Sarcoma: Vascular Tumors (EHE and Angiosarcoma)

2017 ◽  
Author(s):  
Anne Grand'Maison ◽  
Kilian Salerno ◽  
John M Kane III
Keyword(s):  
Radiation Therapy ◽  
Adjuvant Chemotherapy ◽  
Metastatic Disease ◽  
Systemic Therapy ◽  
Primary Tumor ◽  
Epithelioid Hemangioendothelioma ◽  
Wide Resection ◽  
Vascular Tumors ◽  
Adjuvant Radiation ◽  
Chronic Lymphedema

Angiosarcoma (AS) and epithelioid hemangioendothelioma (EHE) are rare but clinically disparate vascular sarcomas. Common AS scenarios include the head and neck/scalp region in older patients or in association with chronic lymphedema or radiation therapy (often in the setting of breast cancer treatment). AS behaves like a high-grade tumor with a propensity for multifocality and hematogenous metastases. The potentially curative treatment is negative margin wide resection. Adjuvant radiation may be considered to does not reduce the risk of local recurrence. Unresectable localized tumor can be treated with definitive radiation therapy. Although data are limited, neoadjuvant and adjuvant chemotherapy are occasionally used. Extensive primary tumor or metastatic disease is treated with systemic therapy. EHE can arise in the liver, bone, lung, pleura, or skin/soft tissue and can also metastasize hematogenously. The tumor is frequently asymptomatic and can be clinically indolent. Therefore, specific therapy is reserved for documented progression. Limited primary tumor is treated with surgical resection and occasionally adjuvant radiation. There is no proven benefit to adjuvant chemotherapy. Liver-directed therapy and occasionally transplantation are used for unresectable hepatic disease. Metastatic disease is treated with systemic therapy, but only in the setting of clinical progression. Key words: angiosarcoma, breast, cutaneous, epithelioid hemangioendothelioma, lymphedema, neoadjuvant chemotherapy, radiation associated, visceral

Sarcoma: Vascular Tumors (EHE and Angiosarcoma)

2017 ◽  
Author(s):  
Anne Grand'Maison ◽  
Kilian Salerno ◽  
John M Kane III
Keyword(s):  
Radiation Therapy ◽  
Adjuvant Chemotherapy ◽  
Metastatic Disease ◽  
Systemic Therapy ◽  
Primary Tumor ◽  
Epithelioid Hemangioendothelioma ◽  
Wide Resection ◽  
Vascular Tumors ◽  
Adjuvant Radiation ◽  
Chronic Lymphedema

Angiosarcoma (AS) and epithelioid hemangioendothelioma (EHE) are rare but clinically disparate vascular sarcomas. Common AS scenarios include the head and neck/scalp region in older patients or in association with chronic lymphedema or radiation therapy (often in the setting of breast cancer treatment). AS behaves like a high-grade tumor with a propensity for multifocality and hematogenous metastases. The potentially curative treatment is negative margin wide resection. Adjuvant radiation may be considered to does not reduce the risk of local recurrence. Unresectable localized tumor can be treated with definitive radiation therapy. Although data are limited, neoadjuvant and adjuvant chemotherapy are occasionally used. Extensive primary tumor or metastatic disease is treated with systemic therapy. EHE can arise in the liver, bone, lung, pleura, or skin/soft tissue and can also metastasize hematogenously. The tumor is frequently asymptomatic and can be clinically indolent. Therefore, specific therapy is reserved for documented progression. Limited primary tumor is treated with surgical resection and occasionally adjuvant radiation. There is no proven benefit to adjuvant chemotherapy. Liver-directed therapy and occasionally transplantation are used for unresectable hepatic disease. Metastatic disease is treated with systemic therapy, but only in the setting of clinical progression. Key words: angiosarcoma, breast, cutaneous, epithelioid hemangioendothelioma, lymphedema, neoadjuvant chemotherapy, radiation associated, visceral

