Nomogram for predicting cancer-specific survival in undifferentiated pleomorphic sarcoma: A Surveillance, Epidemiology, and End Results -based study.

INTRODUCTION: The purpose of this study was to construct and validate a nomogram for predicting cancer-specific survival (CSS) in undifferentiated pleomorphic sarcoma (UPS) patients at 3, 5, and 8 years after the diagnosis. METHODS: Data for UPS patients were extracted from the SEER (Surveillance, Epidemiology, and End Results) database. The patients were randomly divided into a training cohort (70%) and a validation cohort (30%). The backward stepwise Cox regression model was used to select independent prognostic factors. All of the factors were integrated into the nomogram to predict the CSS rates in UPS patients at 3, 5, and 8 years after the diagnosis. The nomogram' s performance was then validated using multiple indicators, including the area under the time-dependent receiver operating characteristic curve (AUC), consistency index (C-index), calibration curve, decision-curve analysis (DCA), integrated discrimination improvement (IDI), and net reclassification improvement (NRI). RESULTS: This study included 2,009 UPS patients. Ten prognostic factors were identified after analysis of the Cox regression model in the training cohort, which were year of diagnosis, age, race, primary site, histological grade, T, N, M stage, surgery status, and insurance status. The nomogram was then constructed and validated internally and externally. The relatively high C-indexes and AUC values indicated that the nomogram has good discrimination ability. The calibration curves revealed that the nomogram was well calibrated. NRI and IDI values were both improved, indicating that our nomogram was superior to the AJCC (American Joint Committee on Cancer) system. DCA curves demonstrated that the nomogram was clinically useful. CONCLUSIONS: The first nomogram for predicting the prognosis of UPS patients has been constructed and validated. Its usability and performance showed that the nomogram can be applied to clinical practice. However, further external validation is still needed.

Vulvar sarcoma outcomes by histologic subtype: a Surveillance, Epidemiology, and End Results (SEER) database review.

OBJECTIVE: Vulvar cancers account for 5% of all gynecologic malignancies; only 1%-3% of those vulvar cancers are primary vulvar sarcomas. Given the rarity of vulvar sarcomas, outcome data specific to histopathologic subtypes are sparse. The aim of this study was to identify clinical and pathologic factors of primary vulvar sarcomas that are associated with survival and may inform treatment decisions. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was searched for women diagnosed with vulvar sarcoma between 1973 and 2018. We identified 315 patients and reviewed their demographic, clinicopathologic, surgical, and survival information. Statistical analyses included χ2 and t-tests, Kaplan-Meier survival, and Cox regression analyses. RESULTS: The most common histopathologies of vulvar sarcomas were dermatofibrosarcomas (85/315, 27%) and leiomyosarcomas (72/315, 22.9%). Rhabdomyosarcomas (18/315, 5.7%), liposarcomas (16/315, 5.1%), and malignant fibrous histiocytomas (16/315, 5.1%) were less frequent. The majority of patients underwent surgery (292/315, 92.7%), which included lymph node dissections in 21.6% (63/292). Survival and lymph node involvement varied significantly with histologic subtype. The 5-year disease-specific survival for dermatofibrosarcomas, liposarcomas, and fibrosarcomas was 100% and only 60.3% and 62.5% for malignant fibrous histiocytomas and rhabdomyosarcomas, respectively. None of the patients with (dermato)fibrosarcomas, liposarcomas, or leiomyosarcomas had positive lymph nodes, in contrast to rhabdomyosarcomas and malignant fibrous histiocytomas with 77.8% and 40% positive lymph nodes, respectively. The 5-year disease-specific survival for women with positive lymph nodes was 0%. CONCLUSIONS: Vulvar sarcomas are heterogeneous with survival highly dependent on the histopathologic subtype. While surgical excision is the mainstay of treatment for all vulvar sarcomas, staging lymphadenectomy should be deferred for (dermato)fibrosarcomas, liposarcomas, and leiomyosarcomas as there were no cases of lymph nodes metastases.

Clinicopathological characteristics and survival of malignant fibrous histiocytoma of the bone: A population-based study using the SEER database.

