2) ◽  
pp. 168-172 ◽  
Author(s):  
Leticia Bornstein-Quevedo ◽  
Fabiola Peralta-Olvera ◽  
Alfonso Marhx-Bracho ◽  
Rodolfo Rodríguez-Jurado ◽  
Beatriz de Leon-Bojorge
Keyword(s):  
Clinical Evidence ◽  
Immunohistochemical Study ◽  
Nerve Sheath Tumor ◽  
Intraparenchymal Hemorrhage ◽  
Malignant Triton Tumor ◽  
Nerve Sheath ◽  
Spindle Cells ◽  
S 100 ◽  
History Of ◽  
The Right

A case of a cerebral malignant triton tumor in a 3-year-old boy with a 2-month history of frontal headache and no clinical evidence of neurofibromatosis is reported. The computed tomography (CT) scan showed a large, irregular tumor in the right parietooccipital lobe. A partial surgical resection was performed. Histologically, the tumor was highly cellular and consisted of spindle cells with hyperchromatic and pleomorphic nuclei. Focally, neoplastic cells with rhabdomyoblastic features were found. The immunohistochemical study showed that tumor cells were positive for S-100 protein and CD57, and the rhabdomyoblasts expressed desmin, Myo-D1, and myoglobin. During the postoperative period, a massive intraparenchymal hemorrhage was identified and surgical drainage was performed. The patient worsened and died 10 days after the first surgery. Postmortem study was not authorized. Six cases of cerebral malignant nerve sheath tumor have been described; however, primary intraparenchymal malignant triton tumor has not been previously described.

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.

scholarly journals Spinal Canal Involvement in Solitary Infantile Myofibromatosis: Case Report

2017 ◽  
Vol 06 (03) ◽  
pp. 223-227
Author(s):  
S.N. Gautam ◽  
Siddharth Mittal
Keyword(s):  
Spinal Canal ◽  
Smooth Muscle Actin ◽  
Lower Limbs ◽  
Nerve Sheath Tumor ◽  
Infantile Myofibromatosis ◽  
Nerve Sheath ◽  
Biopsy Report ◽  
S 100 ◽  
History Of ◽  
Intradural Space

AbstractInfantile myofibromatosis involving the spinal canal is very rare; only 11 cases have been reported so far in the literature. The authors present a case of an 18-month-old male child who presented with the history of dribbling of urine and weakness in bilateral lower limbs since 2 months. MRI of spine revealed single intramedullary intradural space-occupying lesion (SOL) at D1 to D2.The patient underwent laminectomy with excision of SOL with biopsy report suggestive of benign nerve sheath tumor, and immunohistochemistry report revealed desmin negative, smooth muscle actin positive, and S-100 focally positive infantile myofibromatosis. The patient gradually recovered and had a clear stream of urine with improved movements and tone of bilateral lower limbs at the time of discharge.

scholarly journals Cervical Intramedullary Schwannoma with Syrinx: Case Report and Review of the Literature

2017 ◽  
Vol 07 (03) ◽  
pp. 239-248
Author(s):  
Abdulaziz AlQarni ◽  
A. AlArifi ◽  
Ali Alassiri ◽  
Amjed Kouli ◽  
M. Abbas
Keyword(s):  
Spinal Cord ◽  
Cervical Spinal Cord ◽  
Lower Limbs ◽  
Nerve Sheath Tumor ◽  
Resonance Imaging ◽  
Spinal Cord Tumors ◽  
Nerve Sheath ◽  
Spindle Cells ◽  
The Right ◽  
Intramedullary Schwannoma

AbstractSchwannoma is a nerve sheath tumor originating from the Schwann cell. It is benign in nature and it arises from anywhere where Schwann cells can be found. It is rarely found in the parenchyma of the spinal cord. Intramedullary schwannomas (or neurilemmomas) without evidence of neurofibromatosis are rare spinal cord tumors. Intramedullary schwannoma was first reported in 1932 by Penfield. Our patient presented with neck pain, gradually worsening, weakness in the right upper and lower limbs, numbness in both shoulders, and a decrease in the grasping strength of both hands over a 4-year period. A magnetic resonance imaging of the spine showed a heterogeneously enhancing mass in the cervical spinal cord extending from the C2 to T1 levels with associated hemorrhagic changes. Histologically, the tumor was found to be composed of bland spindle cells with blunt-ended and sometimes wavy nuclei admixed with hyalinized vasculature. Surrounding reactive spinal cord parenchyma with frequent Rosenthal fibers was also observed. Focal Verocay bodies were evident, and with immunohistochemistry, there was diffuse and strong positivity for S100, which is confirmatory for the diagnosis of schwannoma. We report a case of cervical intramedullary schwannoma presented with syringobulbia in a young adult.

scholarly journals Seorang Laki-Laki dengan Tumor Mediastinum Posterior ( Malignant Peripheral Nerve Sheath Tumor )

2019 ◽  
Vol 2 (3) ◽  
pp. 82
Author(s):  
Daniel Maranatha ◽  
Shinta Karina Yuniati
Keyword(s):  
Peripheral Nerve ◽  
Malignant Tumors ◽  
Posterior Mediastinum ◽  
Surrounding Tissue ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
S 100 ◽  
The Right