Genetic and immune divergence in pancreatic cancer lesions at the time of metastatic relapse compared to primary tumor.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 4140-4140
Author(s):  
Bereket Gebregziabher ◽  
Derek A. Oldridge ◽  
Emma E. Furth ◽  
Michael D. Feldman ◽  
Andrew J. Rech ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Adjuvant Chemotherapy ◽  
Rna Sequencing ◽  
Primary Tumor ◽  
Metastatic Tumors ◽  
Kras Mutations ◽  
Primary Tumors ◽  
Metastatic Relapse ◽  
Resected Tumor ◽  
First Relapse

4140 Background: Relapse of pancreatic ductal adenocarcinoma (PDA) is common even after complete resection and adjuvant therapy. Compared to the resected tumor, the biological characteristics of metastatic tumors at the time of first relapse are poorly understood. Methods: Whole-exome sequencing (WES) (250x) and bulk RNA sequencing were performed on samples from 30 patients with PDA. Paired primary tumor samples were obtained after R0 or R1 resection, and metastatic tumor samples were obtained by biopsy at the time of first relapse. 74.1% of patients had received adjuvant chemotherapy and radiation therapy, 7.4% had received adjuvant chemotherapy only, and 3.7% had received adjuvant radiation therapy only. Most common metastatic sites were liver and lung. The cohort was 60% male with a median age at diagnosis of 64 years. The vast majority of patients had stage IIA or IIB disease at diagnosis. Median disease-free survival was 481 days. Analysis used Freebayes (somatic variant calling), Kallisto (transcript quantification), Danaher et al. (cell type deconvolution), and antigen.garnish (neoantigen prediction). Results: High-quality WES and/or RNA sequencing were available for 27/30 patients. Among these were 16 pairs of primary and metastatic samples for WES and 15 paired samples for RNA sequencing. Median tumor purity was 32% (primary) and 42% (metastatic). KRAS mutations were present in 43/48 evaluable samples, with conserved KRAS mutations in 14/16 primary-metastatic pairs. Tumors were otherwise highly variable, with 13/16 patients developing oncogenic mutations in metastatic tumors that were undetected in primary tumors (BRCA1 [3/16], AKT3 [3/16], TP53 [2/16], ROS1 [2/16]). Overall, primary and metastatic tumors had similar tumor mutation burden and neoantigen production rate. However, neoantigens were highly variable at the peptide and gene level, with conservation rates of 2.73% and 11.57%, respectively, across primary-metastatic pairs. PDA transcriptomic subtype also differed across primary-metastatic pairs in all cases. Furthermore, metastatic tumors contained lower immune suppressive signal by transcripts and deconvolution (CTLA4: p = 0.0012, FOXP3: p = 0.0026, PDCD11: p = 0.012, regulatory T cells: p = 0.012), while myeloid cells were higher (CD33: p = 0.0067). Conclusions: With the exception of KRAS, metastatic PDA tumors at relapse contain new oncogenes, distinct neoantigens, and lower immune-suppressive signal compared to primary PDA tumors. These data suggest a potential clinical utility for tumor biopsies at the time of first metastatic relapse and caution against clinical decisions for relapsed, metastatic patients based solely on sequencing of the originally resected tumor.

Value of adjuvant radiation therapy in breast cancer patients with one to three positive lymph nodes undergoing a modified radical mastectomy and systemic therapy

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 507-507 ◽  
Author(s):  
S. Dawood ◽  
A. M. Gonzalez-Angulo ◽  
W. Woodward ◽  
F. Meric-Bernstam ◽  
K. Hunt ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Lymph Nodes ◽  
Systemic Therapy ◽  
Distant Recurrence ◽  
Axillary Lymph Nodes ◽  
Adjuvant Radiation ◽  
Axillary Lymph ◽  
Adjuvant Radiation Therapy ◽  
Increased Risk ◽  
Segmental Mastectomy