Malignant fibrous histiocytoma of the bone (MFH-B) is an extremely rare and aggressive malignancy. The clinicopathological characteristics and prognosis of patients with MFH-B have not been defined. We conducted a retrospective study using the data of all MFH-B patients from the Surveillance, Epidemiology and End Results (SEER) database between 1975 and 2016. Initially, the clinicopathological characteristics were described. The difference in prognosis between patients with MFH-B and those with osteosarcoma was compared using propensity score matching analysis. Then, the features affecting the prognosis of patients with MFH-B were further determined using Cox regression analysis. A total of 318 patients with MFH-B were identified. The median overall survival (mOS) of all 318 patients with MFH-B was 29.0 months. The 1-, 3-, 5-, and 10- year survival rates were 67.4%, 53.6%, 38.7%, and 28.7%, respectively. The multivariate Cox regression analysis showed that older age, distant metastases, and flat bone lesion were independent factors for worse prognosis, whereas surgery was an independent factor for favorable survival, and this intervention could decrease risk of death by 61% (HR = 0.39, 95% CI 0.28-0.54). Apart from this, the prognosis of patients with MFH-B was significantly worse than that of patients with osteosarcoma in both unmatched and matched cohorts. In conclusion, MFH-B is a rare malignant bone cancer, with relatively worse prognosis than osteosarcoma. Older age, distant metastases, flat bone lesion, and surgery were independently associated with prognosis. In order to understand this disease more thoroughly and accurately, more cases with adequate information are required in the future.

Malignant fibrous histiocytoma of bone: A survival analysis from the National Cancer Database.

BACKGROUND AND OBJECTIVES: Malignant fibrous histiocytoma (MFH) of bone, now known as undifferentiated pleomorphic sarcoma of bone, is a rare neoplasm that accounts for less than 2% of all primary malignant bone tumors. The objective of the current study was to evaluate prognosis and survival for MFH of bone. METHODS: The 2004 to 2016 National Cancer Database was queried to identify patients with a primary MFH of bone. Kaplan-Meier survival and Cox regression analyses were used to analyze overall survival and risk factors associated with overall mortality. RESULTS: The overall 5-year and 10-year survival rates were 38.3% and 30.5%, respectively. Increasing stage and metastatic disease at presentation were associated with poor overall survival (P < .001). Patients aged 18 to 50 years (hazard ratio [HR], 0.51), 51 to 75 years (HR, 0.61), and those undergoing surgery (HR, 0.39) had improved survival. Having Medicare insurance (HR, 1.48), residing in a low educated area (HR, 2.56), and positive surgical margins (HR, 1.80) were associated with poor survival. CONCLUSIONS: The overall prognosis of MFH of bone is poor with a reported 5-year survival rate of 38.3%. Undergoing surgery and younger age were associated with a better prognosis. Older age, having Medicare insurance, and positive surgical margins were predictors of mortality.

Prognostic relevance of SMC family gene expression in human sarcoma.

OBJECTIVE: To explore the prognostic value of the expression of genes encoding structural maintenance of chromosomes (SMCs) in human sarcoma. RESULTS: We found that the levels of SMC1A, SMC2, SMC3, SMC4, SMC5 and SMC6 mRNA were all higher in most tumors compared to normal tissues, and especially in sarcoma. According to the Cancer Cell Line Encyclopedia (CCLE), SMC1A, SMC2, SMC3, SMC4, SMC5 and SMC6 are also highly expressed in sarcoma cell lines. Results of Gene Expression Profiling Interactive Analysis (GEPIA) indicated that high expression of SMC1A was significantly related to poor overall survival (OS) (p<0.05) and disease-free survival (DFS) in sarcoma (p<0.05). Additionally, strong expression of SMC2 was significantly related to poor OS in sarcoma (p<0.05). In contrast, SMC3, SMC4, SMC5, and SMC6 expression had no significant impact on OS or DFS in sarcoma. CONCLUSIONS: Expression of SMC family members is significantly different in sarcoma relative to normal tissues, and SMC1A and SMC2 may be useful as prognostic biomarkers. METHODS: We performed a detailed comparison of cancer and normal tissues regarding the expression levels of mRNA for SMC family members in various cancers including sarcoma through ONCOMINE and GEPIA (Gene Expression Profile Interactive Analysis) databases.

The Outcome of Patients With Localized Undifferentiated Pleomorphic Sarcoma of the Lower Extremity Treated at Stanford University.

