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OBJECTIVE The aim of this study was to identify predictors of desmoid progression during observation. SUMMARY OF BACKGROUND DATA Untreated desmoids can grow, remain stable, or regress, but reliable predictors of behavior have not been identified. METHODS Primary or recurrent desmoid patients were identified retrospectively from an institutional database. In those managed with active observation who underwent serial magnetic resonance imaging (MRIs) with T2-weighted sequences, baseline tumor size was recorded, and 2 radiologists independently estimated the percentage of tumor volume showing hyperintense T2 signal at baseline. Associations of clinical or radiographic characteristics with progression-free survival (PFS; by RECIST) were evaluated by Cox regression and Kaplan-Meier statistics. RESULTS Among 160 patients with desmoids, 72 were managed with observation, and 37 of these had serial MRI available for review. Among these 37 patients, median age was 35 years and median tumor size was 4.7 cm; all tumors were extra-abdominal (41% in abdominal wall). Although PFS was not associated with size, site, or age, it was strongly associated with hyperintense T2 signal in ≥90% versus

OBJECTIVE: We sought to define the prognostic significance of histologic subtype for extremity/truncal liposarcoma (LPS). SUMMARY OF BACKGROUND DATA: LPS, the most common sarcoma, is comprised of five histologic subtypes. Despite their distinct behaviors, LPS outcomes are frequently reported as a single entity. METHODS: We analyzed data on all patients from a single-institution prospective database treated from July 1982 to September 2017 for primary, non-metastatic, extremity or truncal LPS of known subtype. Clinicopathologic variables were tested using competing risk analyses for association with disease-specific death (DSD), distant recurrence (DR), and local recurrence (LR). RESULTS: Among 1001 patients, median follow-up in survivors was 5.4 years. Tumor size and subtype were independently associated with DSD and DR. Size, subtype, and R1 resection were independently associated with LR. DR was most frequent among pleomorphic and round cell LPS; the former recurred early (43% by 3 years), and the latter over a longer period (23%, 3 years; 37%, 10 years). LR was most common in dedifferentiated LPS, in which it occurred early (24%, 3 years; 33%, 5 years), followed by pleomorphic LPS (18%, 3 years; 25%, 10 years). CONCLUSIONS: Histologic subtype is the factor most strongly associated with DSD, DR, and LR in extremity/truncal LPS. Both risk and timing of adverse outcomes vary by subtype. These data may guide selective use of systemic therapy for patients with round cell and pleomorphic LPS, which carry a high risk of DR, and radiotherapy for LPS subtypes at high risk of LR when treated with surgery alone.

OBJECTIVE To determine the prognostic significance of histologic type/subtype in a large series of patients with primary resected retroperitoneal sarcoma. BACKGROUND The histologic diversity and rarity of retroperitoneal sarcoma has hampered the ability to predict patient outcome. METHODS From a single-institution, prospective database, 675 patients treated surgically for primary, nonmetastatic retroperitoneal sarcoma during 1982 to 2010 were identified and histologic type/subtype was reviewed. Clinicopathologic variables were analyzed for association with disease-specific death (DSD), local recurrence (LR), and distant recurrence (DR). RESULTS Median follow-up for survivors was 7.5 years. The predominant histologies were well-differentiated liposarcoma, dedifferentiated liposarcoma, and leiomyosarcoma. Five-year cumulative incidence of DSD was 31%, and factors independently associated with DSD were R2 resection, resection of 3 or more contiguous organs, and histologic type. Five-year cumulative incidence for LR was 39% and for DR was 24%. R1 resection, age, tumor size, and histologic type were independently associated with LR; size, resection of 3 or more organs, and histologic type were independently associated with DR. Liposarcoma and leiomyosarcoma were associated with late recurrence and DSD (as long as 15 years from diagnosis). For solitary fibrous tumor, LR was uncommon (

