Surgical Management of SDH-Deficient Gastrointestinal Stromal Tumors (GIST): A National Cancer Database Review

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Background

To evaluate factors predicting surgical resection of SDH-deficient GIST and outcomes of adjuvant therapies. SDH-deficient GIST are very rare, comprising 5-7.5% of all GIST and most frequently occurring in the stomach or small bowel. Veterans who were exposed to burn pit toxins have an increased risk of developing these tumors. While most patients undergo surgery, there is limited information available regarding prognosis and survivability

Methods

The National Cancer Database was used to identify patients diagnosed with SDH-deficient GIST from 2004 to 2019 using histology code 8936 as assigned by the Commission on Cancer Accreditation program. Kaplan-Meier, ANOVA Chi-Square, and Logistic Regression tests were performed, and data were analyzed using SPSS version 29. Statistical significance was set at α = 0.05.

Results

721 patients with SDH-deficient GIST were queried, with 606 (84.05%) receiving surgical resection. Surgical patients experienced longer overall survival than non-surgical patients (116.3 months vs. 48.05 months, p< 0.001), with 248 (40.9%) patients undergoing a lobectomy and 29 (4.79%) patients undergoing a wedge/segmental resection. Patients who received wedge/segmental resection survived for 109.5 months while those who received a lobectomy survived for 108.6 months. Both surgeries showed a greater survival than other types of resections (p< 0.001). Of the initial sample, 42 (6.93%) patients received adjuvant chemotherapy, 3 (0.50%) patients received adjuvant radiation, and 3 (0.50%) patients received both. None of these adjuvants impacted overall survival. Stage I-II disease and well to moderately differentiated disease predicted an increased likelihood of receiving surgery (p< 0.001), while liver metastases predicted a decreased likelihood of receiving surgery (p< 0.001). Income status, race, insurance, facility type, and age were not significant predicting factors of receiving surgery.

Conclusions

Surgical resection of SDH-deficient GIST is associated with improved overall survival. Adjuvant therapies do not significantly improve survival over surgery alone. Patients with lower stage and grade of disease are more likely to receive surgery, while other social, economic, and demographic factors do not significantly affect the likelihood of receiving surgery. Surgical resection of SDH-deficient GIST is significantly associated with improved overall survival without the need for adjuvant therapies.

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Background

To evaluate factors predicting surgical resection of SDH-deficient GIST and outcomes of adjuvant therapies. SDH-deficient GIST are very rare, comprising 5-7.5% of all GIST and most frequently occurring in the stomach or small bowel. Veterans who were exposed to burn pit toxins have an increased risk of developing these tumors. While most patients undergo surgery, there is limited information available regarding prognosis and survivability

Methods

The National Cancer Database was used to identify patients diagnosed with SDH-deficient GIST from 2004 to 2019 using histology code 8936 as assigned by the Commission on Cancer Accreditation program. Kaplan-Meier, ANOVA Chi-Square, and Logistic Regression tests were performed, and data were analyzed using SPSS version 29. Statistical significance was set at α = 0.05.

Results

721 patients with SDH-deficient GIST were queried, with 606 (84.05%) receiving surgical resection. Surgical patients experienced longer overall survival than non-surgical patients (116.3 months vs. 48.05 months, p< 0.001), with 248 (40.9%) patients undergoing a lobectomy and 29 (4.79%) patients undergoing a wedge/segmental resection. Patients who received wedge/segmental resection survived for 109.5 months while those who received a lobectomy survived for 108.6 months. Both surgeries showed a greater survival than other types of resections (p< 0.001). Of the initial sample, 42 (6.93%) patients received adjuvant chemotherapy, 3 (0.50%) patients received adjuvant radiation, and 3 (0.50%) patients received both. None of these adjuvants impacted overall survival. Stage I-II disease and well to moderately differentiated disease predicted an increased likelihood of receiving surgery (p< 0.001), while liver metastases predicted a decreased likelihood of receiving surgery (p< 0.001). Income status, race, insurance, facility type, and age were not significant predicting factors of receiving surgery.

