Kang BY

Background: Chondrosarcoma (CHS) is the second most common primary malignant solid bone tumor. Present management of CHS is based largely on case series from individual treatment centers. This study summarizes descriptive epidemiologic and prognostic characteristics associated with CHS patients utilizing the National Cancer Database (NCDB).

Methods: We identified 6,559 patients with CHS of the bone, joints, and/or articular cartilage in the NCDB from 2004-2015. Kaplan-Meier analyses were used to estimate 2- and 5-year survival, and median survival. Survival comparisons were made using log-rank tests.

Results: Median age at diagnosis was 53 years. 53.3% of cases were in males, and 86.4% were in white patients. 28.2% of cases were in lower limb long bones and associated joints. The most common NCDB analytic stage at presentation was stage I (60.5%). 6.4% of patients had known metastases at diagnosis. 86.6%, 7%, and 12.5% of patients received surgery, chemotherapy, and radiation, respectively. Overall probability of 5-year survival was 74.4%. Approximate 5-year survival for NCDB analytic stages I-IV was 86.2%, 53.1%, 52.4%, and 17.4%, respectively. The dedifferentiated subtype of CHS had the poorest 5-year survival (25.4%), while juxtacortical had the highest (93.4%). In 355 patients with high grade CHS, perioperative chemotherapy (33.7% 5-year survival) did not provide improved survival compared to surgery alone (51.2% 5-year survival).

Conclusions: To the best of our knowledge, this is the first NCDB study to evaluate survival by treatment type and stage in CHS, as well as to analyze epidemiologic and prognostic characteristics in all CHS subtypes. This study found CHS was most common in white patients, had a median age at diagnosis of 53 years, and was most frequently found in lower limb long bones and associated joints. The 5-year survival was much worse at stage IV compared to stages I, II, and III, as well as in dedifferentiated and mesenchymal tumor subtypes compared to conventional or other types of CHS. In high grade tumors, perioperative chemotherapy did not improve survival compared to surgery alone.

Background: Chondrosarcoma (CHS) is the second most common primary malignant solid bone tumor. Present management of CHS is based largely on case series from individual treatment centers. This study summarizes descriptive epidemiologic and prognostic characteristics associated with CHS patients utilizing the National Cancer Database (NCDB).

Methods: We identified 6,559 patients with CHS of the bone, joints, and/or articular cartilage in the NCDB from 2004-2015. Kaplan-Meier analyses were used to estimate 2- and 5-year survival, and median survival. Survival comparisons were made using log-rank tests.

Results: Median age at diagnosis was 53 years. 53.3% of cases were in males, and 86.4% were in white patients. 28.2% of cases were in lower limb long bones and associated joints. The most common NCDB analytic stage at presentation was stage I (60.5%). 6.4% of patients had known metastases at diagnosis. 86.6%, 7%, and 12.5% of patients received surgery, chemotherapy, and radiation, respectively. Overall probability of 5-year survival was 74.4%. Approximate 5-year survival for NCDB analytic stages I-IV was 86.2%, 53.1%, 52.4%, and 17.4%, respectively. The dedifferentiated subtype of CHS had the poorest 5-year survival (25.4%), while juxtacortical had the highest (93.4%). In 355 patients with high grade CHS, perioperative chemotherapy (33.7% 5-year survival) did not provide improved survival compared to surgery alone (51.2% 5-year survival).

Conclusions: To the best of our knowledge, this is the first NCDB study to evaluate survival by treatment type and stage in CHS, as well as to analyze epidemiologic and prognostic characteristics in all CHS subtypes. This study found CHS was most common in white patients, had a median age at diagnosis of 53 years, and was most frequently found in lower limb long bones and associated joints. The 5-year survival was much worse at stage IV compared to stages I, II, and III, as well as in dedifferentiated and mesenchymal tumor subtypes compared to conventional or other types of CHS. In high grade tumors, perioperative chemotherapy did not improve survival compared to surgery alone.

Background: Chondrosarcoma (CHS) is the second most common primary malignant solid bone tumor. Present management of CHS is based largely on case series from individual treatment centers. This study summarizes descriptive epidemiologic and prognostic characteristics associated with CHS patients utilizing the National Cancer Database (NCDB).

Methods: We identified 6,559 patients with CHS of the bone, joints, and/or articular cartilage in the NCDB from 2004-2015. Kaplan-Meier analyses were used to estimate 2- and 5-year survival, and median survival. Survival comparisons were made using log-rank tests.

