• Archives of Plastic Surgery
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• v.37 no.1
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• pp.67-70
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• 2010

Purpose: Nerve sheath myxoma is a rare cutaneous neoplasm originating from the peripheral nerve sheath and divided into three groups : myxoid, cellular and mixed type. There is a controversy on it's origin whether schwannian cell or perineurial differentiation, or anything else. Myxoid nerve sheath myxoma is asymptomatic, soft, papule or nodule in middle-age adults. We report a case of myxoid nerve sheath myxoma on the fingertip. Methods: A 53-year-old woman presented with a painful, $0.4{\times}0.4{\times}0.6\;cm $sized, corn shaped nodule on the left 3rd fingertip. We put into surgical excision and studied it by histopathologically and specific immnohistochemical stain. Results: The tumor has well defined nodules separated by thin fibrous connective tissue with abundant myxoid stroma and were positively stainded for S-100 protein, NSE and GFAP. After surgical treatment it was healed without recurrence. Conclusion: Nerve sheath myxoma is rare neoplasm and located mainly on face, but very rarely on the fingertip. We report a case of painful myxoid nerve sheath myxoma located on the 3rd fingertip.

• 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.

• Archives of Plastic Surgery
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• v.47 no.1
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• pp.92-96
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• 2020

Malignant peripheral nerve sheath tumor (MPNST) is a rare and often aggressive soft tissue sarcoma originating from the sheaths of peripheral nerves. Approximately 50% of MPNSTs occur in patients with neurofibromatosis (NF). These tumors often present as deep soft tissue lesions, arising from the nerve plexuses of the extremities or from the nerves extending from the trunk. They rarely occur in the skin, especially in patients with NF. Herein, we report our experience with an MPNST of the skin in a patient with NF.

• 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.

• 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 Yeungnam Medical Science
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• v.36 no.1
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• pp.63-66
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• 2019

Malignant peripheral nerve sheath tumor (MPNST) is rare, accounting for 5-10% of all soft tissue sarcomas. MPNST is characteristically aggressive and has a poor prognosis. Fifty percent of patients with MPNST have neurofibromatosis type 1 (NF1). NF-associated MPNST occurs more often at younger ages than sporadic MPNST, but the survival difference is controversial. Superficial MPNST from a recurrent neurofibroma is extremely rare and only a limited number of cases have been reported in the literature. Herein, we report an unusual case of superficial MPNST from a recurrent neurofibroma in a patient without NF1.

• Journal of Chest Surgery
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• v.30 no.10
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• pp.1051-1053
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• 1997

Malignant schwannoma is rare tumor which is derived from schwas cells or nerve sheath cells, and it is frequently associated with Von Rechlinghausen's disease. We experienced one case of malignant schwannoma on the right chest wall without Von Rechlinghausen's disease. Patient was 64-year-old man who presented painless palpable mass on the right lower -chest wall for about 2 months. On chest computed tomography, the mass which was 6$\times$6 cm in size and had central necrosis, involved 11th rib with destruction, invaded the diaphragm and displaced the kidney anteriorlly. He underwent on-bloc resection of the tumor and discharged without any problem after 20 days.

• Journal of the Korean Society of Radiology
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• v.84 no.6
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• pp.1384-1390
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• 2023

Primary masses rarely originate from the heart and great vessels, and a malignant peripheral nerve sheath tumor (MPNST) is extremely rare. A 76-year-old male with pleural effusion underwent contrast-enhanced computed tomography, which revealed a hypoattenuating mass involving the right pulmonary vein and left atrium. Ultrasonography showed that the mass originated from the right pulmonary vein. Surgical resection confirmed an MPNST that originated from the pulmonary vein. We report the first Korean case of a primary MPNST originating from the pulmonary vein. We have also described the radiologic findings suggestive of a pulmonary vein mass.

• Korean Journal of Head & Neck Oncology
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• v.16 no.1
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• pp.69-72
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• 2000

The neurilemmoma is a relatively uncommon benign neoplasm which is known to be originated from the schwann sheath of the nerve fiber. Parapharyngeal neurilemmomas may originate from any nerve traversing this space, but the vast majority arise from the vagus nerve and sympathetic chain. The neurilemmomas arising from the extracranial portion of the hypoglossal nerve are extremely rare. To our knowledge, the case we present is the ninth one to be reported occurring in the parapharyngeal space. Recently we experienced a case of parapharyngeal neurilemmoma arising from the hypoglossal nerve and so we report our case with a brief review of literatures.

• Korean Journal of Head & Neck Oncology
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• v.24 no.2
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• pp.174-178
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• 2008

Background and Objectives：Peripheral nerve sheath tumors(PNSTs) are uncommon neoplasm in the head and neck region. The treatment of PNSTs is surgical removal, but excision cause neurologic complications. This study was performed to evaluate the proper diagnosis and treatment of PNSTs with our experiences. Subjects and Method：During the period from October 1994 to July 2007, 58 patients were diagonised with PNSTs in head and neck region. We reviewed medical records and imaging study retrospectively. Result：95%(55/58) of the PNSTs in head and neck were benign；5%(3/58) were malignant peripheralnerve sheath tumors(MPNSTs). 63%(37/58) were neurilemoma, 20%(17/20) were neurofibroma, 2%(1/58) was perineuroma. 55 patients underwent surgery. 80%(45/55) of cases were treated with excision. Enucleation was performed in 7(14%) patients. All cases of benign neurogenic tumors showed no recurrence. Among 3 of MPNSTs 2 patients were Von Recklinghausen’s disease and expired with regional recurrence and lung metastasis. Conclusion：The benign PNSTs can be treated with enucleation if possible and observation can be another choice to minimize neurologic sequele.