Background: Malignant tumors arising from peripheral nerves or displaying differentiation along the lines of the various elements of the nerve sheath are referred to Malignant peripheral nerve sheath tumors (MPNST) and also called malignant schwannomas or neurofibrosarcomas. The common sites of involvement are head, neck, extremities and thorax. This case was discussed due to its rare incidence, namely less than 5% of soft tissue malignant tumor. The overall five year survival rate is approximately 50% in patients with resectable tumor. Case: We report a case of MPNST arising from mediastinum, the patient was a male, 41-years-old, with chief complaint are chest pain and mass in the back. Contrast enhanced chest CT Scan showed a solid mass (10.02 × 6.97 × 10.53 cm) in the right side of the posterior mediastinum. On microscopic examination with hematoxylin eosin imunostaining, of the mass on mediastinum showed typical features of MPNST, which were positive for S-100 imunostaining. Based on convensional histopathologic and imunostaining, this case was concluded as Malignant peripheral nerve sheath tumors (MPNST). Conclusion: The treatment of chest wall MPNSTs is multimodality, including wide local excision, adjuvant radiotherapy, and chemotherapy. In our case, we performed direct excision due to the infiltrating mass to the surrounding tissue, so the prognosis for this case was good. This case indicates the role of chemotherapy in treatment of advanced MPNST. Studies shows the superiority of the doxorubicin–ifosfamide regimen. As in this case the patient showed complete respons after surgery and adjuvant chemotherapy.

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.

scholarly journals Case report and immunohistochemical study of a malignant peripheral nerve sheath tumor in a dog

2017 ◽  
Vol 54 (4) ◽  
pp. 420
Author(s):  
Thaís De Almeida Moreira ◽  
Leandro Willian Borges ◽  
Talita Lopes Serra ◽  
Diego Petrocino Caetano ◽  
Marcelo Carrijo da Costa ◽  
...  
Keyword(s):  
Case Report ◽  
Peripheral Nerve ◽  
Immunohistochemical Study ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
S 100

O tumor maligno da bainha do nervo periférico é raro e pouco descrito em cães. Classicamente, o termo “neurofibroma/sarcoma” é empregado quando o tumor é composto por células de Schwann e células perineurais. Neste relato são descritos os achados clínicos, histopatológicos e imuno-histoquímicos de um caso de tumor maligno da bainha do nervo periférico, provavelmente neurofibrossarcoma, localizado no subcutâneo do membro pélvico de um cão da raça Rottweiler. Os achados histopatológicos associados às observações imuno-histoquímicas contribuíram para o diagnóstico, sendo observada positividade para proteína S-100, vimentina e CD57, permitindo a diferenciação da neoplasia em questão de outros tumores malignos com características morfológicas similares.

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.

Malignant Triton Tumor without Von Recklinghausen’s Neurofibromatosis in a 16 year-old Indonesian Girl: A Case Report

2017 ◽  
Vol 43 (1) ◽  
pp. 3
Author(s):  
Neni Anggraini ◽  
Nurjati Chairani Siregar
Keyword(s):  
Visual Acuity ◽  
Case Report ◽  
Early Detection ◽  
Peripheral Nerve ◽  
Correct Diagnosis ◽  
Nerve Sheath Tumor ◽  
Malignant Triton Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
S 100

Background: To diagnose malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation in malignant triton tumor which has a rare incidence. Case Illustration: We reported a case in a 16 year-old girl who presented with progressively painless proptosis of the left eye and decreased visual acuity. There was no evidence of Recklinghausen’s disease. She underwent surgery and radiotherapy. The histopatologic findings showed malignant peripheral nerve sheath tumor (MPNST). Four years later, she suffered recurrence proptosis and decreased visual acuity. Histomorphology supported by immunostaining with S-100 protein confirmed the diagnosis of malignant triton tumor. She underwent chemotherapy and enucleation. Conclusion: This case highlights the prudent use of immunohistochemistry that is essential in making an early detection and a correct diagnosis.

scholarly journals RARE-20. MALIGNANT PERIPHERAL NERVE SHEATH TUMOR OF A CRANIAL NERVE IN AN INFANT WITH NEUROCUTANEOUS MELANOSIS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii446-iii446
Author(s):  
Lacey Carter ◽  
Naina Gross ◽  
Rene McNall-Knapp ◽  
,and Jo Elle Peterson
Keyword(s):  
Peripheral Nerve ◽  
Cranial Nerve ◽  
Leptomeningeal Disease ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Neurocutaneous Melanosis ◽  
Spindle Cell Neoplasm ◽  
Lower Back ◽  
Nerve Sheath ◽  
S 100

Abstract At one month of age, a female presented with a giant congenital nevus along lower back and thighs and hydrocephalus. A ventriculoperitoneal shunt was placed. An MRI was done at six months, initially reported as normal. At eleven months of age, five months after original MRI, patient presented with dysconjugate gaze and lethargy. MRI showed new 3.8 x 3.7 x 3.4 cm right cerebellopontine angle mass extending into Meckel’s cave and foramen ovale along with leptomeningeal disease extending from the mass along the entire length of the spinal cord. Retrospective review of prior MRI revealed subtle leptomeningeal enhancement concerning for neurocutaneous melanosis (NCM). Given the leptomeningeal disease, family elected for open biopsy and debulking of lesion instead of aggressive resection. Histologically, the mass showed hypercellular spindle cell neoplasm with mitotic activity and necrosis mixed with remnants of normal cranial nerve. GFAP was negative, excluding a glioma. HMB-45, MITF, panmelanoma, and Melan-A were negative, excluding melanoma. A negative myogenin stain ruled out ectomesenchymoma. S-100 protein and SOX-10 positivity with variable loss of staining for trimethylation of histone H3 K27 were indicative of malignant peripheral nerve sheath tumor (MPNST). Given the course of the mass, trigeminal nerve MPNST was presumed. Given the poor prognosis of intracranial MPNST and NCM, family elected to forgo treatment and was discharged with hospice. She died 25 days after surgery. Cranial nerve MPNST is rare. MPNST in patients with NCM has not previously been reported to our knowledge.

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