• Title/Summary/Keyword: Malignant peripheral nerve sheath tumors

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Intraosseous Malignant Peripheral Nerve Sheath Tumor of Multiple Bones of the Midfoot: A Case Report (중족부에 발생한 다발성 악성 말초 신경초 종양 1예: 증례 보고)

  • Lee, Hyobeom;Kim, Gab-Lae;Kim, Donghyeon
    • Journal of Korean Foot and Ankle Society
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    • v.24 no.4
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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.

The Early Detection of Recurrence of Malignant Peripheral Nerve Sheath Tumor by Frequent Magnetic Resonance Imaging

  • Lee, Chang-Sub;Huh, Ji-Soon;Chang, Jee-Won;Park, Ji-Kang
    • 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.

Analysis According to Characteristics of 18 Cases of Brachial Plexus Tumors : A Review of Surgical Treatment Experience

  • Jung, In-Ho;Yoon, Kyeong-Wook;Kim, Young-Jin;Lee, Sang Koo
    • Journal of Korean Neurosurgical Society
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    • v.61 no.5
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    • pp.625-632
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    • 2018
  • Objective : Because the anatomical structure of the brachial plexus is very complex, surgical treatment of tumors in this region is challenging. Therefore, a lot of clinical and surgical experience is required for successful treatment; however, many neurosurgeons have difficulty accumulating this experience owing to the rarity of brachial plexus tumors. The purpose of this report is to share our surgical experience with brachial plexus tumor with other neurosurgeons. Methods : The records of 18 consecutive patients with brachial plexus tumors who underwent surgical treatment between January 2010 and December 2017 in a single institution were retrospectively reviewed. The surgical approach was determined according to the tumor location and size, and intraoperative neurophysiological monitoring (IONM) was used in most of cases to prevent iatrogenic nerve injury during surgery. In addition, to evaluate the differences in tumor characteristics according to pathologic diagnosis, the tumors were divided twice into two groups, based on two separate classifications, and statistical analysis was performed. Results : The 18 brachial plexus tumors comprised 15 (83.3%) benign peripheral nerve sheath tumors including schwannoma and neurofibroma, one (5.6%) malignant peripheral nerve sheath tumor, one (5.6%) benign tumor of non-neural sheath origin (neurogenic cyst), and one (5.6%) metastatic tumor (papillary carcinoma). The authors analyzed relationship between tumor size/location and tumor characteristic parameters such as age, size, right-left, and pathology. There were no statistically significant differences except a tendency of bigger tumor size in young age. Conclusion : For a successful surgical outcome, an appropriate surgical approach is essential, and the appropriate surgical approach is determined by the location and size of the tumor. Furthermore, applying IONM may prevent postoperative complications and it is favorable option for brachial plexus tumors surgery.

A Clinical Analysis of Peripheral Nerve Sheath Tumors in the Head and Neck Region (두경부 말초신경초종의 임상적 고찰)

  • Kang, Seok-Young;Shin, Myung-Chul;Yoo, Han-Seok;Lee, Yong-Seop;Park, Chul-Won;Tae, Kyung
    • 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.

Brachial Plexus Tumors in a Consecutive Series of Twenty One Patients

  • Go, Myeong-Hoon;Kim, Sang-Hyun;Cho, Ki-Hong
    • Journal of Korean Neurosurgical Society
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    • v.52 no.2
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    • pp.138-143
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    • 2012
  • Objective : This is a retrospective review of 22 surgically treated benign and malignant tumors of brachial plexus region to describe clinical presentation, the characteristics of brachial plexus tumor and clinical outcomes with a literature review. Methods : Twenty-one patients with consecutive 22 surgeries for primary brachial plexus tumors were enrolled between February 2002 and November 2011 were included in this study. The medical records of all patients were reviewed. Results : Eleven male and 10 female patients were enrolled. Mean age was 39 years. Three patients had brachial plexus tumor associated with neurofibromatosis (13.6%). Presenting signs and symptoms included parenthesis and numbness (54.5%), radiating pain (22.7%), direct tenderness and pain (27.2%), palpable mass (77.3%). Twelve patients presented preoperative sensory deficit (54.5%) and 9 patients presented preoperative motor deficit (40.9%). Twenty tumors (90.9%) were benign and 2 tumors (9.1%) were malignant. Benign tumors included 15 schwannomas (68.2%), 4 neurofibromas (18.2%) and 1 granular cell tumor (4.5%). There were 1 malignant peripheral nerve sheath tumor (MPNST) and 1 malignant granular cell tumor. Gross total resection was achieved in 16 patients (72.7%), including all schwannomas, 1 neurofibroma. Subtotal resection was performed in 6 tumors (27.3%), including 3 neurofibromatosis associated with brachial plexus neurofibromas, 1 MPNST and 2 granular cell tumor in one patient. Conclusion : Resection of tumor is the choice of tumor in the most of benign and malignant brachial plexus tumors. Postoperative outcomes are related to grade of resection at surgery and pathological features of tumor.