Conclusions

Surgical resection of SDH-deficient GIST is associated with improved overall survival. Adjuvant therapies do not significantly improve survival over surgery alone. Patients with lower stage and grade of disease are more likely to receive surgery, while other social, economic, and demographic factors do not significantly affect the likelihood of receiving surgery. Surgical resection of SDH-deficient GIST is significantly associated with improved overall survival without the need for adjuvant therapies.

Background

To evaluate factors predicting surgical resection of SDH-deficient GIST and outcomes of adjuvant therapies. SDH-deficient GIST are very rare, comprising 5-7.5% of all GIST and most frequently occurring in the stomach or small bowel. Veterans who were exposed to burn pit toxins have an increased risk of developing these tumors. While most patients undergo surgery, there is limited information available regarding prognosis and survivability

Methods

The National Cancer Database was used to identify patients diagnosed with SDH-deficient GIST from 2004 to 2019 using histology code 8936 as assigned by the Commission on Cancer Accreditation program. Kaplan-Meier, ANOVA Chi-Square, and Logistic Regression tests were performed, and data were analyzed using SPSS version 29. Statistical significance was set at α = 0.05.

Results

721 patients with SDH-deficient GIST were queried, with 606 (84.05%) receiving surgical resection. Surgical patients experienced longer overall survival than non-surgical patients (116.3 months vs. 48.05 months, p< 0.001), with 248 (40.9%) patients undergoing a lobectomy and 29 (4.79%) patients undergoing a wedge/segmental resection. Patients who received wedge/segmental resection survived for 109.5 months while those who received a lobectomy survived for 108.6 months. Both surgeries showed a greater survival than other types of resections (p< 0.001). Of the initial sample, 42 (6.93%) patients received adjuvant chemotherapy, 3 (0.50%) patients received adjuvant radiation, and 3 (0.50%) patients received both. None of these adjuvants impacted overall survival. Stage I-II disease and well to moderately differentiated disease predicted an increased likelihood of receiving surgery (p< 0.001), while liver metastases predicted a decreased likelihood of receiving surgery (p< 0.001). Income status, race, insurance, facility type, and age were not significant predicting factors of receiving surgery.

Conclusions

Surgical resection of SDH-deficient GIST is associated with improved overall survival. Adjuvant therapies do not significantly improve survival over surgery alone. Patients with lower stage and grade of disease are more likely to receive surgery, while other social, economic, and demographic factors do not significantly affect the likelihood of receiving surgery. Surgical resection of SDH-deficient GIST is significantly associated with improved overall survival without the need for adjuvant therapies.

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An NCDB Analysis of Factors Associated With the Receipt of Surgery in Myxoid/Round Cell Liposarcoma

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Background

Myxoid/round cell liposarcoma (MRCLS) is a rare soft tissue sarcoma originating from adipocytes and most commonly occurs in patients aged 20 to 40. Though slow-growing, MRCLS has a high propensity to metastasize. Complete surgical resection is central in the treatment of MRCLS. However, no significant study has analyzed the factors that predict the utilization of surgical therapy in MRCLS patients. This study also aims to characterize the effect of different treatment modalities on overall survival of these patients.

Methods

The National Cancer Database (NCDB) was used to identify patients diagnosed with MRCLS from 2004 to 2019 using the histology code 8852 as assigned by the Commission on Cancer Accreditation program. Kaplan-Meier, ANOVA Chi-Square, and Multilevel Logistic Regression were performed, and data were analyzed using SPSS version 29. Statistical significance was set at α = 0.05.