BACKGROUND: As a diagnosis of exclusion, Undifferentiated Pleomorphic Sarcoma (UPS) has unclear clinical characteristics. The objective of this retrospective cohort study is to investigate which clinical and prognostic factors of primary lower-extremity UPS will determine failure. METHODS: We retrospectively reviewed 55 primary lower-extremity UPS cases treated at Stanford between 1998 and 2015. Overall Survival (OS) and Disease-Free Survival (DFS) curves were calculated. Univariate Fisher's Exact Tests were used to examine relationships between disease recurrence, treatment, patient factors, tumor characteristics, and surgical margins. RESULTS: 5-year DFS and OS rates were 60% (95% CI, 45%-72%) and 68% (95% CI, 53%-79%), respectively. The 5-year DFS rate for patients with positive margins was 33.3% (95% CI, 5%-68%) compared with 63% (95% CI, 47%-76%) for patients with negative margins. (Log-rank, P=0.03). The OS rate for those with disease recurrence was 42% % (95% CI, 16%-67%) compared with 76% (95% CI, 59%-87%) for patients who did not have disease recurrence (log-rank, P=0.021). Local failure occurred more frequently with omission of radiation therapy (Fisher's exact test, P=0.009). CONCLUSIONS: Positive surgical margins are an important prognostic factor for predicting relapse in UPS. Relapse of any kind led to worse OS. Radiation therapy improved local control of disease but had no statistically significant effect on DFS, highlighting the need for improved diagnostics to identify those at highest risk for hematogenous metastasis and for selection of patients for adjuvant systemic treatment.

Prognostic significance of microscopic tumor extension in local recurrence of myxofibrosarcoma and undifferentiated pleomorphic sarcoma.

Myxofibrosarcoma (MFS) and undifferentiated pleomorphic sarcoma (UPS) frequently display infiltrative growth into the adjacent normal soft tissue. In this study, we aimed to determine whether the microscopic extension into surrounding normal tissue can influence the local recurrence of MFS and UPS. A total of 42 cases (22 MFS and 20 UPS) were examined. The length of the microscopic extension was measured from the edge of the main tumor mass to the end of infiltration. The length of 5.5 mm was selected as the optimal cut-off value that could predict local recurrence using the receiver operating characteristic (ROC) curve and Youden index. Longer length of microscopic extension was significantly correlated with the status of resection margin (P = 0.032). The group with longer length of microscopic extension (>5.5 mm) had significantly worse recurrence-free survival than the group with shorter length of microscopic extension (≤5.5 mm) (P = 0.000). Multivariate analysis revealed that the length of microscopic extension was independent predictors of recurrence-free survival (P = 0.018). In conclusion, microscopic extensions at the edge of the main mass into the surrounding normal tissue were observed in most MFS and UPS patients, and the length of microscopic extension was associated with local recurrence.

Atypical fibroxanthoma: Systematic review and meta-analysis of treatment with Mohs micrographic surgery or excision.

BACKGROUND: Atypical fibroxanthoma (AFX) is a fibrohistiocytic tumor with relatively high local recurrence rates but low metastatic potential. Wide local excision (WLE) and Mohs micrographic surgery (MMS) are common treatments, although no consensus exists regarding optimal therapy. OBJECTIVE: To systematically review evidence of AFX recurrence and metastatic rates after different surgical modalities. METHODS: A comprehensive search was performed for articles published from 1946 or database inception to March 20, 2017. Studies selected included those that had 5 or more patients with atypical fibroxanthoma treated surgically. Two reviewers independently abstracted the data. Risk of bias was assessed with the Newcastle-Ottawa scale. Main outcomes and measures included recurrence and metastasis. RESULTS: In total, 23 studies were selected (907 patients and 914 tumors); 175 patients were treated with MMS (recurrence rate 2.0%, 95% confidence interval [CI] 0%-4.1%; metastatic rate 1.9%, 95% CI 0.1%-3.8%), and 732 were treated with WLE (recurrence rate 8.7%, 95% CI 5%-12.3%; metastasis rate 1%, 95% CI 0.2%-1.9%). Among immunocompromised patients, no recurrence or metastases developed in the MMS subgroup, although 4 of 10 recurred and 1 of 10 metastasized in the WLE subgroup. LIMITATIONS: Low quality of the studies published. CONCLUSION: MMS for atypical fibroxanthoma is associated with a lower recurrence rate than WLE.