Malignant Peripheral Nerve Sheath Tumor of the Sciatic Nerve in a Patient with Neurofibromatosis - A Case Report - (신경섬유종증 환자의 좌골 신경에 발생한 악성 신경초종 - 증례 보고 -)

  • Song, Sang-Ho;Cho, Seong-Woo;Shim, Chang-Goo
    • The Journal of the Korean bone and joint tumor society
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    • v.6 no.2
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    • pp.77-81
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    • 2000
  • Malignant peripheral nerve sheath tumors(MPNSTs) are uncommon sarcomas that mostly arise in the soft tissue. They can develop from the pre-existing neurofibromas or schwannomas, or denovo from the peripheral nerves, or they can occur following the radiation therapy. We report a case of MPNST that developed in the sciatic nerve of the patient with neurofibromatosis type-1(NF-1). The patient was a 39-year-old man with the history of NF-1, who's main symptom was a rapidly enlarging painful mass in his posterior thigh. The well demarcated tumor, $6.5{\times}5{\times}4.5$cm in size, was composed of closely packed spindle cells. Since the patients with NF-1 have a high risk for developing a recurred MPNST, the importance of the clinical follow up is emphasized.

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Tumors in the Foot and Ankle (185 Cases) (족부 및 족근관절에 발생한 종양 (185예))

  • Choi, Woo-Jin;Shin, Kyoo-Ho;Lee, Jin-Woo;Han, Chang-Wook
    • Journal of Korean Foot and Ankle Society
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    • v.11 no.1
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    • pp.1-7
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    • 2007
  • Purpose: Tumors arising in the foot and ankle are uncommon and the malignant tumors are known to be rare compared with those of the other sites. We analyzed the clinical data of patients who have been diagnosed as having a tumor of the foot and ankle. Materials and Methods: From 1989 to 2006, we analyzed 185 patients who have been treated surgically and were pathologically confirmed of having tumors of the foot and ankle. Their clinical characteristics were reviewed retrospectively. Results: One hundred and fifty-seven cases were benign (84.9%) and 28 cases (15.1%) were malignant. 108 cases (58.4%) were benign soft tissue tumors and 49 cases (26.5%) were benign bone tumors. Malignant tumors included 17 cases (9.2%) of soft tissue tumors, 8 cases (4.3%) of primary bone tumors and 3 cases (1.6%) of metastatic bone tumors. The most common benign soft tissue tumor was ganglion (23 cases). Enchondroma (9 cases) was the most common among the benign bone tumors. Malignant peripheral nerve sheath tumor was the most common malignant tumor (4 cases). The predilection site for benign tumors was at the forefoot around toes while for the malignant tumor was around the ankle. 4.6% of benign soft tissue tumors and 8.2% of benign bone tumors had locally recurred and 14 cases (50%) of malignant tumor were confirmed as having distant metastasis. Conclusion: The ratio of malignant tumor and its metastasis rate was high. Therefore, the histopathologic confirmation is essential when treating tumors of the foot and ankle.

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Treatment of Malignant Peripheral Nerve Sheath Tumor Using Surgery and Metronomic Chemotherapy in a Dog (개에서 발생한 악성 말초 신경집 종양의 외과적 절제와 메트로놈 화학요법을 이용한 치료 증례)

  • Son, Jin-Na;Park, Seong-Kyu;Choi, Seok-Hwa;Kim, Gon-Hyung
    • Journal of Veterinary Clinics
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    • v.28 no.3
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    • pp.310-313
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    • 2011
  • A 6-year-old female Shih-tzu dog was referred with left elbow joint mass associated with weight bearing lameness. Ultrasonography demonstrated an encapsulated hyperechoic mass at the left elbow joint. Radiography was performed on elbow joint, chest, and abdomen, but there was no evidence of metastasis. Clinicopathologic examination revealed the existence of neoplastic cells with anisocytosis, pleomorphism and increased nuclear-cytoplasmic ratio. The left forelimb was amputated for cure. Histopathological examination diagnosed the mass as a malignant peripheral nerve sheath tumor. During the 4 weeks follow-up, the patient showed full remission and adopted to walk with remaining three legs. Thereafter, the tumor recurred after 5 months of first surgery at the operation site and other two cutaneuos regions. Cytology test revealed the mesenchymal originated tumor cells with malignancy. These tumors were surgically removed and histopathological examination of the resected tissue revealed the recurrence of primary tumor and metastasis. Metronomic therapy with cyclophophamide (10 mg/$m^2$, PO, sid) and piroxicam (0.3 mg/kg, PO, sid) had been adopted for 5 months. At 26 months of follow up after the first surgery, the dog alive with satisfactory quality of life. Aggressive surgical resection with metronomic chemotherapy should be the most effective treatment for malignant peripheral nerve sheath tumor.

