• Korean Journal of Head & Neck Oncology
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• v.39 no.2
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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 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.

• 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.

• The Journal of the Korean bone and joint tumor society
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• v.9 no.2
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• pp.131-138
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• 2003

Purpose: We analyzed our malignant peripheral nerve sheath tumor (MPNST) cases to find out their oncologic results following by each treatment modalities. Materials and Methods: Thirty four patients with MPNST were registered in Korea Cancer Center Hospital from Feb. 1986 to Nov. 1996. Seventeen cases were male and 17, female. Average age was 41 years (range 18 to 74). Location of the tumor was as follows; 17 in lower extremity, 11 upper extremity, 4 trunk, and 2 retroperitoneum. Following the AJC classification, stage IA were 2 cases, stage IIA 2, stage IIB 6, stage III 16 and stage IV 8. Twenty six patients took operations and adjuvant chemotherapy and/or radiation therapy, 3 operation only and 3 adjuvant chemotherapy or radiation therapy. Average follow up period was 33.5 months (5.6 to 146.1). Kaplan-Meiyer method was done for survival curve, and log rank test for comparison analysis. Results: Fourteen cases were continuous disease free, 2 no evidence of disease, 2 alive with disease and 14 dead of disease states at final follow up. Actual 5-year and 10-year survival rates were 53.5%, 35.7%. Local recurrence rate after operation was 24.1%. 5-year survival rates of stage I/II/III were 100/85.7/55.9% and 2-year survival rate of stage IV was 14.3% (p=0.04). In 21 cases operated with stage II-III, wide margin (15cases) had 76.0% 5-year survival rate, and marginal or intralesional marigin (6cases) had 40.0%. The actual 5-year survival rate of the group which were done 4 or more cycles chemotherapy (8cases) was 71.4% and the actual 3-year survival rate less than 4cycles chemotherapy (6cases) was 83.3% (p=0.96). In 19 cases operated with stage II-III and which had no radiotherapy, marginal or intralesional margin (5cases) had 3 cases of local recurrences (60.0%), though wide margin (14cases) had 4 cases recurrences (28.6%). There was no local recurrence in 8cases which had pre-or post-operative radiotherapy. Conclusions: Surgical margin is an important factor in local recurrence. Resection margin has a tendency to influence the survival despite insufficient statistical significance. Conventional chemotherapy has no defnite statistical sigficance in the effect on local control and survival. Preoperative and postoperative radiotherapy has some positive effect on local control.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.15
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• pp.6545-6548
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• 2015

Background: Synovial sarcoma is a mesenchymal neoplasm that accounts for around 10% of all soft tissue sarcomas. The diagnosis of synovial sarcoma can be a challenging task, particularly with small biopsy specimens. Aim: We investigated transducer-like enhancer of split 1 (TLE1), monoclonal antibody, expression by immunohistochemical analysis in a group of 74 synovial sarcoma cases, 20 cases of MPNST, 12 cases of neurofibroma, 15 cases of schwannoma, 5 cases of MFH, 10 cases of lieomyosarcoma and 10 cases of solitary fibrous tumor. Materials and Methods: Whole tissue sections were examined: (39 biphasic and 35 monophasic). Nuclear immunoreactivity was scored as negative (<5% of cells positive), 1+(mild /5-25%), 2+ (moderate/25-50%), and 3+ (strong >50%). Results: Overall, 71 (96%) of 74 synovial sarcomas were positive for TLE1, including 37 biphasic (95%) and 34 monophasic (97%) tumors. Other spindle cell tumors showed very low or absent staining of TLE1. Conclusions: We conclude that TLE1 is a sensitive marker and can be a useful diagnostic marker for synovial sarcoma, particularly the monophasic forms.