• Archives of Plastic Surgery
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• v.38 no.6
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• pp.886-889
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• 2011

Purpose: Malignant peripheral nerve sheath tumor without neurofibromatosis type 1 is very rare neoplasm. Development in the superficial soft tissue is exremely rare. Authors experienced one rare case of primary malignant peripheral nerve sheath tumor developed on abdomen. The clinical and histologic findings were described. Methods: An 83-year-old man visited hospital with an $11{\times}6.5{\times}4.5$ cm sized ulcerated and hemorrhagic mass on abdomen. The tumor was localized in abdominal skin and started growing 3 years ago. Results: Wide excision with safety margin of 2 cm and limberg flap was done. The postoperative biopsy revealed a malignant peripheral nerve sheath tumor. There was no evidence of recurrence of tumor for 16 months. Conclusion: Malignant peripheral nerve sheath tumor is an aggressive malignant tumor. An abrupt enlargement of size, ulceration and bleeding are suggestive of malignant chnages of the tumor. We recommand early wide excision with enough safety margin as treatment of malignant peripheral nerve sheath tumor.

• Archives of Plastic Surgery
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• v.33 no.6
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• pp.761-763
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• 2006

Purpose: Malignant peripheral nerve sheath tumors most often arise from the anatomically discernible peripheral nerve or neurofibroma. Methods: A 55-year-old man had a rapidly growing pedunculated large mass on the sacrolumbar junction for 2 years. He has congenital neurofibromatosis type I. He had multiple caf-au-lait spots and multiple neurofibromas on the entire body. The mass developed from a subcutaneous nodule on the sacrolumbar junction and grew rapidly. The preoperative punch biopsy revealed a malignant peripheral nerve tumor. The mass was completely excised with 1 cm free margin above the deep fascial plane. Results: There was no evidence of recurrence of tumor for 19 months of follow-up examination. Conclusion: Malignant peripheral nerve sheath tumor is very rare and has unique feature. We report a successful case of malignant peripheral nerve sheath tumor with the review of the literatures.

• The Korean Journal of Pain
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• v.26 no.2
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• pp.160-163
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• 2013

Malignant peripheral nerve sheath tumors (MPNSTs) are very rare sarcomas derived from various cells in the peripheral nerve sheath. Malignant peripheral nerve sheath tumors have a known association with neurofibromatosis type 1. Diagnosis of MPNSTs is difficult in patients with chronic pain, when MPNST occurs at an overlapping area of chronic pain. Therefore, the diagnosis can be missed unless clinicians pay attention to the possibility of this disease. Here in, we report a case of concurrent malignant peripheral nerve sheath tumor with complex regional pain syndrome type 1. A 44-year female patient, who was diagnosed with complex regional pain syndrome (CRPS) type 1 in her left ankle, visited our clinic because of aggravated pain. The cause of the aggravated pain was revealed as concurrent MPNST in the left common peroneal nerve territory, which overlapped the site of pain from CRPS.

• Korean Journal of Otorhinolaryngology-Head and Neck Surgery
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• v.55 no.3
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• pp.181-184
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• 2012

The malignant peripheral nerve sheath tumor is an extremely rare soft tissue sarcoma. It is a highly malignant sarcoma, which is locally invasive, frequently leading to multiple recurrences and eventual metastatic spread. The peak incidence of disease is known to occur sporadically between the age of 20s and 50s, and is usually associated with the neurofibromatosis type I. In human body, the trunk and extremities are the most commonly involved sites, with only 8-14% of all lesions appearing in the head and neck region. We present a case of malignant peripheral nerve sheath tumor involving the right parapharynx in a 48-year-old patient who complained of headaches in the right parietal area and of dysphagia that aggravated over a month. After surgery, tumor was finally diagnosed as malignant peripheral nerve sheath tumor by histopathologic examinations. The authors report a case of malignant peripheral nerve sheath tumor in the right parapharynx with a review of the literature.

