Local Treatment Of Primary Tumor Research Articles

Significance of fusion status, Oberlin risk factors, local and maintenance treatment in pediatric and adolescent patients with metastatic rhabdomyosarcoma: Data of the European Soft Tissue Sarcoma Registry SoTiSaR.

Outcome of primary metastatic rhabdomyosarcoma (RMS) is poor. Certain risk factors as fusion status, Oberlin score, and local treatment of primary tumor are known to influence prognosis. Patients with metastatic RMS were treated according to Cooperative Weichteilsarkom Studiengruppe (CWS) guidance with chemotherapy (CHT), radiotherapy (RT) excluding total lung irradiation (TLI), complete resection of the primary tumor, and metastasectomy if possible. Kaplan-Meier estimators and Cox proportional hazard models were used to examine event-free survival (EFS) and overall survival (OS) involving also landmark analyses. In the European Soft Tissue Sarcoma Registry SoTiSaR (2009-2018), 211 patients were analyzed. Many patients had fusion-positive alveolar RMS (n=83; 39%). Median age was 9.4years [0.1-19.7years]. Treatment primarily consisted of CHT with CEVAIE (carboplatin, epirubicine, vincristine, actinomycin-D, ifosfamide, etoposide: 86%, other regimens: 14%), RT (71%), resection of primary tumor (37%), metastasectomy (19%), and lymph node sampling/dissection (21%). Maintenance treatment (MT) (oral trofosfamide, idarubicin, etoposide) was added in 74% of patients. Oberlin factors, fusion status, and MT were predictive for EFS and OS. MT with O-TIE was not improving outcome when adjusting for the immortal time bias. Local treatment of the primary tumor and radical irradiation (except TLI) improved EFS, not OS, when adjusting for the Oberlin score. Patients with fusion-negative alveolar RMS (n=9) had an excellent outcome with a 5-year EFS and OS of 100%, compared to patients with embryonal RMS (49%/62%), PAX7- (22%/47%) and PAX3/FOXO1-positive ARMS (10/13%), respectively (p<.001). Prognosis of metastatic RMS primarily depends on fusion status and Oberlin score. Fusion status needs to be considered in future trials to optimize treatment outcome. The role of radical irradiation needs further investigation.

Local Lung Mass Masquerading a Very Aggressive Extraskeletal Ewing Sarcoma Presenting as Bilateral Paraparesis in a Young Adult

Ewing sarcoma is typically seen in children involving long bones. Although well described, its presentation in extraskeletal tissues is relatively rare and is classified as an Ewing sarcoma family of tumors. They are mostly curable when they occur in children. An extraskeletal Ewing sarcoma in adults is uncommon, limiting the experience in adult oncologists. The biopsy is essential for definitive diagnosis, which shows small round blue cells that must be differentiated from lymphoma, embryonal rhabdomyosarcoma, and small cell carcinoma. Management is multimodal, involving surgery, radiation for local treatment of primary tumor, and systemic chemotherapy. A multidisciplinary approach, coupled with risk-adapted intensive neoadjuvant and adjuvant multi-agent chemotherapies and other modalities such as radiation and surgery for control of the primary site and metastatic disease, is needed. The primary multidrug chemotherapy regimen consists of alternating cycles of vincristine/doxorubicin/cyclophosphamide (VDC) and ifosfamide/etoposide (IE) given every 2 weeks with growth factor support. Prognosis and the 5-year survival rate are better for localized than the metastatic disease, and in metastatic disease, it is better for patients with lung metastasis than other metastatic disease sites. We describe a rare extraskeletal tumor arising from a lung that tested positive for Ewing sarcoma, also known as Askin’s tumor in a young adult. In our case, the tumor rapidly metastasized locally to involve the thoracic spine causing paraparesis. Timely diagnosis and early management are essential to improve outcomes. We also present how treatment can be delayed due to sepsis and emphasize the careful multispecialty approach’s importance. J Med Cases. 2020;11(12):388-393 doi: https://doi.org/10.14740/jmc3582

Local Lung Mass Masquerading a Very Aggressive Extraskeletal Ewing Sarcoma Presenting as Bilateral Paraparesis in a Young Adult

Ewing sarcoma is typically seen in children involving long bones. Although well described, its presentation in extraskeletal tissues is relatively rare and is classified as an Ewing sarcoma family of tumors. They are mostly curable when they occur in children. An extraskeletal Ewing sarcoma in adults is uncommon, limiting the experience in adult oncologists. The biopsy is essential for definitive diagnosis, which shows small round blue cells that must be differentiated from lymphoma, embryonal rhabdomyosarcoma, and small cell carcinoma. Management is multimodal, involving surgery, radiation for local treatment of primary tumor, and systemic chemotherapy. A multidisciplinary approach, coupled with risk-adapted intensive neoadjuvant and adjuvant multi-agent chemotherapies and other modalities such as radiation and surgery for control of the primary site and metastatic disease, is needed. The primary multidrug chemotherapy regimen consists of alternating cycles of vincristine/doxorubicin/cyclophosphamide (VDC) and ifosfamide/etoposide (IE) given every 2 weeks with growth factor support. Prognosis and the 5-year survival rate are better for localized than the metastatic disease, and in metastatic disease, it is better for patients with lung metastasis than other metastatic disease sites. We describe a rare extraskeletal tumor arising from a lung that tested positive for Ewing sarcoma, also known as Askin’s tumor in a young adult. In our case, the tumor rapidly metastasized locally to involve the thoracic spine causing paraparesis. Timely diagnosis and early management are essential to improve outcomes. We also present how treatment can be delayed due to sepsis and emphasize the careful multispecialty approach’s importance.

