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

Langerhans cell sarcoma is a rare, proliferative tumor of Langerhans cells, which shows cytologic characteristics and clinical features of malignant tumor. Langerhans cell sarcoma primarily occurs in lymph nodes, skin, lung, liver, and spleen. However, very few cases have been reported in the head and neck region. Because of its rarity, an optimal treatment approach is unknown; however, Langerhans cell sarcoma grows aggressively and shows a poor prognosis, such that a more aggressive and multi-modality treatment approach is necessary. Here, we report the case of a 36-year-old male with Langerhans cell sarcoma, who presented with a mass in the submandibular gland area and was treated with wide excision and postoperative radiotherapy.
Adult ; Head ; Humans ; Langerhans Cell Sarcoma ; Langerhans Cells ; Liver ; Lung ; Lymph Nodes ; Male ; Neck ; Prognosis ; Radiotherapy ; Sarcoma ; Skin ; Spleen ; Submandibular Gland

PURPOSE: The quality assurance (QA) chart rounds are multidisciplinary meetings to review radiation therapy (RT) treatment plans. This study focus on describing the changes in RT management based on QA round reviews in a single institution. MATERIALS AND METHODS: After 9 full years of implementation, a retrospective review of all patients whose charts passed through departmental QA chart rounds from 2007 to 2015. The reviewed cases were presented for RT plan review; subcategorized based on decision in QA rounds into: approved, minor modifications or major modifications. Major modification defined as any substantial change which required patient re-simulation orre-planning prior to commencement of RT. Minor modification included treatment plan changes which didn’t necessarily require RT re-planning. RESULTS: Overall 7,149 RT treatment plans for different anatomical sites were reviewed at QA rounds. From these treatment plans, 6,654 (93%) were approved, 144 (2%) required minor modifications, while 351 (5%) required major modifications. Major modification included changes in: selected RT dose (96/351, 27%), target volume definition (127/351, 36%), organs-at-risk contouring (10/351, 3%), dose volume objectives/constraints criteria (90/351, 26%), and intent of treatment (28/351, 8%). The RT plans which required major modification according to the tumor subtype were as follows: head and neck (104/904, 12%), thoracic (12/199, 6%), gastrointestinal (33/687,5%), skin (5/106, 5%), genitourinary (16/359, 4%), breast (104/2387, 4%), central nervous system (36/846, 4%), sarcoma (11/277, 4%), pediatric (7/251, 3%), lymphoma (10/423, 2%), gynecological tumors (2/359, 1%), and others (11/351, 3%). CONCLUSION: Multi-disciplinary standardized QA chart rounds provide a comprehensive and an influential method on RT plans and/ or treatment decisions.
Breast ; Central Nervous System ; Head ; Humans ; Jordan ; Lymphoma ; Methods ; Neck ; Peer Review ; Radiotherapy ; Retrospective Studies ; Sarcoma ; Skin

Ewing sarcoma is the second most frequently occurring malignant tumor of the bone and soft tissue in adolescents and young adults. Genetically, Ewing sarcoma is characterized by balanced chromosomal translocation in which a member of FET gene family is fused with an ETS transcription factor, with the most common fusion being EWSR1-FLI1 (85% of cases). Treatment of Ewing sarcoma is based on multidisciplinary approach (local surgery, radiotherapy and multiagent chemotherapy), which are associated with chronic late effects that may compromise quality of life of survivors. First line treatment includes combination of drugs incorporating doxorubicin, vincristine, cyclophosphamide, ifosfamide, etoposide, and dactinomycin. The beneficial role of high dose chemotherapy has been suggested in high-risk localized Ewing sarcoma patients, and the studies are being performed to investigate the role in metastatic disease. The 5-year overall survival for localized Ewing sarcoma has improved to reach 65% to 75%. But patients with metastatic disease have a 5-year survival rate of <30%, except for those with isolated pulmonary metastasis (approximately 50%). Patients with recurrent tumor have a dismal prognosis. Novel therapeutic strategies based on understanding of molecular mechanisms are needed to improve the outcome of Ewing sarcoma and to lessen the treatment-related late effects.
Adolescent ; Cyclophosphamide ; Dactinomycin ; Doxorubicin ; Drug Therapy ; Etoposide ; Humans ; Ifosfamide ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive, Peripheral ; Prognosis ; Quality of Life ; Radiotherapy ; Sarcoma, Ewing ; Survival Rate ; Survivors ; Transcription Factors ; Translocation, Genetic ; Vincristine ; Young Adult