Results: Median age at diagnosis was 53 years. 53.3% of cases were in males, and 86.4% were in white patients. 28.2% of cases were in lower limb long bones and associated joints. The most common NCDB analytic stage at presentation was stage I (60.5%). 6.4% of patients had known metastases at diagnosis. 86.6%, 7%, and 12.5% of patients received surgery, chemotherapy, and radiation, respectively. Overall probability of 5-year survival was 74.4%. Approximate 5-year survival for NCDB analytic stages I-IV was 86.2%, 53.1%, 52.4%, and 17.4%, respectively. The dedifferentiated subtype of CHS had the poorest 5-year survival (25.4%), while juxtacortical had the highest (93.4%). In 355 patients with high grade CHS, perioperative chemotherapy (33.7% 5-year survival) did not provide improved survival compared to surgery alone (51.2% 5-year survival).

Conclusions: To the best of our knowledge, this is the first NCDB study to evaluate survival by treatment type and stage in CHS, as well as to analyze epidemiologic and prognostic characteristics in all CHS subtypes. This study found CHS was most common in white patients, had a median age at diagnosis of 53 years, and was most frequently found in lower limb long bones and associated joints. The 5-year survival was much worse at stage IV compared to stages I, II, and III, as well as in dedifferentiated and mesenchymal tumor subtypes compared to conventional or other types of CHS. In high grade tumors, perioperative chemotherapy did not improve survival compared to surgery alone.

Background: Primary cardiac sarcomas are rare and highly lethal tumors. Current understanding of clinicopathologic characteristics associated with primary cardiac sarcoma is limited to case reports and small case series. To the best of our knowledge, this is the largest study to evaluate clinicopathologic characteristics of primary cardiac sarcoma, and survival benefit of treatment modalities.

Methods: The National Cancer Database was utilized to retrospectively identify 404 patients diagnosed with a single primary cardiac sarcoma between 2004 and 2015. Kaplan-Meier methods were used to estimate survival distribution. Log-rank tests compared survival distributions between groups.

Results: Median age at diagnosis was 50 years. Men (50.7%) and women (49.3%) were affected equally. The most common histology types were hemangiosarcoma (40.1%), sarcoma NOS (14.9%), spindle cell sarcoma (6.9%) and leiomyosarcoma (5.9%). The most common stage at presentation was stage IV (38.1%). 37% of patients presented with metastases at the time of diagnosis, and the most common site of distant metastasis was the
lung (12.6%). Median overall survival (OS) for the entire cohort was 12 months. Median OS for stages I-IV was 23.06, 22.34, 12.98 and 8.08 months, respectively. 63% of patients underwent surgical resection. 58.4% received chemotherapy, and 22% received radiotherapy. Patients who underwent surgery had improved OS compared to those not receiving surgery (15.74 vs. 7.62 months, respectively). In patients with metastatic disease, OS was improved when treated with chemotherapy (11.14 months), compared to surgery alone (5.22 months). Combined treatment of surgery with chemotherapy did not improve outcomes compared to chemotherapy alone (P = .091 on log-rank test). In non-metastatic disease,
patients receiving multi-modality treatment (radiotherapy, chemotherapy and surgery) had improved OS (27.73 months) compared to those who underwent surgery alone, chemotherapy alone or chemotherapy with surgery (6.67, 14.88 and 20.99 months, respectively).

Conclusions: The median age of diagnosis was 50 years, and men and women are affected equally. Stage IV is the most common stage at diagnosis, and prognosis of Stages III and IV are worse in comparison to Stages I and II. In metastatic disease, receipt of chemotherapy is associated with improved survivorship. Multimodality treatment regimens may be recommended in non-metastatic cases.

Background: Primary cardiac sarcomas are rare and highly lethal tumors. Current understanding of clinicopathologic characteristics associated with primary cardiac sarcoma is limited to case reports and small case series. To the best of our knowledge, this is the largest study to evaluate clinicopathologic characteristics of primary cardiac sarcoma, and survival benefit of treatment modalities.

Methods: The National Cancer Database was utilized to retrospectively identify 404 patients diagnosed with a single primary cardiac sarcoma between 2004 and 2015. Kaplan-Meier methods were used to estimate survival distribution. Log-rank tests compared survival distributions between groups.