507 Background: Whether adjuvant radiation therapy should be utilized for patients (pts) with early stage breast cancer with up to 3 positive axillary lymph nodes treated with mastectomy and systemic therapy is controversial. This retrospective study was performed to determine if adjuvant radiation therapy had an impact on survival for this cohort of pts. Methods: 4240 pts with T1–2N0–1 breast cancers, diagnosed between 1980–2007, who underwent either mastectomy without adjuvant radiation therapy or segmental mastectomy with adjuvant radiation therapy were identified. All pts received systemic treatment. Women with >3 positive axillary lymph nodes were excluded. Overall (OS) and distant disease free survival (DDFS) were estimated using the Kaplan-Meir product method. Cox proportional hazards were used to determine associations between OS/DDFS and type of surgery after controlling for pt and disease characteristics. Results: 1336 (18.8%) had T1N0 disease, 1114 (26.27%) had T2N0 disease, 989 (23.33%) had T1N1 disease and 801 (18.89%) had T2N1 disease. Median follow-up was 54 months.5- year DDFS among women who underwent mastectomy and segmental mastectomy was 81% (95% 78%-83%) and 86% (95% CI 84%-87%), respectively (p < 0.0001). In the Cox analysis, pts who had mastectomy without radiation had a significantly increased risk of distant recurrence (HR= 1.39, 95% CI 1.14–1.70, p= 0.0013) than pts treated with segmental mastectomy and radiation. When looking at subgroups, no significant difference in DDFS was observed between the two groups in pts with lymph node negative disease. However, for pts with 1–3 positive lymph nodes, pts treated with mastectomy without radiation had significantly increased risk of distant recurrence compared to pts treated with segmental mastectomy with radiation (HR=1.614, 95% CI 1.198–2.177, p= 0.002). This difference was most pronounce in the subset of patients with T2N1 disease (HR= 1.794, 95% CI 1.220–2.637, p=0.003). Similar trends were observed for OS. Conclusions: This study provides provocative evidence for benefit of radiation therapy among pts with 1–3 positive axillary lymph nodes who are treated with surgery and systemic therapy. No significant financial relationships to disclose.

Hypofractionated adjuvant radiation therapy of soft tissue sarcoma in elderly patients.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e21521-e21521
Author(s):  
Ofer Merimsky ◽  
Viacheslav Soyfer ◽  
Benjamin W. Corn ◽  
Solomon Dadia ◽  
Yehuda Kollender
Keyword(s):  
Radiation Therapy ◽  
Side Effects ◽  
Acute Toxicity ◽  
Elderly Patients ◽  
Metastatic Disease ◽  
Ambulatory Setting ◽  
Adjuvant Radiation ◽  
Adjuvant Radiation Therapy ◽  
Local Recurrences

e21521 Background: Adjuvant radiation therapy is an essential part of combined limb sparing treatment of STS. The recommended dose of radiation lies in the range of 60 Gy in standard fractionation of 1.8-2 Gy. Elderly or medically unfit patients often have difficulty in completing 6-7w of daily treatment. A prolonged course of radiation may be interrupted by acute side effects, which sometimes demands further extension of the overall course or even discontinuation of treatment. We intended to evaluate the efficacy of a hypofractionated adjuvant approach with radiation therapy for STS in the elderly and debilitated patients. Methods: 21 elderly patients were treated with a short course of adjuvant RT (39 to 48 Gy, 3 Gy per fraction) for STS. The medical records of the patients were retrospectively reviewed for the local or distant recurrence and side effects of RT. Results: Overall, the hypofractionated irradiation regimen of 39-48 Gy in 13-16 fractions was well tolerated with only 3 patients developing Grade 2-3 acute toxicity (mainly dermatitis). Three patients suffered from delayed Grade 2-3 toxicity (chronic pain, skin atrophy, teleangiectasiae) scaled according to CTSC. The mean time from the surgery until the initiation of RT was 65 days (SD 21.6). Mean RT time was 18.4 (SD 3) days. No delay of treatment due to acute toxicity was registered. All patients except for one were able to receive RT in the ambulatory setting. With a mean follow-up of 532 days (SD: 325), three local recurrences (14%) were detected. Three of eight patients with distant metastases died of sarcoma (graph 1). One patient with metastatic disease in the lung received salvage stereo tactic radiation therapy and was still alive 6 month after completion of SBRT with no evidence of disease. At a mean 532 days of follow up three local recurrences (14%) were detected .Eight patients (38%) had lung metastases during the observed period. Three of them died from metastatic disease. The hypofractionated radiation was well tolerated with minimum long term side effects. Conclusions: Hypofractionated adjuvant radiation appears to be an effective treatment in terms of local control in elderly and debilitated patients.

