• Yonsei Medical Journal
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• v.59 no.9
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• pp.1049-1056
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• 2018

Purpose: Local recurrence is the most common cause of failure in retroperitoneal soft tissue sarcoma patients after surgical resection. Postoperative radiotherapy (PORT) is infrequently used due to its high complication risk. We investigated the efficacy of PORT using modern techniques in patients with retroperitoneal soft tissue sarcoma. Materials and Methods: Eighty patients, who underwent surgical resection for non-metastatic primary retroperitoneal soft tissue sarcoma at the Yonsei Cancer Center between 1994 and 2015, were retrospectively reviewed. Thirty-eight (47.5%) patients received PORT: three-dimensional conformal radiotherapy in 29 and intensity-modulated radiotherapy in nine patients. Local failure-free survival (LFFS), overall survival (OS), and RT-related toxicities were investigated. Results: Median follow-up was 37.1 months (range, 5.8-207.9). Treatment failure occurred in 47 (58.8%) patients including local recurrence in 33 (41.3%), distant metastasis in eight (10%), and both occurred in six (7.5%) patients. The 2-year and 5-year LFFS rates were 63.9% and 47.9%, respectively. The 2-year and 5-year OS rates were 87.5% and 71.1%. The 5-year LFFS rate was significantly higher in PORT group than in no-PORT group (74.2% vs. 24.3%, p<0.001). In multivariate analysis, PORT was the only independent prognostic factor for LFFS. However, there was no significant correlation between RT dose and LFFS. OS showed no significant difference between the two groups. Grade ${\leq}2$ acute toxicities were observed in 63% of patients, but no acute toxicity ${\geq}$ grade 3 was observed. Conclusion: PORT using modern technique markedly reduced local recurrence in retroperitoneal sarcoma patients, with low toxicity. The optimal RT technique, in terms of RT dose and target volume, should be further investigated.

• Journal of Veterinary Clinics
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• v.32 no.1
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• pp.115-119
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• 2015

An 11-year-old spayed female maltese was presented for abdominal distention, dysuria and dyschezia. Panting and heart murmur was found and abdominal palpation was difficult due to increased abdominal pressure. A soft tissue mass, $6{\times}3cm$ in size, was identified radiographically in pelvic canal, displacing the descending colon to the medioventral direction and the urinary bladder cranially. On ultrasonography, the mass consisted of homogeneous hypoechoic parenchyma containing the focal hyperechoic region ($1.6{\times}1.5cm$). The mass had distinct margin and no connection with adjacent organs. It was considered as a mass originating from the retroperitoneal cavity. Additional diagnostic procedures were not advanced because of the owner's request, and only a surgical excision of the mass was performed to alleviate the dysuria and dyschezia. Histopathologic examination and immunohistochemistry determined the mass as a soft tissue sarcoma and excluded hemangiosarcoma and osteosarcoma, both are the most common types of the retroperitoneal tumors. This report described non-vascular soft tissue sarcoma originating from the retroperitoneal cavity in a maltese dog.

• Radiation Oncology Journal
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• v.29 no.4
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• pp.260-268
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• 2011

Purpose: To evaluate the treatment outcome and prognostic factor after postoperative radiotherapy in retroperitoneal sarcoma. Materials and Methods: Forty patients were treated with surgical resection and postoperative radiotherapy for retroperitoneal sarcoma from August 1990 to August 2008. Treatment volume was judged by the location of initial tumor and surgical field, and 45-50 Gy of radiation was basically delivered and additional dose was considered to the high-risk area. Results: The median follow-up period was 41.4 months (range, 3.9 to 140.6 months). The 5-year overall survival (OS) was 51.8% and disease free survival was 31.5%. The 5-year locoregional recurrence free survival was 61.9% and distant metastasis free survival was 50.6%. In univariate analysis, histologic type (p = 0.006) was the strongest prognostic factor for the OS and histologic grade (p = 0.044) or resection margin (p = 0.032) had also effect on the OS. Histologic type (p = 0.004) was unique significant prognostic factor for the actuarial local control. Conclusion: Retroperitoneal sarcoma still remains as a poor prognostic disease despite the combined modality treatment including surgery and postoperative radiotherapy. Selective dose-escalation of radiotherapy or combination of effective chemotherapeutic agent must be considered to improve the treatment result especially for the histopathologic type showing poor prognosls.

