• Title/Summary/Keyword: Malignant Peripheral Nerve Sheath Tumor(MPNST)

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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.

Treatment and Survial Rate of Malignant Peripheral Nerve Sheath Tumors (악성 말초신경막 종양의 치료와 생존율)

  • Lee, Jong-Seok;Jeon, Dae-Geun;Cho, Wan-Hyung;Lee, Soo-Yong;Oh, Jung-Moon;Kim, Jin-Wook
    • 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.

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Simultaneous reconstruction of the forearm extensor compartment tendon, soft tissue, and skin

  • Oh, Jeongseok;Ahn, Hee Chang;Lee, Kwang Hyun
    • 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.

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.