To develop a nomogram based on clinicopathologic factors to quantify the risk of local recurrence (LR) after limb-sparing surgery without adjuvant radiation (RT).Review of our prospective sarcoma database identified 684 patients with primary, nonmetastatic, extremity STS treated with limb-sparing surgery alone between June 1982 and December 2006. No patient received adjuvant radiation or chemotherapy. Age, sex, grade, depth, size, site, margin status and histology were analyzed for prognostic significance with respect to local recurrence rates using Gray's test. Variables which were significant in univariate analysis at the 0.05 level were entered into a multivariate competing risk regression model. On the basis of the multivariate analysis, a nomogram for predicting the 3- and 5-year risk of LR was developed using R libraries cmprsk and QHScrnomo. Concordance index (C-index) was calculated to evaluate the discriminatory power of the prognostic model.With a median follow-up of 58 months for censored patients (73 months for all patients), the overall 3- and 5-year actuarial local recurrence rates were 11% and 13%, respectively. Factors included in the nomogram were age (≤ 50 vs.50), size (≤ 5 vs.5 cm), margin status (negative vs. positive), grade (low vs. high), and histology (atypical lipomatous tumor/well differentiated liposarcoma vs. other). The STS nomogram predicted the local recurrence rate with a C-index of 0.73.A nomogram for extremity STS that includes age, size, margin status, grade of tumor, and histology predicts the 3- and 5-year risk of local recurrence after limb-sparing surgery in the absence of adjuvant RT.

To compare outcomes of patients with retroperitoneal or pelvic sarcoma treated with perioperative radiation therapy (RT) versus those treated without perioperative RT.RT for retroperitoneal or pelvic sarcoma is controversial, and few studies have compared outcomes with and without RT.Prospectively maintained databases were reviewed to retrospectively compare patients with primary retroperitoneal or pelvic sarcoma treated during 2003-2011. Multivariate Cox regression models were used to assess associations with the primary endpoints: local recurrence-free survival (LRFS) and disease-specific survival.At 1 institution, 172 patients were treated with surgery alone, whereas at another institution 32 patients were treated with surgery and perioperative proton beam RT or intensity-modulated RT with or without intraoperative RT. The groups were similar in age, tumor size, grade, and margin status (all P0.08). The RT group had a higher percentage of pelvic tumors (P = 0.03) and a different distribution of histologies (P = 0.04). Perioperative morbidity was higher in the RT group (44% vs 16% of patients; P = 0.004). After a median follow-up of 39 months, 5-year LRFS was 91% (95% confidence interval, 79%-100%) in the RT group and 65% (57%-74%) in the surgery-only group (P = 0.02). On multivariate analysis, RT was associated with better LRFS (hazard ratio, 0.26; P = 0.03). Five-year disease-specific survival was 93% (95% confidence interval, 82%-100%) in the RT group and 85% (78%-92%) in the surgery-only group (P = 0.3).The addition of advanced-modality RT to surgery for primary retroperitoneal or pelvic sarcoma was associated with improved LRFS, although this did not translate into significantly better disease-specific survival. This treatment strategy warrants further investigation in a randomized trial.

Background: Many patients with benign serous cystadenoma (SCA) of the pancreas will undergo resection because of the inability to reliably discriminate between SCA and premalignant mucinous cysts (intraductal papillary mucinous neoplasm [IPMN], mucinous cystic neoplasm [MCN]). Methods: Cyst fluid from patients with SCA (n = 15), non main-duct and noninvasive IPMN (n = 32), and noninvasive MCN (n = 12) was aspirated at the time of operative resection and analyzed. Commercially available and custom designed multiplex assays (Luminex) were performed using a biomarker panel developed for pancreatic cancer. Differential protein expression (fluorescence intensity, FI) was compared between the 3 groups for each protein (Wilcoxon rank sum test). Unsupervised sample clustering (hierarchical clustering) and supervised sample classification (prediction analysis for microarrays [PAM]) was then performed. Results: Differential protein expression (P < 0.05) was identified between SCA and IPMN (34/51 proteins, 67%) and between SCA and MCN (13/51 proteins, 25%). The majority of proteins were down-regulated in IPMN and MCN compared with SCA. The only proteins significantly overexpressed in the cyst fluid of patients with mucinous cysts were CEA (median FI: IPMN 11.4, MCN 13.0, SCA 5.3; P < 0.001, IPMN vs. SCA) and CA72.4 (median FI: IPMN 10.4, MCN 10.5, SCA 9.9; P = 0.003, IPMN vs. SCA). Unsupervised cluster analysis demonstrated distinct clustering of SCA and IPMN with some cross-over between MCN. Supervised sample classification with 14 proteins had an overall accuracy rate of 92% between SCA and IPMN. Conclusions: In this study differential cyst fluid protein expression was observed between SCA and IPMN for the majority of proteins assessed and multimarker sample classification accurately discriminated between SCA and IPMN in 92% of patients.