• Radiation Oncology Journal
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• v.34 no.3
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• pp.216-222
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• 2016

Purpose: To evaluate the benefit of adjuvant radiation therapy (RT) for retroperitoneal liposarcoma (RPLS) following gross tumor removal. Materials and Methods: We reviewed 77 patients with primary RPLS surgically treated between January 2000 and December 2013. Cases with gross residual disease were excluded. Tumor grade was evaluated according to the French Federation of Cancer Centers Sarcoma Group (FNCLCC) system. Adjuvant RT was delivered to 32 patients (42%) using external beam RT alone. Median follow-up time was 36 months (range, 5 to 169). Results: Among 77 patients, 33 (43%) presented with well-differentiated, 31 (40%) with de-differentiated, 8 (10%) with myxoid/round and 4 (5%) with pleomorphic morphology. The RT group included less well-differentiated subtype than surgery group (28% vs. 53%). During follow up, 34 patients (44%) showed local recurrence. Local recurrence rate was lower in the RT group (38%) compared to the surgery group (49%). The 3-year local control rate (LC) was 55.6%, and the 3-year overall survival (OS) was 82.1%. Tumor histology and FNCLCC grade were significantly associated with local recurrence. There was no statistical significance of adding adjuvant RT in LC (p = 0.312). However, patients with tumor histology other than well-differentiated subtype showed marginally decreased local recurrence rate after adjuvant RT (3-year LC, RT 43.9% vs. no RT 35.3%; p = 0.087). Conclusion: RPLS patients receiving RT experienced less local recurrence. We suggest that the addition of adjuvant RT may be related to improvement of LCs, especially in patients with non-favorable histologic subtypes.

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.4
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• pp.1759-1767
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• 2016

Background: Uterine sarcoma is a group of rare gynecologic tumors with various natures, and different lines of treatment. Most have a poor treatment outcome. This study targeted clinical characteristics, treatment, overall survival (OS), progression-free survival (PFS), and prognostic factors in uterine sarcoma patients in one tertiary center for cancer care. Materials and Methods: Uterine sarcoma patients who were treated at the Department of Obstetrics and Gynecology, Faculty of Medicine Vajira Hospital between January 1994 and December 2014 were identified. Clinico-pathological data were analyzed. Prognostic outcomes were examined by Kaplan-Meier curves and Cox regression analysis. Results: We identified 46 uterine sarcoma patients: 25 carcinosarcoma (CS) (54.3%), 15 leiomyosarcoma (LMS) (32.6%), and 6 undifferentiated uterine sarcoma (UUS) (13.1%) cases. Mean age was $54.0{\pm}11.9years$ (range 25-82 years). Abnormal uterine bleeding was the most common presenting symptom (63.0%). Among 33 patients (71.7%) who had pre-operative tissue collected, diagnosis of malignancy was correct in 29 (87.9%). All patients received primary surgery and retroperitoneal lymph nodes were resected in 34 (73.9%). After surgery, 5 (10.9%) had gross residual tumors. Stage I disease was most commonly found (56.5%). Adjuvant treatment was given to 27 (58.7%), most commonly chemotherapy. After a median follow-up of 16.0 months (range 0.8-187.4 months), recurrence was encountered in 22 patients (47.8%). Median time to recurrence was 5.8 months (range1.0-105.5 months). Distant metastasis was more common than local or locoregional failure. The 2-year PFS was 45.2% (95% confidence interval [CI], 30.6%-59.7%) and the 2-year OS was 48.3% (95% CI, 33.3%-60.7%). Multivariable analyses found residual disease after surgery as a significant factor only for PFS. Conclusions: Uterine sarcoma is a rare tumor entity. Even with multimodalities of treatment, the prognosis is still poor. Successful cytoreductive surgery is a key factor for a good survival outcome.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.15
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• pp.6041-6046
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• 2014