Malignant Tumor of the Hand (수부에 발생한 악성 종양)

  • Park, Hong-Jun;Shin, Kyoo-Ho;Bae, Im-Don;Hahn, Soo-Bong;Kang, Eung-Shick
    • The Journal of the Korean bone and joint tumor society
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    • v.5 no.4
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    • pp.201-207
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    • 1999
  • Malignant tumors of the hand are very rare. Between 1989 and 1998, 17 patients with malignant tumors of the hand were evaluated for clinical features, prevalence, treatment and results. The mean age was 45 years and median duration of follow-up was 38 months. A painless mass was the most common symptom and the most common lesion was the digit. Fifteen cases of soft tissue and 2 cases of bone tumor were diagnosed. On pathology, soft tissue tumors were comprised of 8 cases of malignant melanoma, 2 cases of angiosarcoma and 1 case each of malignant fibrous histiocytoma, malignant peripheral nerve sheath tumor, extraskeletal chondrosarcoma, and squamous cell carcinoma. Bone tumors were metastatic tumors and each originated from the rectal cancer or lung cancer. Lymph node involvements were noted in 4 cases of malignant melanoma and 1 case of squamous cell carcinoma. Six cases of metacarpo-phalangeal joint disarticulation and 4 cases of phalanx amputation were performed. Wide excision, ray amputation and below-elbow amputation were also performed. Three cases expired due to metastasis and progression of the original lesion. Among the surviving 14 cases, a malignant melanoma had metastasis on the axillary lymph node and 13 cases showed no local recurrence or metastasis during the follow-up.

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Canine nervous-tissue tumors with features of peripheral nerve sheath tumor: histopathological and immunohistochemical findings (말초신경초 종양의 특징을 지닌 개 신경종의 조직병리학적 및 면역조직화학적 진단)

  • Lee, Sun-Gue;Lee, Jae-Ha;Han, Jeong-Hee
    • Korean Journal of Veterinary Service
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    • v.41 no.1
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    • pp.57-61
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    • 2018
  • Canine peripheral nerve sheath tumors (PNSTs) are spindle cell tumors that arise from Schwann cells, perineural cells, fibroblasts or all of them. Based on the morphology and biologic behavior, PNSTs are divided into benign PNST (BPNST) and malignant PNST (MPNST) forms. The aim of this study is to diagnose the two cases of neoplastic tissue samples with features of PNSTs by the histopathology and immunohistochemistry. The study was performed using two specimens from small animal clinic. The first case, A was a mass, 3~4 cm in diameter, extruded from vaginal mucosa of 10-year-old spayed female mixed-breed dog. And the second case, B was a subcutaneous mass, 1.5 cm in diameter, which is originated from right hind leg of 9-year-old castrated male mixed-breed dog. Two cases were stained with hematoxylin and eosin (H&E) for histopathological examination. And also immunohistochemistry (IHC) was performed by the avidin-biotin peroxidase complex (ABC) method with antibodies specific for the following proteins: S-100 protein, smooth muscle actin (SMA) and epidermal growth factor receptor (EGFR). In results, Antoni B schwannoma pattern characterized by pleomorphic, round and fusiform polygonal cells was seen in A. In B, Antoni A pattern, densely packed spindle cells arranged in interlacing bundles was seen in addition to Antoni B pattern. In IHC, cytoplasms of neoplastic cells were diffusely labeled for S-100 expression in A and B. For SMA, both A and B show negative expression. And for EGFR, A shows negative expression but B shows partially positive expression in areas of Antoni B schwannoma pattern. The histopathologic features of two cases coupled with the S-100 immunoreactivity led to a diagnosis of PNST. For SMA, both A and B show negative expression. The diagnosis of A will be a BPNST with the negative result and B will be a MPNST with the positive result for EGFR.