• The Journal of the Korean bone and joint tumor society
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• v.1 no.1
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• pp.77-83
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• 1995

Malignant fibrous histiocytoma was classified as fibrosarcoma, liposarcoma, rhabdomyosarcoma in times past and it was first introduced in 1963 to refer to a group of soft tissue tumors and reported in bone in 1972. It was postulated that the origin of tumor cell was derived from histiocytes that could assume the appearance and function of fibroblasts("facultative fibroblasts") at first. But, recently the immunohistochemical studies suggested origin from a primitive mesenchymal cell. The malignant fibrous histiocytoma were commonly misdiagnosed as other tumors such as myxoid liposarcoma, pleomorphic rhabdomyosarcoma, osteogenic sarcoma, leiomyosarcoma, malignant schwannoma, giant cell tumor, hemangiopericytoma, dermatofibrosarcoma protuberans due to the broad morphologic spectrum. The seventeen cases of the malignant fibrous histiocytomas diagnosed at Hanyang University Hospital since 1979 were reviwed, and the suggestions are as follows : 1. No fundamental histologic differences between the MFHs of soft tissues and bones. 2. The other tumors must be excluded in order to diagnose MFH due to wide histologic variety of MFH. 3. The review of the initial tissue slide was necessary for adequate diagnosis because the recurrent MFHs showed different histologic findings from original tumors.

• Korean Journal of Head & Neck Oncology
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• v.40 no.1
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• pp.49-53
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• 2024

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma, with an incidence of about 0.8% to 5% per million people per year, accounting for 1% of soft tissue sarcomas. In its early stage, DFSP is typically found as a violet or pinkish macule or patch, and it can develop into a palpable mass with ulceration or bleeding. The standard treatment for DFSP is wide local excision of the tumor with a 2- to 3-cm negative margin, and radiation therapy or chemotherapy can be conducted with surgical treatment. A 35-year-old man had a palpable mass on the left side of his occipital scalp without color change, ulceration, or bleeding, which typically are present in malignancy. A magnetic resonance imaging (MRI) scan showed a 3-cm homogenous enhanced mass without adhesion between the scalp and the mass. Unexpectedly, a biopsy revealed the round mass to be DFSP. A wide excision and rotation of the scalp flap were performed. The patient recovered without any complications and received adjuvant radiotherapy at a dose of 60 Gray (Gy) for six weeks. There was no recurrence through six months of follow-up. Here we report this unique case of DFSP with atypical presentation.

• Radiation Oncology Journal
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• v.26 no.3
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• pp.135-141
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• 2008

Purpose: To evaluate the clinical outcomes and prognostic factors in retroperitoneal soft tissue sarcomas treated by postoperative radiotherapy. Materials and Methods: The records of 23 patients with retroperitoneal soft tissue sarcomas, who underwent postoperative radiotherapy between 1985 and 2003, were analyzed. The median follow-up period was 77 months (range, $8{\sim}240$ months). A total of 21 patients presented with primary disease, and two patients presented with recurrent disease. Liposarcomas and leiomyosarcomas represented 78% of the diagnosed tumor cases. Moreover, 17 cases were of high grade (grade 2 or 3). The median tumor size was 13 cm (range, $3{\sim}50\;cm$). Complete excision was achieved in 65% of patients. The median radiation dose was 50.4 Gy (range, 45.0 to 59.4 Gy), with conventional fractionation. Results: The 5-year overall, local recurrence-free, and distant metastasis-free survival rates were 68%, 58%, and 71%, respectively. Eleven patients experienced local recurrence, while 9 patients experienced distant metastasis. The most common site for distant metastasis was the liver. A univariate analysis revealed that adjacent organ invasion and age (>60 years) as the significant risk factors contributing to the prediction of poor overall survival. Moreover, multivariate analyses indicated that adjacent organ invasion remained significantly associated with a higher risk of death. In addition, patient age (>60 years) was the other identified risk factor for local recurrence by univariate and multivariate analyses. Except for one case of grade 3 diarrhea, no patient suffered grade 3 or higher complications. Conclusion: Our results were comparable to previous reports in that adjacent organ invasion and patient age (>60 years) were significant predictors of poor survival and tumor recurrence, respectively.