Undifferentiated pleomorphic sarcoma: Factors predictive of adverse outcomes.

BACKGROUND: Undifferentiated pleomorphic sarcoma (UPS) encompasses rare neoplasms that can arise either in the dermis or in the subfascial soft tissue. The behavior of UPS ranges from indolent to aggressive, but data predicting outcomes are limited. OBJECTIVE: Identify predictors of poor outcomes by analyzing a large collection of UPS cases. METHODS: We evaluated all available cases of UPS (including those termed atypical fibroxanthoma, malignant fibrous histiocytoma, pleomorphic dermal sarcoma, and subfascial UPS) across 3 tertiary care centers. RESULTS: Among the 319 patients, 45 experienced recurrence, 33 experienced metastasis, and 96 died of any cause. Risk factors for recurrence were clinical tumor size larger than 5 cm and invasion beyond subcutaneous fat. Risk factors for distant metastases were tumor site, tumor size larger than 2 cm, invasion beyond subcutaneous fat, and lymphovascular invasion. Risk factors for overall mortality were age, immunosuppression, tumor size larger than 2 cm, and lymphovascular invasion. History of skin cancer was associated with a lower risk of recurrence and metastasis. LIMITATIONS: This was a retrospective study. CONCLUSIONS: Using the unbiased approach of pooling all UPS cases regardless of terminology, we identified clinical and histologic factors predicting poor outcomes. We propose subcategorization of UPS (into superficial versus deep UPS), which is consistent with the American Joint Committee on Cancer staging of soft-tissue sarcoma.

Identifying actionable variants using next generation sequencing in patients with a historical diagnosis of undifferentiated pleomorphic sarcoma.

There are limited data regarding the molecular characterization of undifferentiated pleomorphic sarcomas (UPS; formerly malignant fibrous histiocytoma). This study aimed to investigate the utility of next generation sequencing (NGS) in UPS to identify subsets of patients who harbour actionable mutations. Patients diagnosed with UPS underwent pathological re-evaluation by a pathologist specializing in sarcoma. Tumor DNA was isolated from archived fresh frozen tissue samples and genotyped using NGS with the Illumina MiSeq TruSeq Amplicon Cancer Panel (48 genes, 212 amplicons). In total, 95 patients initially classified with UPS were identified. Following pathology re-review the histological subtypes were reclassified to include: Myxofibrosarcoma (MFS, N = 44); UPS(N = 18); and Others (N = 27; including undifferentiated spindle cell sarcoma (N = 15) and dedifferentiated liposarcoma (N = 6)). Seven cases were excluded from further analysis for other reasons. Baseline demographics of the finalized cohort (N = 88) showed a median age of 66 years (32-95), primarily with stage I-III disease (92%) and high-grade (86%) lesions. Somatic mutations were identified in 31 cases (35%)(Total mutations = 36: solitary mutation(n = 27); two mutations( =n = 3); three mutations(n = 1)). The most commonly identified mutations were in TP53 (n = 24), ATM (n = 3) and PIK3CA (n = 2). Three of 43 patients with MFS and one of 18 patients with UPS had clinically relevant mutations, mainly related to biomarkers of prediction of response; however few had targetable driver mutations. Somatic mutation status did not influence disease free or overall survival. Based on the small number of clinically relevant mutations, these data do not support the routine use of targeted NGS panels outside of research protocols in UPS.

LN2, CD10, and Ezrin Do Not Distinguish Between Atypical Fibroxanthoma and Undifferentiated Pleomorphic Sarcoma or Predict Clinical Outcome.

