• 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 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.

• Korean Journal of Head & Neck Oncology
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• v.40 no.1
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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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• v.34 no.4
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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.

• 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.

• Journal of Chest Surgery
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• v.30 no.7
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• pp.729-732
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• 1997

A case of chest wall malignant peripheral nerve sheath tumor(MPNST) was reported in the U.S.A by Mark and coworkers6)(1991), but none in korea. MPNST accounts for approximately 10% of all soft tissue sarcoma, mostly in patients between 20 and 50 years of age. MPNST arises in association with a major nerve trunk, including the sciatic nerve, brachial plexus, and sacral plexus and the most common anatomical site is the proximal portion of the upper and lower extremity and trunk. Surgical treatment is local excision of mass in low grade sarcoma but unblock resection is necessary in high grade sarcoma. We experienced multiple huge low grade MPNST on left chest wall of a 50 years old man. The tumor and invalved chest wall were removed, and the chest wall defect(15$\times$8 cm) was reconstructed with Teflon. Postoperative course was unevenful.

• Archives of Plastic Surgery
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• v.45 no.5
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• pp.479-483
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• 2018

Malignant peripheral nerve sheath tumor (MPNST) is a very rare type of sarcoma, with an incidence of 0.001%. MPNST has a 5-year survival rate near 80%, so successful reconstruction techniques are important to ensure the patient's quality of life. Sarcoma of the forearm is known for its poor prognosis, which leads to wider excision, making reconstruction even more challenging due to the unique anatomical structure and delicate function of the forearm. A 44-year-old male presented with a large mass that had two aspects, measuring $9{\times}6cm$ and $7{\times}5cm$, on the dorsal aspect of the right forearm. The extensor compartment muscles (EDM, EDC, EIP, EPB, EPL, ECRB, ECRL, APL) and invaded radius were resected with the mass. Tendon transfer of the entire extensor compartment with skin defect coverage using a $24{\times}8cm$ anterolateral thigh (ALT) perforator free flap was performed. The patient was discharged after 18 days without wound complications, and has not complained of discomfort during supination, pronation, or wrist extension/flexion through 3 years of follow-up. To our knowledge, this is the first report of successful reconstruction of the entire forearm extensor compartment with ALT free flap coverage after resection of MPNST.

• Journal of Korean Neurosurgical Society
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• v.47 no.1
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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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• v.26 no.1
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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.