Results

5365 patients with MRCLS were queried. 4811 (89.8%) patients received surgery. Surgical patients experienced greater overall survival compared to nonsurgical patients (159.17 vs 93.72 months, p < 0.001). Wedge/segmental resection (1551 patients, 32.2%) and lobectomy resection (2724 patients, 56.6%) were associated with improved survival over other surgery types (OS =161.0 months, p < 0.001). Private insurance status and care at an academic facility were associated with an increased likelihood of receiving surgery (p< 0.001). Metastasis was associated with a decreased likelihood of receiving surgery (p< 0.001). On nominal regression, grades I-II, stages 1-3, and histologically well to moderately differentiated disease were associated with a greater likelihood of receiving surgery. Adjuvant therapy did not appear to impact survival.

Conclusions

This study reaffirms that tumor resection is associated with increased overall survival in MRCLS patients. Specifically, wedge/segmental and lobectomy surgery types are associated with improved outcomes. It appears that care at an academic facility, private insurance status, lower stage and grade of disease, and well-differentiated histology are correlated to an increased likelihood of receiving surgical treatment. Metastasis is associated with a decreased chance of receiving surgery. This research serves as the start to a better understanding of the factors involved in the receipt of tumor resection, as it is the mainstay of MRCLS treatment.

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Background

Myxoid/round cell liposarcoma (MRCLS) is a rare soft tissue sarcoma originating from adipocytes and most commonly occurs in patients aged 20 to 40. Though slow-growing, MRCLS has a high propensity to metastasize. Complete surgical resection is central in the treatment of MRCLS. However, no significant study has analyzed the factors that predict the utilization of surgical therapy in MRCLS patients. This study also aims to characterize the effect of different treatment modalities on overall survival of these patients.

Methods

The National Cancer Database (NCDB) was used to identify patients diagnosed with MRCLS from 2004 to 2019 using the histology code 8852 as assigned by the Commission on Cancer Accreditation program. Kaplan-Meier, ANOVA Chi-Square, and Multilevel Logistic Regression were performed, and data were analyzed using SPSS version 29. Statistical significance was set at α = 0.05.

Results

5365 patients with MRCLS were queried. 4811 (89.8%) patients received surgery. Surgical patients experienced greater overall survival compared to nonsurgical patients (159.17 vs 93.72 months, p < 0.001). Wedge/segmental resection (1551 patients, 32.2%) and lobectomy resection (2724 patients, 56.6%) were associated with improved survival over other surgery types (OS =161.0 months, p < 0.001). Private insurance status and care at an academic facility were associated with an increased likelihood of receiving surgery (p< 0.001). Metastasis was associated with a decreased likelihood of receiving surgery (p< 0.001). On nominal regression, grades I-II, stages 1-3, and histologically well to moderately differentiated disease were associated with a greater likelihood of receiving surgery. Adjuvant therapy did not appear to impact survival.

Conclusions

This study reaffirms that tumor resection is associated with increased overall survival in MRCLS patients. Specifically, wedge/segmental and lobectomy surgery types are associated with improved outcomes. It appears that care at an academic facility, private insurance status, lower stage and grade of disease, and well-differentiated histology are correlated to an increased likelihood of receiving surgical treatment. Metastasis is associated with a decreased chance of receiving surgery. This research serves as the start to a better understanding of the factors involved in the receipt of tumor resection, as it is the mainstay of MRCLS treatment.

Background

Myxoid/round cell liposarcoma (MRCLS) is a rare soft tissue sarcoma originating from adipocytes and most commonly occurs in patients aged 20 to 40. Though slow-growing, MRCLS has a high propensity to metastasize. Complete surgical resection is central in the treatment of MRCLS. However, no significant study has analyzed the factors that predict the utilization of surgical therapy in MRCLS patients. This study also aims to characterize the effect of different treatment modalities on overall survival of these patients.

Methods

The National Cancer Database (NCDB) was used to identify patients diagnosed with MRCLS from 2004 to 2019 using the histology code 8852 as assigned by the Commission on Cancer Accreditation program. Kaplan-Meier, ANOVA Chi-Square, and Multilevel Logistic Regression were performed, and data were analyzed using SPSS version 29. Statistical significance was set at α = 0.05.