BACKGROUND: Atypical fibroxanthoma (AFX) is a rare cutaneous spindled cell neoplasm. For both diagnostic and therapeutic purposes, it is important to distinguish AFX from other poorly differentiated tumors, including undifferentiated pleomorphic sarcoma (UPS). OBJECTIVE: The authors aimed to identify the clinical, histologic, and immunohistochemical expression of LN2, ezrin, and CD10 in AFX and UPS tumors. METHODS AND MATERIALS: The authors retrospectively examined the charts of patients with AFX and UPS treated with Mohs micrographic surgery (MMS) at 2 academic institutions. Patient demographics, tumor characteristics, and clinical course data were collected. Immunohistochemical stains were performed on primary and recurrent AFX and UPS tumors with monoclonal antibodies against the B-cell marker LN2 (CD74), CD10, and ezrin. RESULTS: In the series of 169 patients with AFX included in this study, local recurrence was rare at 3%. In contrast, the seven patients with UPS had an aggressive clinical course with 1 local recurrence and 2 distant metastases. Immunohistochemistry staining for ezrin, LN2, and CD10 were similar in AFX and UPS tumors. CONCLUSION: AFX can be treated with MMS with rare instances of recurrence. Undifferentiated pleomorphic sarcoma has a more aggressive clinical course with increased risk for recurrence and metastasis. Staining with ezrin, LN2, and CD10 did not differentiate AFX or UPS tumors.

[Efficacy of reconstruction with modular endoprosthesis after resection of periacetabular malignant tumors].

OBJECTIVE: To explore the efficacy of the resection of periacetabular malignant tumors and the reconstruction with modular endoprosthesis.
 METHODS: From August 2006 to December 2012, 22 patients with periacetabular malignant tumors, who received the resection and reconstruction with modular prosthesis, were retrospectively reviewed. There were 11 males and 11 females, and the average age was 44 (16-65) years old. Pathological results showed there were 13 cases of chondrosarcoma, 5 cases of osteosarcoma, 2 cases of Ewing's sarcoma, 1 case of maligant fibrous histiocytoma, and 1 case of giant cell tumor. According to the classification system by Enneking, there were 11 cases of Type II+III resection, 5 cases of Type I+II+III resection, 3 cases of Type I+II resection, and 3 cases of Type II resection.
 RESULTS: All patients were followed up. The average time for follow-up was 49 (11-103) months. At the last time of follow-up, 13 patients (59%) were still alive, 9 patients (41%) died due to their primary disease. Metastasis occurred in 8 patients (36%), and local recurrence occurred in 5 patients (23%). The mean Musculoskeletal Tumor Society (MSTS) score for 13 cases of alive patients at the latest time of follow-up was (18.5±5.7) months. The mean score for 11 patients, whose limb salvage were successful, was 20.7±2.0.
 CONCLUSION: Reconstruction with modular prosthesis after wide resection for periacetabular malignant tumor can achieve satisfied outcome.

Primary malignant fibrous histiocytoma of long bones: long-term follow-up.

OBJECTIVES: This study aims to evaluate patients diagnosed with malignant fibrous histiocytoma and investigate the possible prognostic factors associated with duration of survival. PATIENTS AND METHODS: The study, which was conducted between May 1994 and September 2013, included 14 patients diagnosed as malignant fibrous histiocytoma (12 males, 2 females; median age 48 years; range 17 to 64 years). We evaluated patients' demographic features, location of the pathology, histological findings, surgical margins, and treatment modalities and investigated the effects of these parameters on survival. RESULTS: Femur was the most frequently involved bone, followed by tibia and humerus. The median follow-up duration of the patients was 129 months. We performed limb salvage surgeries in 13 patients and amputation in one patient. Surgical margins were marginal in three patients and postoperative radiotherapy was performed for local control of the disease. Although there was no local recurrence in these patients, distant metastasis developed in two patients, indicating the importance of surgical margin as a significant factor on survival. Five-year survival rate was 81.9% in patients with wide surgical margins and 33.3% in patients with marginal margins. CONCLUSION: Surgical excision with wide margins and adjuvant chemotherapy provided adequate control of the disease and longer survival. The only prognostic factor statistically significantly associated with duration of survival was surgical margins. Neoadjuvant chemotherapy may be used when there is a suspicion of not obtaining adequately wide surgical margin perioperatively due to close association with neurovascular structures.

Balancing Prolonged Survival with QoL Using Low-dose Pazopanib Maintenance: A Comparison with the PALETTE Study.

BACKGROUND: A consensus has not been reached regarding the optimal pazopanib dosing schedule, which we determined in patients who received pazopanib at our Institution. PATIENTS AND METHODS: Twenty-five patients who were prescribed pazopanib between 2012 and 2015 were included in this retrospective analysis. RESULTS: The median progression-free survival (PFS) time was 7.7 months. This time (various doses) was similar to that achieved by high-dose pazopanib in the PALETTE study. The log-rank test revealed no significant differences in the PFS times between the low- and high-dose pazopanib groups, with the majority of patients receiving a dose of 400 mg, indicating that controlling the side-effects might be more critical than administering higher doses. CONCLUSION: Pazopanib should be started from a low dose with careful increase to avoid pazopanib-related side-effects, which is necessary to provide a balance between the life-prolonging effects of pazopanib and quality of life (QoL) of patients.