• The Journal of the Korean bone and joint tumor society
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• v.1 no.1
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• pp.91-97
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• 1995

Synovial sarcoma is a malignant soft tissue tumor which is the most prevalent in adolescents and young adults between 10 and 40 years of age. It occurs primarily in the para-articular regions, usually in close association with tendon sheath, bursae and joint capsules. Favorable clinical factors are young age of the patients, tumor size smaller than 5cm, and distal rather than proximal location in the extremities. We analysed clinical findings of 13 cases of synovial sarcoma that had been experienced from January 1983 to December 1992. There were 8 females and 5 males, whose age was averaged as 28 years and 10 months ranging from 6 years to 54 years. The mean follow-up was 3 years(range : 9 months- 9 years 1 month). Palpable mass was frequent clinical symptom and lower extremity especially around the knee was the most prevalent site. Treatment modalities were the combination of surgery, radiotherapy and chemotherapy. Distant metastasis occured in 5(38%) cases : 4 cases to lung and 1 case to neck, and 2 cases had local recurrences. At final follow-up 6 cases were continous disease free, 2 alive with disease and 5 died of disease. The Kaplan-Meier's estimated 5 year survival rate of total 13 cases was 66% and satisfactory results were obtained with mass size smaller than 5cm.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.11
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• pp.4777-4780
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• 2015

Objective: To analyze clinicopathological characteristics, prognostic factors and survival rates of the patients with urological soft tissue sarcomas treated and followed up in Turkey. Materials and Methods: For overall survival analyses the Kaplan-Meier method was used. From medical records, nine prognostic factors on overall survival were analysed. Results: For the 53 patients (34 males, 19 females) whose charts were reviewed, the median age was 53 (range 22 to 83) years. Most frequently renal location (n=30; 56.6%) was evident and leiomyosarcoma (n=20, 37.7%) was the most frequently encountered histological type. Median survival time of all patients was 40.3 (95% CI, 14.2-66.3) months. In univariate analysis, male gender, advanced age (${\geq}50years$), metastatic stage, unresectability, grade 3, renal location were determined as worse prognostic factors. In multivariate analysis, metastatic stage, unresectability and grade 3 were determined as indicators of worse prognosis. Conclusions: Urological soft tissue sarcomas are rarely seen tumours in adults. The most important factors in survival are surgical resection, stage of the tumour at onset, grade and location of the tumour, gender and age of the patients.

• Asian Pacific Journal of Cancer Prevention
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• v.13 no.8
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• pp.4125-4128
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• 2012

Background: Soft tissue sarcomas (STS) must be managed with a team involving pathologists, radiologists, surgeons, radiation therapists and medical oncologists. Treatment modalities and demographic charasteristics of Turkish STS were analysed in the current study. Material-Methods: Primary adult STS followed between 1999-2010 in Cukurova University Medical Faculty Department of Medical Oncology were analzied retrospectively Results: Of the total of 498 patients, 238 were male and 260 female. The most seen adult sarcomas were leomyosarcoma (23%). Localization of disease was upper extremity (8.8%), lower extremity (24.7%), head-neck 8.2%, thoracic 8%, retroperitoneal 5.6%, uterine 12.4%, abdominal 10%, pelvic region 3.6 and other regions 10%. Some 13.1% were early stage, 10.2% locally advanced, 8.2% metastatic and 12.2% recurrent disease. Patients were treated with neoadjuvant/adjuvant (12%) or palliative chemotherapy (7.2%) and 11.4% patients did not receive chemotherapy. Surgery was performed as radical or conservative. The most preferred regimen was MAID combination chemotherapy in the rate of 17.6%. The most common metastatic site was lung (18.1%). The overall survival was 45 months (95%CI 30-59), 36 months in men and 55 months in women, with no statistically significant difference (p=0.5). The survival rates were not different between the group of adjuvant and palliative chemotherapy (respectively 28 versus 18 months) (p=0.06), but radical surgery at 37 months was better than 22 months for conservative surgery (p=0.0001). No differences were evident for localization (p=0.152). Locally advanced group had higher overall survival rates (72 months) than other stages (p=0.0001). Conclusion: STS can be treated successfully with surgery, chemotherapy and radiotherapy. The survival rates of Turkish people were higher in locally advanced group; these results show the importance of multimodality treatment approach and radical surgery.