scholarly journals Primary and secondary breast angiosarcoma: single center report and a meta-analysis

2019 ◽  
Vol 178 (3) ◽  
pp. 523-533 ◽  
Author(s):  
Yara Abdou ◽  
Ahmed Elkhanany ◽  
Kristopher Attwood ◽  
Wenyan Ji ◽  
Kazuaki Takabe ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Adjuvant Chemotherapy ◽  
Tumor Size ◽  
Meta Analysis ◽  
Systemic Review ◽  
Comprehensive Cancer Center ◽  
Survival Outcomes ◽  
Adjuvant Radiation ◽  
High Grade ◽  
Adjuvant Radiation Therapy

Abstract Background Primary and secondary breast angiosarcoma is a rare and aggressive malignancy with limited published literature. Optimal management is mostly based on expert opinion. Our study aims to describe a single institution experience with breast angiosarcoma and evaluate other publications on this topic to further clarify prognostic outcomes and treatment modalities in this disease. Methods Twenty two cases of breast angiosarcoma from Roswell Park Comprehensive Cancer Center were retrospectively analyzed. Additionally, a systemic review and meta-analysis was conducted to study the association between survival outcomes, overall survival (OS), and recurrence-free survival (RFS) in both primary (PAS) and secondary breast angiosarcoma (SAS). Results 9 PAS patients (41%) and 13 SAS patients (59%) were retrospectively analyzed. No significant differences were noted in tumor characteristics and survival outcomes between PAS and SAS. Treatment modality had no significant effects on survival outcomes although adjuvant chemotherapy demonstrated a trend towards improved RFS in high grade tumors. 380 PAS and 595 SAS patients were included in the outcome meta-analysis. Survival outcomes were significantly worse with high grade tumors and tumor size of > 5 cm. Adjuvant radiation therapy demonstrated significantly better RFS, while adjuvant chemotherapy had no effect on survival outcomes. Conclusion Tumor size and grade seem to be reliable predictors of survival in both PAS and SAS. Mastectomy does not seem to be adding any additional benefit to BCS. Adjuvant radiation therapy showed statistically significant RFS benefit, while adjuvant chemotherapy can be beneficial in high grade tumors.

Predictors of survival in rectal squamous cell carcinoma: An analysis from the National Cancer Database (NCDB).

2019 ◽  
Vol 37 (4_suppl) ◽  
pp. 672-672
Author(s):  
Sri Harsha Tella ◽  
Anuhya Kommalapati ◽  
Gaurav Goyal ◽  
Amit Mahipal
Keyword(s):  
Squamous Cell Carcinoma ◽  
Radiation Therapy ◽  
Cell Carcinoma ◽  
Surgical Resection ◽  
Squamous Cell ◽  
Primary Tumor ◽  
The United States ◽  
R0 Resection ◽  
Adjuvant Radiation ◽  
Stage 1