Results

5365 patients with MRCLS were queried. 4811 (89.8%) patients received surgery. Surgical patients experienced greater overall survival compared to nonsurgical patients (159.17 vs 93.72 months, p < 0.001). Wedge/segmental resection (1551 patients, 32.2%) and lobectomy resection (2724 patients, 56.6%) were associated with improved survival over other surgery types (OS =161.0 months, p < 0.001). Private insurance status and care at an academic facility were associated with an increased likelihood of receiving surgery (p< 0.001). Metastasis was associated with a decreased likelihood of receiving surgery (p< 0.001). On nominal regression, grades I-II, stages 1-3, and histologically well to moderately differentiated disease were associated with a greater likelihood of receiving surgery. Adjuvant therapy did not appear to impact survival.

Conclusions

This study reaffirms that tumor resection is associated with increased overall survival in MRCLS patients. Specifically, wedge/segmental and lobectomy surgery types are associated with improved outcomes. It appears that care at an academic facility, private insurance status, lower stage and grade of disease, and well-differentiated histology are correlated to an increased likelihood of receiving surgical treatment. Metastasis is associated with a decreased chance of receiving surgery. This research serves as the start to a better understanding of the factors involved in the receipt of tumor resection, as it is the mainstay of MRCLS treatment.

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Survival and Treatment in Older Patients With Ewing Sarcoma

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BACKGROUND

Ewing sarcoma (EWS) is a malignancy which primarily arises in adolescence and has been studied extensively in this population. Much less is www.mdedge.com/fedprac/avaho SEPTEMBER 2023 • S23 known about the rare patient cohort over the age of 40 at diagnosis. In this study, we describe the survival outcomes and clinical characteristics of this population.

METHODS

This retrospective cohort study utilized the National Cancer Database (NCDB) to identify 4600 patients diagnosed between 2004 through 2019. Of these patients, 4058 were under the age of 40 and 542 were over 40. Multivariate Cox regression models and Kaplan- Meier curves were used to estimate survival from diagnosis to death between age groups. Chi-square tests were used to compare demographic and socioeconomic patient characteristics. IBM SPSS version 27.0 was used. p<0.05 was used to indicate statistical significance.

RESULTS

EWS patients older than 40 experienced worse survival outcomes compared to patients under the age of 40. 5-year survival was 43.5% for older patients vs. 64.5% for younger patients (p<0.05). A multivariate Cox proportional hazards model showed that age was independently associated with inferior survival. (HR 2.23; p<0.05). EWS patients over the age of 40 were more likely to have tumors originating from the vertebral column (16.2% vs. 9.6%; p<0.05), cranium (5.5% vs. 4.7%; p<0.05), and had a higher rate of axial tumors (43.3% vs. 32.4%; p<0.05) compared to patients under 40. Additionally, patients older than 40 experienced a significantly longer delay between the date of diagnosis and initiation of systemic treatment (29.85 days vs. 19.37 days; p<0.05). Despite presenting with larger tumors , older patients were less likely to undergo a surgical procedure of the primary site (47.6% vs. 52.2%; p<0.05) and had higher rates of micro- and macroscopic residual tumor following surgical resection.

CONCLUSIONS

An age over 40 is associated with decreased survival for patients with EWS. Due to the rarity of EWS in this cohort, the optimal role of systemic treatment remains unknown and has yet to be clearly elucidated. Consequently, our findings suggest that older patients receive disparities in treatment which may be contributing to decreased survival rates.

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BACKGROUND

Ewing sarcoma (EWS) is a malignancy which primarily arises in adolescence and has been studied extensively in this population. Much less is www.mdedge.com/fedprac/avaho SEPTEMBER 2023 • S23 known about the rare patient cohort over the age of 40 at diagnosis. In this study, we describe the survival outcomes and clinical characteristics of this population.