BACKGROUND: Primary breast sarcoma (PBS) is rare, comprising approximately 1% of breast malignancies. Rhabdomyosarcoma (RMS) accounts for an extremely small proportion of PBSs, often leading to delayed histologic confirmation. METHODS: Upon reviewing Asan Medical Center’s pathology database between 2000 and 2018, 41 PBS cases were retrieved, including three cases of primary RMS of the breast. Their clinicopathological features were analyzed, and the literature related to PBS and primary RMS of the breast was reviewed. RESULTS: We identified three primary breast RMS cases from our institution database, comprising 7.3% of PBS: one case each of spindle cell/sclerosing RMS (ssRMS), alveolar RMS (aRMS), and embryonal RMS (eRMS). All cases involved adolescents or young adults (14, 16, and 25 years, respectively) who underwent mastectomy or radiotherapy and were confirmed using immunohistochemical testing for myogenin, desmin, and myogenic differentiation. The ssRMS patient experienced recurrence at the operation site 4 months post-surgery despite undergoing concurrent chemoradiotherapy. The aRMS patient had multiple metastases at diagnosis and showed FAX3-FOXO1 fusion transcripts; she died 22 months after the diagnosis. The eRMS patient had enlarged axillary lymph nodes; post-radiotherapy, the lesion recurred as multiple metastases to the bone and lung. She died 18 months post-diagnosis. CONCLUSIONS: Our experience on RMS cases suggests that spindle cell or small round cell malignancy in breasts of young female should raise suspicion for the possibility of primary or secondary RMS. To our knowledge, this is the second report of primary breast ssRMS and it may help clinicians who encounter this rare disease in the future.
Adolescent ; Arm ; Breast ; Chemoradiotherapy ; Chungcheongnam-do ; Desmin ; Diagnosis ; Female ; Humans ; Lung ; Lymph Nodes ; Mastectomy ; Myogenin ; Neoplasm Metastasis ; Pathology ; Radiotherapy ; Rare Diseases ; Recurrence ; Rhabdomyosarcoma ; Sarcoma ; Young Adult

PURPOSE: Soft tissue sarcoma (STS) is a rare and heterogeneous cancer with over 50 known subtypes. It is difficult to understand the role of adjuvant treatment in STS. We aimed to determine the benefits of adjuvant treatment for a rare STS subset: non-extremity STS with moderate chemosensitivity. MATERIALS AND METHODS: We reviewed medical records from Pusan National University Hospital and Kosin University Gospel Hospital, which had detailed pathological reports on patients diagnosed between 2006 and 2016. The most important inclusion criterion was resection with curative intent. We grouped STS by chemosensitivity based on reported data and analyzed non-extremity STS with moderate chemosensitivity. RESULTS: We investigated 142 patients with 20 pathological subtypes of STS. Eighty-six patients had extremity STS and 56 had non-extremity STS. Thirty-eight of 56 patients were categorized as having moderate chemosensitivity. Seventeen of 38 patients (44.7%) received adjuvant radiotherapy and 14 (36.8%) received adjuvant chemotherapy. A log-rank test showed longer disease-free survival (DFS) in the adjuvant radiotherapy group than in the group treated without adjuvant radiotherapy (not reached vs. 1.468 years, p = 0.037). Multivariate Cox proportional hazard analysis, with covariates including age, stage, resection margin, adjuvant chemotherapy, and adjuvant radiotherapy, revealed that adjuvant radiotherapy was associated with longer DFS (odds ratio = 0.369, p = 0.045). Overall survival was not correlated with adjuvant radiotherapy. CONCLUSION: Adjuvant radiotherapy may be associated with longer DFS in patients with non-extremity STS with moderate chemosensitivity.
Busan ; Chemotherapy, Adjuvant ; Disease-Free Survival ; Extremities ; Humans ; Medical Records ; Radiotherapy, Adjuvant ; Sarcoma

Myxofibrosarcoma is the most common soft tissue sarcoma that occurs in late adult life, mainly occurring in the lower extremities and trunk. However, head and neck myxofibrosarcoma is extremely rare. The most reliable treatment of adult soft tissue sarcoma is surgical resection with negative margin. A 79-year-old man presented with a left cheek mass first detected six months ago. The pathologic report of the mass showed that it was myxofibrosarcoma and consequently postoperative radiotherapy was done. However, distant and locoregional metastasis occurred postoperatively. We report this case with a brief review of literature.
Adult ; Aged ; Cheek* ; Head ; Histiocytoma, Malignant Fibrous ; Humans ; Lower Extremity ; Myxosarcoma ; Neck ; Neoplasm Metastasis ; Radiotherapy ; Sarcoma

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: 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

BACKGROUND: Advances in surgical techniques, implant technology, radiotherapy, and chemotherapy have increased the recovery chances of patients with bone sarcomas. Accordingly, patients' expectations on life quality have also increased, highlighting the importance of objective evaluation of the functional results of reconstruction. METHODS: Thirteen patients with distal femoral endoprosthesis, who had been followed for an average of 2.9 years were evaluated. Postural stability, daily energy expenditure, muscle power, and range of motion were the four parameters analyzed in this study. The Musculoskeletal Tumor Society (MSTS) score and Toronto Extremity Salvage Score (TESS) were used to assess postoperative function and examine correlations with other parameters. RESULTS: Patients had sedentary activities in 84% of their daily lives. They exhibited a slower speed in the walk across test and a higher sway velocity in the sit-to-stand test (p = 0.005). MSTS scores were significantly correlated with the daily energy expenditure and walking speed. CONCLUSIONS: Objective functional results acquired from various clinics will provide significant data to compare reconstruction techniques, rehabilitation protocols, and surgical techniques. In this way, it will be possible to satisfy the expectations of patients that increase in relation to enhanced recovery.
Drug Therapy ; Energy Metabolism* ; Extremities ; Femur ; Humans ; Quality of Life ; Radiotherapy ; Range of Motion, Articular ; Rehabilitation ; Sarcoma ; Walking