Results: Median age at diagnosis was 50 years. Men (50.7%) and women (49.3%) were affected equally. The most common histology types were hemangiosarcoma (40.1%), sarcoma NOS (14.9%), spindle cell sarcoma (6.9%) and leiomyosarcoma (5.9%). The most common stage at presentation was stage IV (38.1%). 37% of patients presented with metastases at the time of diagnosis, and the most common site of distant metastasis was the
lung (12.6%). Median overall survival (OS) for the entire cohort was 12 months. Median OS for stages I-IV was 23.06, 22.34, 12.98 and 8.08 months, respectively. 63% of patients underwent surgical resection. 58.4% received chemotherapy, and 22% received radiotherapy. Patients who underwent surgery had improved OS compared to those not receiving surgery (15.74 vs. 7.62 months, respectively). In patients with metastatic disease, OS was improved when treated with chemotherapy (11.14 months), compared to surgery alone (5.22 months). Combined treatment of surgery with chemotherapy did not improve outcomes compared to chemotherapy alone (P = .091 on log-rank test). In non-metastatic disease,
patients receiving multi-modality treatment (radiotherapy, chemotherapy and surgery) had improved OS (27.73 months) compared to those who underwent surgery alone, chemotherapy alone or chemotherapy with surgery (6.67, 14.88 and 20.99 months, respectively).

Conclusions: The median age of diagnosis was 50 years, and men and women are affected equally. Stage IV is the most common stage at diagnosis, and prognosis of Stages III and IV are worse in comparison to Stages I and II. In metastatic disease, receipt of chemotherapy is associated with improved survivorship. Multimodality treatment regimens may be recommended in non-metastatic cases.

Background: Primary cardiac sarcomas are rare and highly lethal tumors. Current understanding of clinicopathologic characteristics associated with primary cardiac sarcoma is limited to case reports and small case series. To the best of our knowledge, this is the largest study to evaluate clinicopathologic characteristics of primary cardiac sarcoma, and survival benefit of treatment modalities.

Methods: The National Cancer Database was utilized to retrospectively identify 404 patients diagnosed with a single primary cardiac sarcoma between 2004 and 2015. Kaplan-Meier methods were used to estimate survival distribution. Log-rank tests compared survival distributions between groups.

Results: Median age at diagnosis was 50 years. Men (50.7%) and women (49.3%) were affected equally. The most common histology types were hemangiosarcoma (40.1%), sarcoma NOS (14.9%), spindle cell sarcoma (6.9%) and leiomyosarcoma (5.9%). The most common stage at presentation was stage IV (38.1%). 37% of patients presented with metastases at the time of diagnosis, and the most common site of distant metastasis was the
lung (12.6%). Median overall survival (OS) for the entire cohort was 12 months. Median OS for stages I-IV was 23.06, 22.34, 12.98 and 8.08 months, respectively. 63% of patients underwent surgical resection. 58.4% received chemotherapy, and 22% received radiotherapy. Patients who underwent surgery had improved OS compared to those not receiving surgery (15.74 vs. 7.62 months, respectively). In patients with metastatic disease, OS was improved when treated with chemotherapy (11.14 months), compared to surgery alone (5.22 months). Combined treatment of surgery with chemotherapy did not improve outcomes compared to chemotherapy alone (P = .091 on log-rank test). In non-metastatic disease,
patients receiving multi-modality treatment (radiotherapy, chemotherapy and surgery) had improved OS (27.73 months) compared to those who underwent surgery alone, chemotherapy alone or chemotherapy with surgery (6.67, 14.88 and 20.99 months, respectively).

Conclusions: The median age of diagnosis was 50 years, and men and women are affected equally. Stage IV is the most common stage at diagnosis, and prognosis of Stages III and IV are worse in comparison to Stages I and II. In metastatic disease, receipt of chemotherapy is associated with improved survivorship. Multimodality treatment regimens may be recommended in non-metastatic cases.

Background: Dedifferentiated liposarcoma (DDL) is a high-grade tumor that either forms as a de novo tumor or as a progression from a low-grade, well-differentiated liposarcoma. DDL is a clinically aggressive tumor associated with low survival probabilities. Prognosis variables for DDL have never been reported in a large patient population.

Methods: 3,573 patients with primary DDL were analyzed from the National Cancer Database (NCDB). The 5- and 10-year survival probabilities  were calculated, and the groups were compared using log-rank comparisons. Median survival was also calculated.

Results: Males comprised 65% of the cohort, 65.4% of the patients were between the age of 51-75 years old, and the median age at diagnosis was 64 years of age. Approximately 65% of tumors were > 10 cm, 89% had no metastases and the most common site of metastasis was the lung (2.5%). The most common tumors were found in the retroperitoneum or peritoneum (40.5%). Overall 5- and 10-year survival probabilities were 51.5% and 34.8%, respectively. Head and neck tumors had the best 5-year outcomes (86.4%) followed by extremities (67.1%), pelvis (65.8%), thorax or trunk (58.9%), retroperitoneum or peritoneum (45.3%), and abdomen (36.8%). Best outcomes were noted in the 26-50 year age group (5-year survival, 66.8%; 10-year survival, 52.9%), < 1 0 cm diameter size (5-year survival: 66.1%; 10-year survival, 46%), stage II (5-year survival, 66.7%), and moderately differentiated tumors (5-year survival, 68.3%). Radiation therapy yielded the best 5- and 10-year survival probabilities of 59% and 39.3%, respectively. Out of all the adjuvant therapies, surgery with radiation resulted in the best 5-year survival of 63.4%.