Analysis of Clinical and Molecular Factors Impacting Oncologic Outcomes in Undifferentiated Pleomorphic Sarcoma.

BACKGROUND: Undifferentiated pleomorphic sarcomas (UPS) present a diagnostic and therapeutic challenge. Identification of prognostic molecular markers is required for the discovery of novel treatment approaches. The purpose of this study was to correlate clinicopathologic variables, expression of tyrosine kinase receptors, and markers of cell cycle progression and survival with oncologic outcomes. METHODS: A tissue microarray containing 208 primary UPS samples was analyzed by immunohistochemistry for protein markers and in situ hybridization for microRNA. Staining results were correlated with clinicopathologic features and oncologic outcomes. Univariate and multivariate analyses were conducted to assess associations between expression of protein markers, mi-RNA, and outcome. RESULTS: At a median follow-up of 3.9 years (9 years for survivors), 5-year disease-specific survival (DSS) was 63 %. Clinical variables associated with improved DSS included age <61 years, tumor size <10 cm, margin-negative resection, and sporadic-tumor status. At the protein level, loss of cyclin D1 (p = 0.06), pEGFR (p = 0.023), pIGF-1R (p = 0.022), and PTEN (p < 0.001) and overexpression of AXL (p = 0.015) were associated with reduced DSS on univariate analysis. Ki67, PCNA, and pEGFR were more highly expressed in sporadic UPS than radiation-associated (RA-UPS), whereas RA-UPS samples expressed higher levels of both phosphorylated and total IGF-1R. DISCUSSION: Loss of cyclin D1, overexpression of AXL, and loss of PTEN are associated with poor cancer-specific outcomes and warrant further investigation in UPS. The differences in protein expression in sporadic versus RA-UPS may indicate that the activated molecular signaling nodes may be different for each specific histology and also could explain the aggressive phenotype seen in RA-UPS compared with the sporadic lesions.

Effect of Non-Hodgkin Lymphoma on Survival in Patients With Malignant Fibrous Histiocytoma, Kaposi Sarcoma, and Sebaceous Carcinoma: A SEER Population-Based Study.

OBJECTIVE: To quantify the behavior of dermatofibrosarcoma protuberans (DFSP), malignant fibrous histiocytoma (MFH), Kaposi sarcoma (KS), and sebaceous carcinoma (SC) in patients with a history of non-Hodgkin lymphoma (NHL). PATIENTS AND METHODS: Subjects with a diagnosis of DFSP, MFH, KS, or SC between 1990 and 2006 were identified in the Surveillance, Epidemiology, and End Results Program database. For each skin cancer type, the standardized mortality ratio (SMR) for death due to any cause and death due to skin cancer was estimated. RESULTS: From 1990 through 2006, 25,357 skin cancers were identified: 4,192 DFSP, 6,412 MFH, 10,543 KS, and 4,222 SC. For patients with a history of non-CLL NHL, SMRs for death due to any cause were 1.45 (95% confidence interval [CI], 1.03-2.04; p = 0.04) for MFH, 2.90 (95% CI, 2.50-3.36; p < 0.001) for KS, and 3.25 (95% CI, 1.84-5.75; p < 0.001) for SC and SMRs for death due to skin cancer were 0.55 (95% CI, 0.23-1.31; p = 0.18) for MFH, 2.93 (95% CI, 2.49-3.43; p < 0.001) for KS, and 4.07 (95% CI, 1.28-12.94; p < 0.001) for SC. CONCLUSION: Among patients with KS and SC, patients with a history of non-CLL NHL have a greater risk of overall and cause-specific death than expected.

The value of the high-sensitivity modified Glasgow prognostic score in predicting the survival of patients with a soft-tissue sarcoma.

