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

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.

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

10.21203/rs.3.rs-942103/v1 ◽

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.

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

Bosnian Journal of Basic Medical Sciences ◽

10.17305/bjbms.2010.2681 ◽

2010 ◽

Vol 10 (4) ◽

pp. 328-330 ◽

Cited By ~ 5

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.

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Malignant Peripheral Nerve Sheath Tumor of the Infraorbital Nerve

Craniomaxillofacial Trauma & Reconstruction ◽

10.1055/s-0035-1563698 ◽

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.

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

Journal of Neurosurgery Spine ◽

10.3171/2014.4.spine13739 ◽

2014 ◽

Vol 21 (3) ◽

pp. 367-371 ◽

Cited By ~ 5

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.

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Pleomorphic Corneal Sarcoma Resembling Malignant Peripheral Nerve Sheath Tumor in a Horse

Veterinary Pathology ◽

10.1354/vp.08-vp-0067-k-bc ◽

2009 ◽

Vol 46 (3) ◽

pp. 444-448 ◽

Cited By ~ 8

Author(s):

E. C. Kappe ◽

K. KÖHLER ◽

I. v. Felbert ◽

J. P. Teifke ◽

J. TÓTH ◽

...

Keyword(s):

Peripheral Nerve ◽

Domestic Animals ◽

Bovine Papillomavirus ◽

Nerve Sheath Tumor ◽

Human Beings ◽

Immunohistochemical Examination ◽

Peripheral Nerve Sheath Tumor ◽

Mesenchymal Neoplasms ◽

Nerve Sheath ◽

The Right

A 14-year-old Haflinger gelding presented with a protruding mass involving the cornea of the right eye. The mass was resected and submitted for histopathologic and immunohistochemical examination. The preliminary diagnosis was corneal sarcoma, most likely fibrosarcoma. The immunohistochemical results confirmed the mesenchymal origin of the neoplastic cells, which were most consistent with a malignant peripheral nerve sheath tumor. Corneal mesenchymal neoplasms are extremely uncommon tumors in human beings and domestic animals. The cause for this tumor was not determined; infection with bovine papillomavirus was not detected.

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Malignant peripheral nerve sheath tumor of the distal phalanx of the fifth toe: a case report

Acta Radiologica Short Reports ◽

10.1177/2047981613516033 ◽

2014 ◽

Vol 3 (1) ◽

pp. 204798161351603 ◽

Cited By ~ 1

Author(s):

Yuki Iwama ◽

Makoto Kunisada ◽

Hajimu Goto ◽

Yoshiharu Ohno ◽

Junji Yamashita ◽

...

Keyword(s):

Peripheral Nerve ◽

Distal Phalanx ◽

Nerve Sheath Tumor ◽

Resonance Imaging ◽

Peripheral Nerve Sheath Tumor ◽

Histological Features ◽

Nerve Sheath ◽

Subcutaneous Tissues ◽

Fifth Toe ◽

The Right

Malignant peripheral nerve sheath tumor (MPNST) involving bone is rare. We report a case of MPNST of the fifth toe. The lesion was located in the distal phalanx of the right fifth toe and extended into surrounding subcutaneous tissues. Findings on magnetic resonance imaging and histological features of the case are described and the literature is briefly reviewed.

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First documented case of intracranial falcine malignant peripheral nerve sheath tumor: illustrative case

Journal of Neurosurgery: Case Lessons ◽

10.3171/case21255 ◽

2021 ◽

Vol 2 (6) ◽

Author(s):

Renato J. Galzio ◽

Mattia Del Maestro ◽

Diamantoula Pagkou ◽

Massimo Caulo ◽

Sofia Asioli ◽

...

Keyword(s):

Peripheral Nerve ◽

Cranial Nerves ◽

Illustrative Case ◽

Nerve Sheath Tumor ◽

Falx Cerebri ◽

Peripheral Nerve Sheath Tumor ◽

Documented Case ◽

Nerve Sheath ◽

First Case ◽

The Right

BACKGROUND The authors reported the first documented case of intracranial extraaxial nonneurofibromatosis type 1–related nontriton malignant peripheral nerve sheath tumor (MPNST) originating from the falx cerebri. OBSERVATIONS A 34-year-old man with headache, short-term memory deficit, postural instability, and blurred vision presented with a large heterogenous contrast-enhanced intraventricular cystic lesion originating from the free margin of the falx cerebri. The patient received surgery using the right posterior interhemispheric approach. Gross total resection was performed, and the inferior border of the falx cerebri was resected. The postoperative course was uneventful. Histological examination revealed hypercellular foci of neoplastic spindle cells with hyperchromatic and wavy nuclei. Hence, a diagnosis of MPNST was made based on concomitant immunochemistry findings, including mouse double minute 2 homolog focal positivity and geographic loss of H3K27me3. The patient received adjuvant radiotherapy, and recurrence was not observed. LESSONS Intracranial MPNSTs are extremely rare tumors, typically originating from the cranial nerves in the posterior cranial fossa. An even rarer variant of these tumors, referred to as malignant intracerebral nerve sheath tumors, may directly arise from the brain parenchyma. The authors reported the first case of an intracranial MPNST originating from the dura mater of the falx cerebri, acting as an extraaxial lesion with prevalent expansion in the right ventricle.