• The Journal of the Korean bone and joint tumor society
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• v.15 no.1
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• pp.13-25
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• 2009

Purpose: The DNA repair protein, $O^6$-methylguanine-DNA methyltransferase (MGMT), removes alkyl adducts from the $O^6$ position of guanine. Epigenetic inactivation of MGMT has been found in human neoplasia and considered one of the implicated factors in chemoresistance. Materials and Methods: Sixty-two patiensts with soft tissue sarcomas (STS) were analyzed for the status of MGMT protein expression by immunohistochemistry and the promoter hypermethylation of the MGMT gene using methylation-specific PCR. Result: The loss of MGMT expression was found in 20 cases (32.3%) of total 62 STS. MGMT promoter hypermethylation rate was 25.0% (11/44 cases). The loss of MGMT expression showed significant association with high AJCC stage, high FNCLCC grade, and aggressive behavior. However,when the group who received chemotherapy was analyzed (n=27), loss of MGMT expression was correlated with worse survival in multivariate analysis (p=0.024). MGMT promoter hypermethylation is associated with high FNCLCC grade. MGMT promoter hypermethylation status had a strong correlation with loss of MGMT expression (p=0.000). Conclusion: Our results suggest that MGMT promoter hypermethylation and loss of MGMT expression had a tendency to be associated with poor prognosis and that loss of MGMT protein expression is frequently occurs via MGMT promoter hypermethylation.

• Asian Pacific Journal of Cancer Prevention
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• v.14 no.5
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• pp.2967-2971
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• 2013

Background: Extraskeletal Ewing sarcoma (EES)/primitive neuroectodermal tumours (PNET) are rare soft tissue sarcomas. Prognostic factors and optimal therapy are still unconfirmed. Materials and Methods: We performed a retrospective analysis on patients to explore the clinic characteristics and prognostic factors of this rare disease. A total of 37 patients older than 15 years referred to our institute from Jan., 2002 to Jan., 2012 were reviewed. The characteristics, treatment and outcome were collected and analyzed. Results: The median age was 28 years (range 15-65); the median size of primary tumours was 8.2 cm (range 2-19). Sixteen patients (43%) had metastatic disease at the initial presentation. Wide surgical margins were achieved in 14 cases (38%). Anthracycline or platinum-based chemotherapy was performed on 29 patients (74%). Radiotherapy was delivered in 13 (35%). At a median follow-up visit of 24 months (range 2-81), the media event-free survival (EFS) and overall survival (OS) were 15.8 and 30.2 months, respectively. The 3-year EFS and OS rates were 24% and 43%, respectively. Metastases at presentation and wide surgical margins were significantly associated with OS and EFS. Tumour size was significantly associated with OS but not EFS. There were no significant differences between anthracycline and platinum based chemotherapy regarding EFS and OS. Conclusions: EES/PNET is a malignant tumour with high recurrence and frequent distant metastasis. Multimodality therapy featuring wide surgical margins, aggressive chemotherapy and adjuvant local radiotherapy is necessary for this rare disease. Platinum-based chemotherapy can be used as an adjuvant therapy.

• Radiation Oncology Journal
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• v.7 no.2
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• pp.235-245
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• 1989

Rhabdomyosarcoma is a highly malignant soft tissue sarcoma that can arise in any site of the body containing striated muscle or its mesenchymal analgae. It is the most common childhood sarcoma with two peak age frequencies, one at ages 2 to 6, and one in the adolescence. The site, stage and extent of disease, and pathologic characteristics of the tumor contribute to prognostic factors that influence therapeutic decisions. The results of treatment of 52 patients with rhabdomyosarcoma, who were treated at Department of Radiation Oncology, Yonsei University College of Medicine, Yosei Cancer Center from 1976 to 1987 were retrospectively analyzed. The most frequent clinical group and primary sites were IRS group III (57.7%) and head and neck (42.3%) including orbit (11.5%) and parameningeal region(13.5%). The overall and disease free 5 year survival rate of eligible 41 patients were 31.7%, 29.3%, respectively. The complete remission (CR) rate was 50% in clinical group III and 0% in IV. Primary tumors of the orbit, clinical group 1 and embryonal subtype had the best prognosis. The Survival rate was improved by additiion of chemotherapy to operation and radiation therapy.

• Korean Journal of Otorhinolaryngology-Head and Neck Surgery
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• v.55 no.3
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• pp.181-184
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• 2012

The malignant peripheral nerve sheath tumor is an extremely rare soft tissue sarcoma. It is a highly malignant sarcoma, which is locally invasive, frequently leading to multiple recurrences and eventual metastatic spread. The peak incidence of disease is known to occur sporadically between the age of 20s and 50s, and is usually associated with the neurofibromatosis type I. In human body, the trunk and extremities are the most commonly involved sites, with only 8-14% of all lesions appearing in the head and neck region. We present a case of malignant peripheral nerve sheath tumor involving the right parapharynx in a 48-year-old patient who complained of headaches in the right parietal area and of dysphagia that aggravated over a month. After surgery, tumor was finally diagnosed as malignant peripheral nerve sheath tumor by histopathologic examinations. The authors report a case of malignant peripheral nerve sheath tumor in the right parapharynx with a review of the literature.