The aim of this study was to determine whether the high-sensitivity modified Glasgow prognostic score (Hs-mGPS) could predict the disease-specific survival and oncological outcome in adult patients with non-metastatic soft-tissue sarcoma before treatment. A total of 139 patients treated between 2001 and 2012 were retrospectively reviewed. The Hs-mGPS varied between 0 and 2. Patients with a score of 2 had a poorer disease-specific survival than patients with a score of 0 (p < 0.001). The estimated five-year rate of disease-specific survival for those with a score of 2 was 0%, compared with 85.4% (95% CI 77.3 to 93.5) for those with a score of 0. Those with a score of 2 also had a poorer disease-specific survival than those with a score of 1 (75.3%, 95% CI 55.8 to 94.8; p < 0.001). Patients with a score of 2 also had a poorer event-free rate than those with a score of 0 (p < 0.001). Those with a score of 2 also had a poorer event-free survival than did those with a score of 1 (p = 0.03). A multivariate analysis showed that the Hs-mGPS remained an independent predictor of survival and recurrence. The Hs-mGPS could be a useful prognostic marker in patients with a soft-tissue sarcoma.

Radiation-Associated Undifferentiated Pleomorphic Sarcoma is Associated with Worse Clinical Outcomes than Sporadic Lesions.

BACKGROUND: Radiation therapy is used increasingly as a component of multidisciplinary treatment for many solid tumors. One complication of such treatment is the development of radiation-associated sarcoma (RAS). Undifferentiated pleomorphic sarcoma (UPS), previously termed "malignant fibrous histiocytoma" (MFH) is the most common histologic subtype of RAS. This study investigated the clinical outcomes for patients with radiation-associated UPS (RA-UPS/MFH). METHODS: The study identified 1068 patients with UPS/MFH treated at the authors' institution. Patient and tumor factors were collected and compared. Regression analysis was performed to identify independent predictors of survival. A matched-cohort survival and recurrence analysis was performed for radiation-associated and sporadic UPS/MFH. RESULTS: The findings showed that RA-UPS/MFH comprised 5.1 % of the UPS population. The median latency to the development of RA-UPS/MFH was 9.3 years. The 5-year disease-specific survival (DSS) was 52.2 % for patients identified with RA-UPS/MFH (n = 55) compared with 76.4 % for patients with unmatched sporadic UPS/MFH (n = 1,013; p < 0.001). A matched-cohort analysis also demonstrated that the 5-year DSS was significantly worse for RA-UPS/MFH (52.2 vs 73.4 %; p = 0.002). Furthermore, higher local recurrence rates were observed for patients with RA-UPS/MFH than for patients with sporadic lesions (54.5 vs 23.5 %; p < 0.001). Radiation-associated status and incomplete resection were identified as independent predictors of local recurrence. CONCLUSION: This study demonstrated worse clinical outcomes for patients with RA-UPS/MFH than for patients with sporadic UPS/MFH. Local recurrence was significantly higher for patients with RA-UPS/MFH, suggesting a unique tumor biology for this challenging disease.

MFH and high-grade undifferentiated pleomorphic sarcoma-what's in a name?

BACKGROUND AND OBJECTIVES: In 2002, with the advent of better classification techniques, the World Health Organization declassified malignant fibrous histiocytoma (MFH) as a distinct histological entity in favor of the reclassified entity high-grade undifferentiated pleomorphic sarcoma (HGUPS). To date, no study has evaluated comparative outcomes between patients designated historically in the MFH group and those classified in the new HGUPS classification. Our goal was to determine the presence of clinical prognostic implications that have evolved with this new nomenclature. METHODS: Sixty-eight patients were retrospectively evaluated between January 1998 and December 2007. Forty-five patients diagnosed with MFH between 1998 and 2003 were compared to 23 patients in the HGUPS group, from 2004 to 2007. Primary prognostic outcomes assessed included overall survival, metastatic-free, and local recurrence-free survival. RESULTS: Five-year survivorship between MFH and HGUPS populations, using Kaplan-Meier or competing risk methods, did not show statistical difference for overall survival (60% vs. 74%, P=0.36), 5-year metastasis-free survival (31% vs. 26%, P=0.67), or local recurrence-free survival (13% vs. 16%, P=0.62). CONCLUSION: Despite new classification nomenclature, there appears to be no identifiable prognostic implications for sarcomas that remain in the unclassifiable HGUPS group, as compared to the previously accepted MFH group.