Objective: Liposarcoma (LPS) is the most common soft tissue sarcoma and accounts for approximately 20% of all mesenchymal malignancies, often occurring in deep soft tissue of retroperitoneal space. Accurate preoperative diagnosis is therefore necessary. We explored whether computed tomography (CT) could be used to differentiate between the various types of retroperitoneal liposarcoma (RPLS). Method: Forty-seven cases of RPLS, diagnosed surgically and histologically, were analyzed retrospectively. CT features were correlated with postoperative pathological appearance. Results: The study radiologist identified 29, 11, 2, 2 and 3 RPLS as atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDL), dedifferentiated liposarcoma (DDL), myxoid/round cell liposarcoma (ML/RCL), pleomorphic liposarcoma (PL) and mixed-type liposarcoma. Analysis of CT scans revealed the following typical findings of the different subtypes of RPLS: ALT/WDL was mainly visible as a well-delineated fatty hypodense tumor with uniform density and integrity margin; DDL was marked by the combination of focal nodular density and hypervascularity. ML/RCL, PL and mixed liposarcoma showed malignant biological behaviour and CT findings need further studies. Conclusions: CT scanning can reveal important details including internal components, margins and surrounding tissues. Based on CT findings, tumor type can be roughly evaluated and biopsy location and therapeutic scheme guided.

• 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.

• Clinical and Experimental Pediatrics
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• v.57 no.11
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• pp.500-504
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• 2014

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor of intermediate malignancy with resemblance to Kaposi sarcoma. It occurs predominantly in pediatric age groups as a cutaneous lesion with focal infiltration into the adjacent soft tissue and bone. Although visceral involvement is very uncommon, several cases with bone, retroperitoneal, or mediastinal involvement have been described. KHE has been reported to occasionally occur in unusual sites such as the thymus, tonsils, larynx, paranasal sinuses, deltoid muscle, spleen, uterine cervix, thoracic spine, and even the breast. Multifocal KHE is an extremely rare entity with few reports available in the literature, none of which describes pulmonary involvement. Herein, we report a unique case of multifocal KHE in a 13-year-old boy presenting with a huge soft tissue mass in the upper extremity complicated by bilateral pulmonary nodules that developed into large, necrotic tumor masses.

• Korean Journal of Head & Neck Oncology
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• v.26 no.2
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• pp.256-258
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• 2010

Although liposarcoma is the second most common soft tissue sarcoma in adults, the incidence of liposarcoma in the head and neck is low. The histologic nature of liposarcoma is correlated clinically with treatment outcome, but histologic classification of liposarcoma is controversial. Well-differentiated liposarcoma and atypical lipoma are pathologic synonyms because they are identical lesions both morphologically and karyotypically. They represent the lowest grade lesions in the spectrum of liposarcoma. The terms "atypical lipoma" were introduced specifically to describe well-differentiated liposarcomas occurring in the extremities, because of their better prognosis in comparison with their retroperitoneal counterparts. We present 1 case of atypical lipomatous tumor of the neck.

• Archives of Plastic Surgery
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• v.38 no.4
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• pp.505-507
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• 2011

Purpose: Liposarcoma is the most common soft tissue sarcoma, and usually occurs on the thigh or in the retroperitoneal space, but rarely in the oral region. This report presents a case of liposarcoma of the cheek and includes a review of the literatures. Methods: A 21-year-old woman was admitted with a palpable mass in her cheek of about two years duration, which increased in size gradually initially, but had increased rapidly over the three months. There was no particular pain or tenderness. MRI showed a well-enhanced, well-defined mass, which suspected to be hemangioma. Results: The spherical, well-encapsulated mass was surgically excised. Biopsy results revealed myxoid liposarcoma. FDG PET-CT on the seventh postoperative day, revealed a minimal to mild FDG-uptake soft tissue lesion around the mass defect area without evidence of distant metastasis. The patient is being observed and undergoing radiation therapy. Conclusion: Liposarcoma in the head and neck region is a rare disease, and can be overlooked as a benign tumor without a pathologic diagnosis. Therefore, proper treatment and follow-up are required based on an understanding of this disease.