• 대한두경부종양학회지
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• 제40권1호
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• pp.43-47
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• 2024

Malignant peripheral nerve sheath tumor (MPNST) is an extremely rare sarcoma of soft tissue. It is locally invasive, frequently recurred and metastasizes distantly, therefore has a very poor prognosis. the most involved sites are human body and extremities, and MPNST occurs and involved very rarely in the parapharynx of neck. MPNST does not react to radiotherapy and chemotherapy, therefore complete surgical resection is the most important treatment. Since immunohistochemical staining is essential for the final diagnosis, it is common to be confirmed after surgery. We report a case of giant MPNST without symptom in the head and neck involving the right parapharynx with multiple distant metastases in a 74-year-old patient on a review of the literature.

• Archives of Plastic Surgery
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• 제47권1호
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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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• 제34권4호
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• pp.495-497
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• 2007

Purpose: Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, usually arising from somatic soft tissues or peripheral nerves. Primary MPNST of the scalp is extremely rare. The case is being reported for its rarity. Methods: A 53-year-old female was presented with a scalp mass on vertex area. The tumor was localized in scalp skin and did not invade underlying periosteum or skull and treated with complete surgical excision followed by adjuvant chemotherapy and radiotherapy. Results: Histologically, the tumor showed malignant spindle cells with focal S-100 positivity on immunohistochemistry and a diagnosis of MPNST was made. Conclusion: Authors experienced a rare case of primary scalp MPNST and report the case.

• 대한족부족관절학회지
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• 제24권4호
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• pp.156-160
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• 2020

Malignant peripheral nerve sheath tumors (MPNSTs) usually arise in soft tissues; they are rarely found in the bone. This paper reports a case of MPNST in the foot and ankle joint involving the distal tibia, talus, calcaneus, navicular, medial intermediate, and lateral cuneiform, cuboid, and 2nd to 4th metatarsal bone. Palliative treatment was performed. The authors encountered a patient with intraosseous MPNST of the midfoot who presented with nonspecific clinical and radiologic findings. This case shows that a high index of suspicion and a histopathology examination, including immunohistochemistry, will be necessary for an accurate diagnosis.

• 대한수의학회지
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• 제54권1호
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• pp.7-12
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• 2014

Peripheral nerve sheath tumors (PNSTs) are heterogeneous tumor groups of peripheral nerves that originate from either Schwann cells or modified Schwann cells, fibroblasts, or perineural cells. In this study, signalment and clinical data such as tumor location and size were evaluated for 15 cases of PNSTs collected from local animal hospitals. The mean age of dogs with malignant PNST was higher than that of dogs with benign PNST. Additionally, the male to female ratio in dogs with PNST was 1 : 4. In dogs with PNST, the primary sites of involvement were the hindlimb, forelimb, around the mammary glands, the neck, and the abdomen. Histiopathologic examination revealed that eight PNSTs were benign and seven were malignant. The tumor cells were composed of loosely to densely arranged interlacing bundles and wavy spindle cells arranged in short bundles, palisading, and whirling. High mitotic figures, local invasion, multifocal necrosis and atypical multinucleated giant cells were observed in malignant PNST cases. All PNSTs showed immunoreactivity for vimentin and S-100. However, only 93.3% and 73.3% were immunoreactive for NSE and GFAP, respectively. Overall, these results indicated that immunohistochemical markers such as vimentin, S-100 and NSE could help confirm the diagnosis of canine PNSTs.

• 대한두경부종양학회지
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• 제39권2호
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• pp.45-48
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• 2023

Malignant peripheral nerve sheath tumor (MPNST) is one of the soft tissue sarcoma believed to originate from neural crest cells. The patients with neurofibromatosis type I (NF1) have about 8-13% of the lifetime risk of the malignant transformation. Neurofibroma on patients with NF1 can be surgically resected and has good prognosis if approach to the tumor is possible. We experienced a case of a 50-year-old woman with NF who had incompletely resected neurofibroma, which presented a rapid malignant transformation to MPNST, 3 months after the first surgery. We reported this case with a brief review of literature.

• Journal of Yeungnam Medical Science
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• 제36권1호
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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 Korean Neurosurgical Society
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• 제47권1호
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• pp.51-54
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• 2010

Surgery has a key role in the treatment of malignant peripheral nerve sheath tumors (MPNSTs), but the resectability of paraspinal MPNSTs is only 20%. Therefore, spinal MPNSTs show frequent recurrence and poor prognosis. Local recurrence is much more common than metastasis for MPNSTs, and surgery still has a key role in the treatment of local recurrence. Therefore, it is important that recurrence must be detected before resectability is lost. However, no evidence-based follow-up protocol has been established for MPNST. The authors performed gross total resection in a 34-year-old woman presented with thoracic MPNST. Adjuvant radiotherapy and chemotherapy were not administered since these adjuvant therapies generally do not improve survival in MPNST and may cause additional neurovascular damage. Instead, the authors monitored the primary site every 3 months using magnetic resonance imaging to detect local recurrence at the earliest opportunity. The tumor recurred locally on two occasions without overt symptoms at 21 and 24 months postoperatively. These recurrences were treated successfully by gross total removal.

• Archives of Reconstructive Microsurgery
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• 제26권1호
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• pp.23-25
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• 2017

The malignant peripheral nerve sheath tumor (MPNST) originates from neurofibromatosis type 1 (NF1). Because NF1 patients have many accompaniments with growth of additional masses, they usually overlook potential malignant changes in their masses. Our patient had two growing mass near the left elbow for several months; however, she ignored these masses until 7 days prior to writing this article, at which time they began bleeding. Traditionally, sarcoma including MPNST treatment consisted of amputation of the involved extremity. However, treatment now consists of surgical resection with adjuvant therapy. Therefore, we conducted resection of the mass and subsequent coverage with a local advancement flap. We believe that the most effective treatment for MPNST is early diagnosis and fast surgery, coupled with notification that there is always potential for malignant change in NF1 patient's masses.

• 대한영상의학회지
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• 제84권3호
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• pp.770-775
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• 2023

악성 말초 신경 초종은 드문 연부조직 종양으로, 주로 몸통, 사지, 두경부에 주로 위치하며, 유방에는 드물다. 저자들은 신경섬유종증 1을 가진 27세 여자 환자에서 유방으로 전이된 악성 말초 신경 초종 증례를 보고하고자 한다. 흉부 단층촬영에서 오른쪽 유방에 경계가 분명한 타원형의 약간 조영 증강되는 종괴가 보였다. 초음파에서 혈류와 증등도의 탄성을 가진 경계가 분명한 타원형의 비균질한 에코의 종괴로 보였다. 유방 종괴는 수술로 제거되었고, 악성 말초 신경 초종으로 최종 진단되었다. 드물지만, 악성 말초 신경 초종은 신경섬유종증 1을 가진 환자에서 유방 종괴의 감별진단에 포함되어야 한다.