672 Background: Rectal squamous cell carcinoma (RSCC) is a rare form of gastrointestinal malignancy. Using the NCDB, we determined the prognostic factors and survival outcomes of RSCC in the United States. Methods: We identified histologically confirmed cases of RSCC from the National Cancer Data Base (2004-2015). Univariate and multivariable methods were used to assess factors associated with survival. Kaplan-Meier method and log-rank test were used to perform overall survival (OS) analysis. Results: Of the 5,527 cases included in our analysis, 67% were female. Median age at diagnosis was 61 years and did not differ by sex. The proportion of patients with stage 1, 2, 3 and 4 diseases were 22%, 16%, 20%, and 11%, respectively (30% unknown stage). Among the ones who received surgical resection of primary tumor, 41%, 30%, 20% and 8% are of stages 1, 2, 3 and 4 respectively. The rate of R0 resection was 54%, 63%, 55% and 38% in stage 1, 2, 3 and 4, respectively. The R0 resection rate was much higher in patients who received neoadjuvant chemo or radiation therapy or both (87%, 78%, 74%, and 57% in stages I, 2, 3 and 4, respectively) as compared to that of their counterparts. On stage wise sub-group OS analysis, stage 1-3 patients had OS benefit from surgery (as compared to no-surgery) (145 vs 90 months, p<0.001) as opposed to 4 disease (16 vs 11 months, p=0.06). Adjuvant radiation therapy improved the median OS in stage 1-3 patients (as compared to no-adjuvant radiation) in patients with positive surgical margins (not reached vs 40 months, p<0.0001). Patients with stage 4 disease treated with radiation therapy had a better median OS than those without (16 vs 6 months, p<0.0001). On therapy wise sub-group analysis, the patients who received surgery only had a median OS of 145 months; surgery + chemoradiation (adjuvant and neoadjuvant) = not reached; whereas patients with chemoradiation only had median OS of 80 months. Conclusions: This is the largest registry-based study on RSCC to date. RSCC had a diverse OS varied significantly according to stage of the disease at presentation and therapy received. Surgical resection of primary tumor was associated with improved OS as compared to that of patients who received chemoradiation.

The Prognostic Value of the Primary Tumor's Nodal Status after Surgery for Colorectal Liver Metastases in the Era of Effective Systemic Therapy

2015 ◽  
Vol 32 (3) ◽  
pp. 208-216 ◽  
Author(s):  
Eric P. van der Stok ◽  
Dirk J. Grünhagen ◽  
Wijnand J. Alberda ◽  
Maxime Reitsma ◽  
Joost Rothbarth ◽  
...  
Keyword(s):  
Colon Cancer ◽  
Lymph Node ◽  
Adjuvant Chemotherapy ◽  
Systemic Therapy ◽  
Primary Tumor ◽  
Prognostic Value ◽  
Colorectal Liver Metastases ◽  
Nodal Status ◽  
Lymph Node Status ◽  
Primary Colon

Background: The nodal status of primary colorectal cancer is of prognostic value for survival after the resection for colorectal liver metastases (CRLM). However, in the past decade, effective adjuvant chemotherapy for lymph node positive primary colon cancer was introduced. This study evaluated the prognostic value of primary lymph node status in patients with resectable metachronous CRLM in the era of effective systemic therapy. Methods: Between January 2000 and December 2011, all consecutive patients undergoing curative liver resection for CRLM were retrospectively analyzed. Overall survival (OS) was analyzed by the localization of the primary tumor (colon vs. rectum) and by lymph node status (positive vs. negative) of the primary tumor. Results: A total of 286 patients with metachronous CRLM's were selected. Five-year OS was similar for colon and rectal primaries (42 and 40%, p = 0.62). Lymph node positivity was only a prognostic factor in rectal primaries (N+ 32% vs. N0 49%, p = 0.04) and not in colon primaries (N+ 42% vs. N0 41%, p = 0.99). In multivariate analysis, these results were confirmed. Conclusion: The current study demonstrates that the nodal status of primary colon malignancies does not have prognostic value in patients undergoing resection for metachronous CRLM. A possible explanation might be the administration of effective adjuvant chemotherapy in node positive colon cancer.

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