• Korean Journal of Head & Neck Oncology
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• v.28 no.1
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• pp.37-41
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• 2012

The malignant peripheral nerve sheath tumor(MPNST) is an extremely rare soft tissue sarcoma of ectomesenchyme also known as malignant schwannoma or malignant neurilemoma. It is a highly malignant sarcoma, which is locally invasive, frequently leading to multiple recurrences and eventual metastatic spread. The peak incidence of this disease is known to arise in the seventh decades. In human body, trunk and extremities are the most commonly involved sites, and only 8-14% of all lesions appear in head and neck region. Because immunohistochemical staining is essential in final diagnosis. The authors report a case of malignant peripheral nerve sheath tumor in neck with hypopharyngeal cancer simultaneously on a review of the literature.

• Journal of Korean Neurosurgical Society
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• v.44 no.2
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• pp.91-94
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• 2008

Malignant peripheral nerve sheath tumors (MPNSTs) are very rare tumors. We experienced a case of MPNST in the cervical paraspinal space which was not associated with neurofibromatosis. The tumor located in left C6-7 foramen and compressed C7 root. The tumor was removed through the occipital triangle. We report a case of the primary cerivcal MPNST in a patient who did not have neurofibromatosis-1.

• Journal of Korean Neurosurgical Society
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• v.53 no.3
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• pp.190-193
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• 2013

A malignant peripheral nerve sheath tumor (MPNST) is a type of sarcoma that arises from peripheral nerves or cells of the associated nerve sheath. This tumor most commonly metastasizes to the lung and metastases to the spinal cord and brain are very rare. We describe a case of young patient with spinal cord and brain metastases resulting from MPNST. An 18-year-old man presented with a 6-month history of low back pain and radiating pain to his anterior thigh. Magnetic resonance imaging showed a paraspinal mass that extended from the central space of L2 to right psoas muscle through the right L2-3 foraminal space. The patient underwent surgery and the result of the histopathologic study was diagnostic for MPNST. Six months after surgery, follow-up images revealed multiple spinal cord and brain metastases. The patient was managed with chemotherapy, but died several months later. Despite complete surgical excision, the MPNST progressed rapidly and aggressively. Thus, patients with MPNST should be followed carefully to identify local recurrence or metastasis as early as possible.

• Archives of Craniofacial Surgery
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• v.15 no.3
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• pp.125-128
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• 2014

Malignant peripheral nerve sheath tumors are extremely rare soft tissue sarcomas. Among various locations, the trunk and extremities are the most commonly involved sites, with only 15% of such lesions occuring in head and neck region. Here, we report a case of a 74-year-old male who presented with forehead swelling and right eye deviation. Computed tomography images revealed a tumor involving the frontal sinus, ethmoid sinus, and the orbital cavity. The patient underwent a surgical excision of the lesion, which histopathological examination revealed to be a malignant peripheral nerve sheath tumor.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.30 no.3
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• pp.228-233
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• 2004

Summary: The malignant peripheral nerve sheath tumor(MPNST) is an aggressive neoplasm and can either arise independently or result from malignant change in preexisting neurofibromatosis (von Recklinghausen's disease). Its histologic characteristics remain controversial, but currently it is believed that the schwann cell is the origin of the peripheral nerve sheath tumors. MPNST is an uncommon neoplasm of the head and neck region, and its presentation in the oral cavity is quite rare. In this study, we report a patient with a rare case of a MPNST involving the maxilla. A case report: A 29-year-old female presented with a chief complaint of painless swelling with bleeding tendency on the left maxillary tuberosity area 2 months ago. Clinical examination showed a $5.0{\times}3.0cm^2$ sized, indurative swelling on the site. Conventional radiographs showed a relatively well-defined soft tissue mass involving the left maxillary sinus, and destruction of the anterior, posterolateral walls of the left maxillary sinus. Subtotal maxillectomy and split-thickness skin graft from thigh were undertaken. In histochemical and immunohistochemical studies, the specimen revealed positive reactivities to Vimentin and S-100 protein. Final diagnosis was made as MPNST.

• Journal of Korean Neurosurgical Society
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• v.30 no.sup2
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• pp.356-360
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• 2001

Malignant peripheral nerve sheath tumor(MPNST, primary malignant schwannoma) within the spinal canal is very rare. The prognosis of MPNST in the spinal canal is very poor due to the tendency of perineural spread, dissemination throughout subarachnoid space and local recurrance. This report details the authors' experience on the case of primary malignant spinal schwannoma with review of the literatures and other studies.