Conclusions: In the largest and most comprehensive study to date on DDL, major findings include that primary anatomical site is a significant prognostic variable and age at presentation, tumor size, stage, grade, and type of adjuvant therapy significantly affect median survival and overall survival probabilities.

Background: Dedifferentiated liposarcoma (DDL) is a high-grade tumor that either forms as a de novo tumor or as a progression from a low-grade, well-differentiated liposarcoma. DDL is a clinically aggressive tumor associated with low survival probabilities. Prognosis variables for DDL have never been reported in a large patient population.

Methods: 3,573 patients with primary DDL were analyzed from the National Cancer Database (NCDB). The 5- and 10-year survival probabilities  were calculated, and the groups were compared using log-rank comparisons. Median survival was also calculated.

Results: Males comprised 65% of the cohort, 65.4% of the patients were between the age of 51-75 years old, and the median age at diagnosis was 64 years of age. Approximately 65% of tumors were > 10 cm, 89% had no metastases and the most common site of metastasis was the lung (2.5%). The most common tumors were found in the retroperitoneum or peritoneum (40.5%). Overall 5- and 10-year survival probabilities were 51.5% and 34.8%, respectively. Head and neck tumors had the best 5-year outcomes (86.4%) followed by extremities (67.1%), pelvis (65.8%), thorax or trunk (58.9%), retroperitoneum or peritoneum (45.3%), and abdomen (36.8%). Best outcomes were noted in the 26-50 year age group (5-year survival, 66.8%; 10-year survival, 52.9%), < 1 0 cm diameter size (5-year survival: 66.1%; 10-year survival, 46%), stage II (5-year survival, 66.7%), and moderately differentiated tumors (5-year survival, 68.3%). Radiation therapy yielded the best 5- and 10-year survival probabilities of 59% and 39.3%, respectively. Out of all the adjuvant therapies, surgery with radiation resulted in the best 5-year survival of 63.4%.

Conclusions: In the largest and most comprehensive study to date on DDL, major findings include that primary anatomical site is a significant prognostic variable and age at presentation, tumor size, stage, grade, and type of adjuvant therapy significantly affect median survival and overall survival probabilities.

Background: Dedifferentiated liposarcoma (DDL) is a high-grade tumor that either forms as a de novo tumor or as a progression from a low-grade, well-differentiated liposarcoma. DDL is a clinically aggressive tumor associated with low survival probabilities. Prognosis variables for DDL have never been reported in a large patient population.

Methods: 3,573 patients with primary DDL were analyzed from the National Cancer Database (NCDB). The 5- and 10-year survival probabilities  were calculated, and the groups were compared using log-rank comparisons. Median survival was also calculated.

Results: Males comprised 65% of the cohort, 65.4% of the patients were between the age of 51-75 years old, and the median age at diagnosis was 64 years of age. Approximately 65% of tumors were > 10 cm, 89% had no metastases and the most common site of metastasis was the lung (2.5%). The most common tumors were found in the retroperitoneum or peritoneum (40.5%). Overall 5- and 10-year survival probabilities were 51.5% and 34.8%, respectively. Head and neck tumors had the best 5-year outcomes (86.4%) followed by extremities (67.1%), pelvis (65.8%), thorax or trunk (58.9%), retroperitoneum or peritoneum (45.3%), and abdomen (36.8%). Best outcomes were noted in the 26-50 year age group (5-year survival, 66.8%; 10-year survival, 52.9%), < 1 0 cm diameter size (5-year survival: 66.1%; 10-year survival, 46%), stage II (5-year survival, 66.7%), and moderately differentiated tumors (5-year survival, 68.3%). Radiation therapy yielded the best 5- and 10-year survival probabilities of 59% and 39.3%, respectively. Out of all the adjuvant therapies, surgery with radiation resulted in the best 5-year survival of 63.4%.

Conclusions: In the largest and most comprehensive study to date on DDL, major findings include that primary anatomical site is a significant prognostic variable and age at presentation, tumor size, stage, grade, and type of adjuvant therapy significantly affect median survival and overall survival probabilities.