Myxofibrosarcoma is a rare tumor, refractory to cytotoxic chemotherapy and radiotherapy. Pembrolizumab is an innovative immunotherapy drug consisting of programmed death receptor ligand 1 antibody proven to be useful for numerous types of cancer cells. A patient had been diagnosed with metastatic myxofibrosarcoma, refractory to radiotherapy and conventional cytotoxic chemotherapy. The patient achieved a partial response during palliative chemotherapy with pembrolizumab for 14 cycles. To the best of our knowledge, this is the first case report demonstrating the efficacy of pembrolizumab for refractory myxofibrosarcoma.
Drug Therapy ; Humans ; Immunotherapy ; Radiotherapy ; Sarcoma

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.
Follow-Up Studies ; Humans ; Liposarcoma* ; Radiotherapy ; Recurrence ; Sarcoma

We report a case of classic Kaposis sarcoma(KS) in a 74-year-old man who had asymptomatic red-dish-blue or dark-brown macules, papules and nodules on his bo hower extrimities. The biopsy specimen showed typical histopathologic features of FS including a highly vascularized lesion with a proliferation of spindle-shaped cells accompanied by etravasated erythrocytes and variable number of inflammatery cells. Many of the spindle-shaped cells associated with the proliferating vessels in KS lesion expressed factor VIII-related antigen. He was treated with 1500 cGy of radiation(300 cGy per day for 5 successive day. Four weeks after radiotherpy, gross and histopathologic findings were mucl improved and healed leaving hyperpigmentation.
Aged ; Biopsy ; Erythrocytes ; Humans ; Hyperpigmentation ; Radiotherapy* ; Sarcoma* ; von Willebrand Factor

PURPOSE: To analyze the prognostic factors for survivals and to evaluate the impact of postoperative whole pelvic radiotherapy (WPRT) on pelvic failure in patients with uterine sarcoma treated with radical surgery. MATERIALS AND METHODS: We retrospectively analyzed 75 patients with uterine sarcoma who underwent radical surgery with (n = 22) or without (n = 53) radiotherapy between 1990 and 2010. There were 23 and 52 patients with carcinosarcoma and non-carcinosarcoma (leiomyosarcoma, 22; endometrial stromal sarcoma, 25; others, 5), respectively. The median follow-up period was 64 months (range, 17 to 269 months). RESULTS: The 5-year overall survival (OS) and pelvic failure-free survival (PFFS) of total patients was 64.2% and 83.4%, respectively. Multivariate analysis revealed that mitotic count (p = 0.006) was a significant predictor of OS. However, factors were not found to be associated with PFFS. On analyzing each of the histologic subtypes separately, postoperative WPRT significantly reduced pelvic failure in patients with carcinosarcoma (10.0% vs. 53.7%; p = 0.046), but not in patients with non-carcinosarcoma (12.5% vs. 9.9%; p = 0.866). Among the patients with carcinosarcoma, 4 patients (17%) had recurrence within the pelvis and 3 patients (13%) had recurrence in other sites as an initial failure, whereas among the patients with non-carcinosarcoma, 3 patients (6%) experienced pelvic failure and 13 patients (25%) experienced distant failure. CONCLUSION: The most significant predictor of OS was mitotic count. Based on the improved PFFS after postoperative WPRT only in patients with carcinosarcoma and the difference in patterns of failure between histologic subtypes, optimal adjuvant treatment options should be offered to patients based on the risk of recurrence patterns.
Carcinosarcoma ; Follow-Up Studies ; Humans ; Multivariate Analysis ; Pelvis ; Radiotherapy ; Radiotherapy, Adjuvant ; Recurrence ; Retrospective Studies ; Sarcoma* ; Sarcoma, Endometrial Stromal ; Uterus

Synovial sarcoma (SS) is a highly malignant tumor that accounts for 10% of all soft-tissue sarcomas. Primary SS arising from the lung is extremely rare, and the prognosis is poor. We report a case of pulmonary SS presenting with a mass lesion invading the right upper and middle lobes, extending to the mediastinum and the chest wall. After tru-cut biopsy, surgical resection was performed. The final diagnosis was SS (biphasic type) based on histological and immunohistochemical findings. There are no guidelines for optimal treatment due to the rarity of these tumors. Current treatment includes surgery and adjuvant chemotherapy and/or radiotherapy.
Biopsy ; Chemotherapy, Adjuvant ; Diagnosis ; Immunohistochemistry ; Lung* ; Mediastinum ; Prognosis ; Radiotherapy ; Sarcoma ; Sarcoma, Synovial* ; Thoracic Wall

A case of metastatic alveolar soft part sarcoma is presented with clincal, pathological and radiological features. Alveolar soft part sarcoma is a rare soft-tissue neoplasm that is malignant and invariably fatal. It was first described and named by Christopherson, et al. in 1952. Since 1952 numerous examples of this tumor have been reported and have been studied with the electron microscope, but there is still considerable uncertainty as to the exact histogenesis of the tumor. Most cases occur in young females involving the lower extrimities especially in the right side. The most initial presenting symptom is a slowgrowing painless mass and the principal metastatic sites are the lungs, followed by the brain and skeleton. Cerebral metastasis, in fact, may be the first manifestation of the disease. Treatment is radical surgical excision but radiotherapy and chemotherapy are less effective. We present the reported case of metastatic alveolar soft pat sarcoma with electron microscopic findings.
Brain ; Drug Therapy ; Female ; Humans ; Lung ; Neoplasm Metastasis ; Radiotherapy ; Sarcoma ; Sarcoma, Alveolar Soft Part* ; Skeleton ; Uncertainty

Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor that accounts for 5% of pediatric non-rhabdomyosarcoma soft tissue sarcomas. The disease presents as a indolent asymptomatic mass and affects primarily younger patients. The clinical course is often indolent and late metastases can develop even after prolonged disease-free intervals. Radiotherapy and chemotherapy play a very limited role in the treatment. We report a pediatric case of ASPS with a brief review of the literature.
Child* ; Drug Therapy ; Humans ; Neoplasm Metastasis ; Radiotherapy ; Sarcoma ; Sarcoma, Alveolar Soft Part* ; Viperidae

Humans ; Extremities ; Sarcoma/radiotherapy* ; Soft Tissue Neoplasms/radiotherapy* ; Neoplasm Recurrence, Local

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 ≤2 acute toxicities were observed in 63% of patients, but no acute toxicity ≥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.
Follow-Up Studies ; Humans ; Multivariate Analysis ; Neoplasm Metastasis ; Radiotherapy* ; Radiotherapy, Conformal ; Radiotherapy, Intensity-Modulated ; Recurrence ; Retrospective Studies ; Sarcoma* ; Treatment Failure

Synovial sarcoma is a rare tumour found in soft tissue; it is a mesenchymal spindle cell tumour that is not related to the synovial membrane. This tumour has a low incidence, and the most frequent place of occurrence is the lower extremities in young adults. Synovial sarcoma of the head and neck accounts for 3%-5% of sarcomas in this anatomical region. The tumor in the nasal cavity is less than 1%. The treatment of choice for synovial sarcoma of the head and neck is complete surgical excision of the tumour mass followed by adjuvant radiotherapy.
Humans ; Nasal Cavity ; pathology ; Nose Neoplasms ; diagnosis ; radiotherapy ; surgery ; Paranasal Sinuses ; pathology ; Radiotherapy, Adjuvant ; Sarcoma, Synovial ; diagnosis ; radiotherapy ; surgery