• Title/Summary/Keyword: Malignant Peripheral Nerve Sheath Tumor

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Ancient Schwannoma of the Thigh mimicking a Plexiform Malignant Peripheral Nerve Sheath Tumor: A Case Report (총상악성말초신경초종양으로 오인한 넓적다리에 생긴 고대 신경초종: 1예 보고)

  • Lee, Yeon-Soo;Park, Sang-Eun;Lee, Jung-Uee
    • Investigative Magnetic Resonance Imaging
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    • v.15 no.2
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    • pp.170-175
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    • 2011
  • Ancient schwannoma is a rare variant of schwannoma and a slow growing benign tumor associated with degeneration that may be diagnosed as a malignant tumor, because it presents with a large size and an inhomogeneous signal intensity. The main differential diagnosis of plexiform soft tissue tumor includes plexiform neurofibroma, malignant peripheral nerve sheath tumor (MPNST). In this case, we describe the MRI findings in a case of ancient schwannoma involving left thigh of a 63-year-old woman mimicking a plexiform MPNST. The tumor appeared as an inhomogeneous signal intensity and multinodular appearance, causing misdiagnosis as a plexiform MPNST.

Chest Wall Giant Malignant Peripheral Nerve Sheath Tumor -One case report (흉벽에 발생한 거대 악성 말초신경초종-치험 1례-)

  • Park, Jin-Gyu;Kim, Min-Ho;Jo, Jung-Gu
    • 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.

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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.

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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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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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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An Ectopic Hamartomatous Thymoma : A Case Report (경부에 발생한 이소성 과오종성 흉선종 1례)

  • Lee, Dong-Jin;Park, Su-Kyung;Kim, Han-Shin;Shin, Mi-Kyung;Chu, Hyung-Ro
    • Korean Journal of Bronchoesophagology
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    • v.14 no.2
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    • pp.48-52
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    • 2008
  • Ectopic Hamartomatous thymoma(EHT) is a rare benign tumor that occurs mainly in the supraclavicular or suprasternal area. Since this entity was first reported by Smith et al. in 1982, less than 50 cases have been reported in the literature. Recognition of EHT is important and needs to be differentiated from high-grade sarcomas such as synovial sarcoma or malignant peripheral nerve sheath tumor because EHT follows a benign clinical course. We experienced a case of ectopic hamartomatous thymoma in the suprasternal area in a 53-year-old man. Here, we present the case with a review of the related literatures.

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Application of alkaline phosphatase staining of cytology specimen for differential diagnosis of canine osteosarcoma (세포 검사시료에서 alkaline phosphatase 염색법을 활용한 개 골육종의 감별 진단)

  • Park, Byoung-Yong;Park, Chul;Cho, Ho-Seong
    • Korean Journal of Veterinary Service
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    • v.34 no.4
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    • pp.397-401
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    • 2011
  • Aspiration of lytic bone lesions is an excellent diagnostic test in the initial evaluation of primary bone tumor. However, cytologically, it can be difficult to differentiate osteosarcoma (OSA) from other bone neoplasms, including fibrosarcoma, chondrosarcoma, synovial cell sarcoma, malignant fibrous histiocytoma and malignant peripheral nerve sheath tumor. The purpose of this study is to introduce alkaline phosphatase (ALP) staining to differentiate OSA from other mesenchymal tumors. Tumors actively producing bone are specifically positive for ALP staining. Unstained, cytologic specimens were incubated for 10 minutes with nitroblue tetrazolium chloride/5-bromo-4-chloro-3-indolyl phosphate toluidine salt-phosphatase substrate. Among 20 cases of cytology specimen, 14 were positive for ALP staining and histopathology, 6 were negative for ALP staining and histopathology. ALP staining was 100% sensitive and specificity for the diagnosis of OSA. Aspirate cytology with ALP staining was a simple, fast, safe and accurate diagnostic test for the evaluation of suspected OSA lesions in dogs.

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.

Prognostic Factors of Soft Tissue Sarcomas - analysis of 205 cases - (연부 육종의 예후 인자 - 205례 분석 -)

  • Lee, Jong-Seok;Jeon, Dae-Geun;Lee, Soo-Yong;Kim, Sug-Jun;Jung, Dong-Whan;Park, Hyun-Soo
    • The Journal of the Korean bone and joint tumor society
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    • v.3 no.2
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    • pp.89-97
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    • 1997
  • Twenty hundred and five out of 266 patients who were registered in Korea Cancer Center Hospital from Mar. 1985 to Jan. 1994, were analyzed in the aspect of survival and local recurrence. Fifty one patients were excluded due to inadequate data and follow up. Prognostic factors for survival were evaluated statistically. One hundred and four cases were male, 101 female. Average age was 39.7(range 1 to 77) year with a peak incidence around 4th decade. The most frequent diagnosis was malignant fibrous histiocytoma(MFH)(24.1%). Liposarcoma, synovial sarcoma, rhabdomyosarcoma, malignant peripheral nerve sheath tumor and fibrosarcoma were relatively common diagnostic entities, in decreasing order. In location, extremity was 179(87.3%) and trunk 26(12.7%). Average follow up period was 7.5 years(6 months to 10 years). Actuarial 5 years and 10 years survival rate were 64.0% and 40.8% respectively. In univariate analysis with log-lank test, significant differences in survival rate were noted in histopathological diagnosis, size(10 cm), stage and metastasis. Age, sex, tumor location, tumor depth and local recurrence didn't affect the survival rate. Adjuvant chemotherapy and/or radiotherapy did not affect overall survival rate, but lowered the local recurrence rate when compared with surgery only. Surgical margin did not affect the survival rate, but local recurrence rate was different according to each margin; 5.7% in more than wide; 39.5% in marginal; and 60.0% in intralesional excision. In multivariate analysis for results of univariate analysis with Cox's propotional model, metastasis was a meaningful factor for survival of soft tissue sarcoma.

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