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Malignant Peripheral Nerve Sheath Tumor of the Inguinum and Angiosarcoma of the Scalp in a Child with Neurofibromatosis Type 1

Case Reports in Pathology ◽

10.1155/2017/7542825 ◽

2017 ◽

Vol 2017 ◽

pp. 1-4

Author(s):

Marija Milković Periša ◽

Tihana Džombeta ◽

Jasminka Stepan Giljević ◽

Božo Krušlin

Keyword(s):

Peripheral Nerve ◽

Neurofibromatosis Type 1 ◽

Malignant Tumors ◽

Young Patients ◽

Neurofibromatosis Type ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath ◽

The Right

Benign and malignant tumors are common in the setting of neurofibromatosis type 1 (NF1). Malignant peripheral nerve sheath tumor (MPNST) and angiosarcoma are rare tumors in children and adolescents and mostly occur in young patients in relation to NF1. Both histological types can be present in the same tumor mass in patients with NF1. We present a case of 12.5-year-old girl with NF1 who first presented with MPNST of the right inguinal region and 1.5 years later with unrelated angiosarcoma of the scalp.

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RT-5 Boron Neutron Capture Therapy has extended progression-free survival about recurrent malignant peripheral nerve sheath tumor - A case report

Neuro-Oncology Advances ◽

10.1093/noajnl/vdab159.056 ◽

2021 ◽

Vol 3 (Supplement_6) ◽

pp. vi15-vi15

Author(s):

Yuko Watanabe ◽

Hiroshi Igaki ◽

Ayumu Arakawa ◽

Shunsuke Yanagisawa ◽

Masamichi Takahashi ◽

...

Keyword(s):

Peripheral Nerve ◽

Neutron Capture ◽

Progression Free Survival ◽

Neutron Capture Therapy ◽

Nerve Sheath Tumor ◽

Free Survival ◽

Peripheral Nerve Sheath Tumor ◽

Boron Neutron Capture ◽

Nerve Sheath ◽

The Right

Abstract Introduction: Recurrent malignant peripheral nerve sheath tumor (MPNST) is intractable. Boron neutron capture therapy (BNCT) is a treatment using tumor-selective particle radiation, and is indicated for medical treatment for head and neck cancer, and also used for malignant glioma and malignant meningioma.<Case>20-year-old woman who has MPNST that extends from the subcutaneous tissue of the right neck to the posterior fossa. <Medical History>Chemotherapy and local irradiation(50.4Gy/28fr) for primary embryonal rhabdomyosarcoma of the right ear canal at the age six.<Current medical history>Right cervical tumor developed at 17-year-old, some chemotherapy regimens and tumor resections were performed as recurrence of rhabdomyosarcoma at the previous hospital. After she was diagnosed with MPNST in the pathology consultation at our hospital, she was irradiated with heavy ion beam 70.8Gy(RBE)/16fr and received additional chemotherapies at our department, but her tumor was refractory. Although BNCT for MPNST is not covered by health insurance in Japan, she wanted to try to be treated for BNCT. After confirming boron accumulation in the tumor (SUVmax 4.28) by FBPA-PET, tumor growth and hydrocephalus occurred while waiting for travel to Taiwan due to the spread of COVID-19 infection. She was performed tumor resection(NTR) and irradiated with SRS 20Gy/fr for the residual lesion, but tumor had a rapid recurrence from the margin of the excision cavity. Finally, she could travel about 3 months after the operation and underwent BNCT, that used neutrons and 10B-boronophenylalanine from the Tsinghua University research reactor in Taiwan. No serious adverse events including cerebral edema were observed, and dramatic tumor shrinkage was maintained after treatment. FBPA-PET of 3 months later showed accumulation in the part of the margin of the cavity, the recurrence was observed on MRI after 3 and a half months. Discussion: BNCT for refractory/recurrent MPNST showed acceptable safety and was able to prolong progression-free survival.

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Case report of malignant peripheral nerve sheath tumor of parotid in a patient with neurofibromatosis type 1

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20214688 ◽

2021 ◽

Vol 7 (12) ◽

pp. 1927

Author(s):

Anuradha P. ◽

Bathi Reddy ◽

Suhail Gaur

Keyword(s):

Parotid Gland ◽

Peripheral Nerve ◽

Protein A ◽

Soft Tissue Sarcomas ◽

Neurofibromatosis Type ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath ◽

Parotid Swelling ◽

The Right

Malignant peripheral nerve sheath tumor (MPNST) refers to spindle cell sarcomas arising from or separating in the direction of cells of the peripheral nerve sheath. The MPNST of the parotid gland is an extremely rare tumor, accounts for <5% of all soft tissue sarcomas, and carries a poor prognosis. Diagnosis of MPNSTs is usually based on histopathology aided by IHC, which reflects the Schwann cell differentiation in this neoplasm. Approximately 50-90% of MPNSTs are positive for S-100 protein. A 56-year-old male presented with right parotid swelling for 3 months and incomplete right eye closure. The patient was diagnosed with neurofibromatosis 1 (NF-1) and pulmonary tuberculosis in the past. On Examination, a hard, tender swelling measuring 5×4 cm involving the right parotid gland and right grade 2 LMN facial palsy was noted. Histopathology was suggestive of a MPNST.

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