METHODS

This retrospective cohort study utilized the National Cancer Database (NCDB) to identify 4600 patients diagnosed between 2004 through 2019. Of these patients, 4058 were under the age of 40 and 542 were over 40. Multivariate Cox regression models and Kaplan- Meier curves were used to estimate survival from diagnosis to death between age groups. Chi-square tests were used to compare demographic and socioeconomic patient characteristics. IBM SPSS version 27.0 was used. p<0.05 was used to indicate statistical significance.

RESULTS

EWS patients older than 40 experienced worse survival outcomes compared to patients under the age of 40. 5-year survival was 43.5% for older patients vs. 64.5% for younger patients (p<0.05). A multivariate Cox proportional hazards model showed that age was independently associated with inferior survival. (HR 2.23; p<0.05). EWS patients over the age of 40 were more likely to have tumors originating from the vertebral column (16.2% vs. 9.6%; p<0.05), cranium (5.5% vs. 4.7%; p<0.05), and had a higher rate of axial tumors (43.3% vs. 32.4%; p<0.05) compared to patients under 40. Additionally, patients older than 40 experienced a significantly longer delay between the date of diagnosis and initiation of systemic treatment (29.85 days vs. 19.37 days; p<0.05). Despite presenting with larger tumors , older patients were less likely to undergo a surgical procedure of the primary site (47.6% vs. 52.2%; p<0.05) and had higher rates of micro- and macroscopic residual tumor following surgical resection.

CONCLUSIONS

An age over 40 is associated with decreased survival for patients with EWS. Due to the rarity of EWS in this cohort, the optimal role of systemic treatment remains unknown and has yet to be clearly elucidated. Consequently, our findings suggest that older patients receive disparities in treatment which may be contributing to decreased survival rates.

BACKGROUND

Ewing sarcoma (EWS) is a malignancy which primarily arises in adolescence and has been studied extensively in this population. Much less is www.mdedge.com/fedprac/avaho SEPTEMBER 2023 • S23 known about the rare patient cohort over the age of 40 at diagnosis. In this study, we describe the survival outcomes and clinical characteristics of this population.

METHODS

This retrospective cohort study utilized the National Cancer Database (NCDB) to identify 4600 patients diagnosed between 2004 through 2019. Of these patients, 4058 were under the age of 40 and 542 were over 40. Multivariate Cox regression models and Kaplan- Meier curves were used to estimate survival from diagnosis to death between age groups. Chi-square tests were used to compare demographic and socioeconomic patient characteristics. IBM SPSS version 27.0 was used. p<0.05 was used to indicate statistical significance.

RESULTS

EWS patients older than 40 experienced worse survival outcomes compared to patients under the age of 40. 5-year survival was 43.5% for older patients vs. 64.5% for younger patients (p<0.05). A multivariate Cox proportional hazards model showed that age was independently associated with inferior survival. (HR 2.23; p<0.05). EWS patients over the age of 40 were more likely to have tumors originating from the vertebral column (16.2% vs. 9.6%; p<0.05), cranium (5.5% vs. 4.7%; p<0.05), and had a higher rate of axial tumors (43.3% vs. 32.4%; p<0.05) compared to patients under 40. Additionally, patients older than 40 experienced a significantly longer delay between the date of diagnosis and initiation of systemic treatment (29.85 days vs. 19.37 days; p<0.05). Despite presenting with larger tumors , older patients were less likely to undergo a surgical procedure of the primary site (47.6% vs. 52.2%; p<0.05) and had higher rates of micro- and macroscopic residual tumor following surgical resection.

CONCLUSIONS

An age over 40 is associated with decreased survival for patients with EWS. Due to the rarity of EWS in this cohort, the optimal role of systemic treatment remains unknown and has yet to be clearly elucidated. Consequently, our findings suggest that older patients receive disparities in treatment which may be contributing to decreased survival rates.

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