Prognostic value of site-specific metastases for patients with advanced intrahepatic cholangiocarcinoma: A SEER database analysis.

Intrahepatic cholangiocarcinoma (ICC) is an aggressive malignancy with poor prognosis and increasing incidence. Due to its asymptomatic manifestation, ICC often progresses to a metastatic stage on diagnosis. The current study attempted to evaluate the prognostic value of site-specific metastases in patients with metastatic ICC.Surveillance, Epidemiology, and End Results (SEER) database (2010–2015) was queried and metastatic ICC patients were classified according to the metastatic sites. Kaplan–Meier analysis was used for survival comparisons and multivariate analysis was performed to elicit characteristics independently associated with survival.A total of 1567 patients were identified and included in the analysis. Compared with those with multiple-site metastases, patients with single-site metastases had better prognostic outcomes. Among the single-site metastases, regional lymph nodes metastases had the best prognosis; liver metastases had better prognostic outcomes than bone metastases; no significant difference was found between lung and bone or liver metastasis. Local treatment of primary tumor might benefit patients with isolated lymph nodes metastases and few exceptional cases of patients with liver metastases.Different metastatic sites have distinct impact on the survival outcomes of patients with advanced ICC and highly selected subset of them might benefit from the local treatment of the primary tumor.

The clinical progress of the local treatment for metastatic prostate cancer

Recent clinical research shows that the local treatment of primary tumor can improve cancer-specific-survival and overall-survival for metastatic prostate cancer patients. Differ with lymph node metastasis, there is not enough high-level randomized controlled trial data to decide whether the prostate cancer patients with bone metastasis will be benefited from local treatment, meanwhile, it also needs to figure out the factors influencing prognosis of patients. How to efficiently combine multi local treatment with system treatment is a current research hot spot as well. Key words: Prostatic neoplasms/SC/TH; Review

Local treatment of breast in de novo stage IV breast cancer patients.

e11526 Background: Stage IV breast cancer is an incurable disease. Systemic therapy is usually the main treatment for these patients. Locoregional therapy, such as surgery or radiotherapy, is controversial. Recent studies suggested locoregional treatments of the primary breast cancer can provide some benefit for these patients. Methods: We conducted a chart review of de novo stage IV breast cancer patients at a cancer center hospital in TAIWAN from 1990 to 2008. A total of 276 patients were reviewed and 268 patients met the inclusion criteria. Tumor characteristics, anti-tumor treatments and survival were analyzed. Results: The median survival of 268 patients was 21.5 months. We divided these patients into two groups. There were 140 patients with less than 21.5 months of survival and 128 patients with more than 21.5 months of survival. In univariate analysis, infiltrating ductal carcinoma (p=0.002), ER-positive (p&lt;0.0001), PR-positive (p&lt;0.0001), and no overexpression of HER2 (p=0.0125) were associated with longer survival. The clinical primary tumor size (p=0.11) and positive axillary lymph node involvement (p=0.61) were not different significantly between two groups. About metastatic site, patients with liver mets (p&lt;0.0001) and lung mets (p=0.025) were associated with shorter survival. Bone mets (p=0.63) was not associated with survival. Patients receiving local treatment of primary tumor, including surgery (p&lt;0.0001) or locoregional radiation (p=0.0034), had longer survival. In multivariate analysis, patients who received surgery of primary breast cancer (HR=0.52, p=0.0006) or received systemic chemotherapy (HR=0.47, p=0.002) had better survival. In subgroup analysis, patients without liver metastasis who received surgery of primary breast cancer had longer overall survival significantly (p&lt;0.0001). In contrast, surgery to the primary breast cancer had no benefit in survival (p=0.91) in patients with liver metastasis. Conclusions: Our institutional experience suggests locoregional treatment for primary breast cancer appear to be beneficial for de novo breast cancer patients, especially those without liver mets.

The value of local treatment in patients with primary, disseminated, multifocal Ewing sarcoma (PDMES)

The value of local treatment in patients with primary, disseminated, multifocal Ewing sarcoma (PDMES) was investigated. We analyzed 120 patients registered into the European Ewing Tumor Working Initiative of National Groups (EURO-E.W.I.N.G. 99) trial at the trial center of Muenster from 1998 to 2006. Median age was 16.2 years. Local treatment of the primary tumor was surgery in 26 of 120 patients, surgery and radiotherapy in 21 patients, and definitive radiotherapy in 40 patients. For treatment of metastases, 6 of 120 patients received surgery; 9 patients, surgery and radiotherapy; and 33 patients, definitive radiotherapy. Forty-seven (39%) patients had local treatment of both the primary tumor and metastases, 41 (34%) patients of either the primary tumor or metastases, and 32 (27%) received no local therapy. Event-free survival (EFS) at 3 years was 0.24 (95% CI, 0.16-0.33). Univariate analyses demonstrated the impact of local therapy given to the primary tumor: 3-year EFS was 0.25 with surgery, 0.47 with surgery and radiotherapy, 0.23 with radiotherapy, and 0.13 when no local therapy was administered (P < .001). Three-year EFS in PDMES was also influenced by the local treatment: surgery, 0.33; surgery and radiotherapy, 0.56; radiotherapy, 0.35; no local therapy, 0.16 (P = .003). Three-year EFS was 0.39 in patients who received local treatment of both primary tumor and PDMES, compared with 0.17 in patients with any local treatment of either primary tumor or PDMES and 0.14 in patients with no local therapy (P < .001). Multivariate analysis showed absence of local treatment to be the major risk factor (HR = 2.21; P = .027; n = 20). Local therapy of involved sites is important for patients with PDMES and should complement systemic treatment whenever possible.