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Background: Gastric cancer (GC) is clinically heterogenous according to location (cardia/non-cardia) and histopathology (diffuse/intestinal). We aimed to characterize the genetic risk architecture of GC according to its subtypes. Another aim was to examine whether cardia GC and oesophageal adenocarcinoma (OAC) and its precursor lesion Barrett's oesophagus (BO), which are all located at the gastro-oesophageal junction (GOJ), share polygenic risk architecture. Methods: We did a meta-analysis of ten European genome-wide association studies (GWAS) of GC and its subtypes. All patients had a histopathologically confirmed diagnosis of gastric adenocarcinoma. For the identification of risk genes among GWAS loci we did a transcriptome-wide association study (TWAS) and expression quantitative trait locus (eQTL) study from gastric corpus and antrum mucosa. To test whether cardia GC and OAC/BO share genetic aetiology we also used a European GWAS sample with OAC/BO. Findings: Our GWAS consisting of 5816 patients and 10,999 controls highlights the genetic heterogeneity of GC according to its subtypes. We newly identified two and replicated five GC risk loci, all of them with subtype-specific association. The gastric transcriptome data consisting of 361 corpus and 342 antrum mucosa samples revealed that an upregulated expression of MUC1, ANKRD50, PTGER4, and PSCA are plausible GC-pathomechanisms at four GWAS loci. At another risk locus, we found that the blood-group 0 exerts protective effects for non-cardia and diffuse GC, while blood-group A increases risk for both GC subtypes. Furthermore, our GWAS on cardia GC and OAC/BO (10,279 patients, 16,527 controls) showed that both cancer entities share genetic aetiology at the polygenic level and identified two new risk loci on the single-marker level. Interpretation: Our findings show that the pathophysiology of GC is genetically heterogenous according to location and histopathology. Moreover, our findings point to common molecular mechanisms underlying cardia GC and OAC/BO. Funding: German Research Foundation (DFG).

Purpose The aim of this study was to create and validate dynamic nomograms to predict overall survival (OS) and disease-free survival (DFS) at different time points during follow-up in patients who had undergone resection of primary retroperitoneal sarcoma (RPS). Methods Patients with primary RPS operated upon between 2002 and 2017 at four and six referral centres comprised the development and external validation cohorts, respectively. Landmark analysis and multivariable Cox models were used to develop dynamic nomograms. Variables were selected using two backward procedures based on the Akaike information criterion. The prediction window was fixed at 5 years. Nomogram performances were tested in terms of calibration and discrimination on the development and validation cohorts. Results Development and validation cohorts totalled 1357 and 487 patients (OS analysis), and 1309 and 452 patients (DFS analysis), respectively. The final OS model included age, landmark time (TLM), tumour grade, completeness of resection and occurrence of local/distant recurrence. The final DFS model included TLM, histologic subtype, tumour size, tumour grade, multifocality and the interaction terms between TLM and size, grade and multifocality. For OS, Harrell C indices were higher than 0.7 in both cohorts, indicating very good discriminative capability. For DFS, Harrell C indices were between 0.64 and 0.72 in the development cohort and 0.62 and 0.68 in the validation cohort. Calibration plots showed good agreement between predicted and observed outcomes. Conclusion Validated nomograms are available to predict the 5-year OS and DFS probability at different time points throughout the first 5 years of follow-up in RPS survivors.

Pelvic soft tissue sarcomas are rare. Potentially curative resection remains challenging due to anatomical constraints of true pelvis and tumour spread through various anatomical hiatus. We sought to review the oncological outcomes of surgically managed cases at our centre and determine whether outcomes differ for patients with localised (limited to pelvis) versus extensive disease (with extra-pelvic extension).Sixty-seven patients who underwent surgical resection with curative intent at the centre for primary, non-metastatic, WHO intermediate to high-grade soft tissue sarcoma of the true pelvis from January 2012 through January 2020 were analysed. Establishment of the extent of disease was made by review of pre-treatment imaging and surgical notes. Oncologic endpoints examined were resection margin, recurrence rate, disease-free and overall survival.Rates of complete oncological resection and disease control were similar for tumours with localised or extensive disease. On logistic regression analysis, tumour grade, and a negative resection margin (R0) correlated with the risk of recurrence (p=0.05). On further multinomial analysis, R0 resection was associated with improved local control, but not metastatic relapse (p = 0.003). 5-year local recurrence-free and distant metastasis-free survival were 61.3% and 67.1%, respectively. Five and 10-year overall survival were 64% and 36%, respectively. Age50 years and high tumour grade were associated with a worse outcome (p 0.05).When potentially curative surgery is performed for pelvic sarcoma, disease-extent does not influence oncologic outcomes. While a complete oncologic resection determines the risk of local recurrence, tumour grade and metastatic relapse remain primary prognostic determinants for overall survival.

Contains fulltext : 235268.pdf (Publisher’s version ) (Open Access) BACKGROUND: Oesophageal gastrointestinal stromal tumours (GISTs) account for ≤1% of all GISTs. Consequently, evidence to guide clinical decision-making is limited. METHODS: Clinicopathological features and outcomes in patients with primary oesophageal GIST from seven European countries were collected retrospectively. RESULTS: Eighty-three patients were identified, and median follow up was 55.0 months. At diagnosis, 59.0% had localized disease, 25.3% locally advanced and 13.3% synchronous metastasis. A biopsy (Fine Needle aspiration n = 29, histological biopsy n = 31) was performed in 60 (72.3%) patients. The mitotic count was low (5/5hpf) mitotic count were associated with worse disease free survival. CONCLUSION: Based on the current study, enucleation can be recommended for oesophageal GIST smaller than 4 cm, while oesophagectomy should be preserved for larger tumours. Patients with larger tumours (>4 cm) and/or high mitotic count should be treated with adjuvant therapy.

ZusammenfassungDas undifferenzierte pleomorphe Sarkom („undifferentiated pleomorphic sarcoma“ [UPS]) gehört zur Gruppe der Weichteilsarkome und macht fast 10 % aller Weichteilsarkome aus. Der Fall eines 49-jährigen Patienten wird vorgestellt, bei dem die kompartmentorientierte Resektion eines primären UPS im linken Musculus gluteus maximus mit adjuvanter Radiotherapie (60 Gy) durchgeführt wurde. Im Rahmen der Tumornachsorge (3 Jahre später) wurde eine lokoregionäre Metastase an einer ungewöhnlichen Lokalisation im M. quadratus femoris festgestellt, welche mittels einer In-toto-Resektion mit intraoperativer Radiotherapie (10 Gy) behandelt wurde. Der intra- und postoperative Verlauf gestalten sich komplikationslos ohne neurologische Defizite. Im Rahmen der Nachtuntersuchung 6 Monate postoperativ war der Patient tumor- und beschwerdefrei.

Introduction The management of locally advanced extremity soft tissue sarcomas, particularly in terms of a limb salvage strategy, represents a challenge, especially in recurrent tumors. In the context of a patient-tailored multimodal therapy, hyperthermic isolated limb perfusion (ILP) is a promising limb-saving treatment option. We report the outcome of patients with primarily irresectable and locally recurrent soft tissue sarcoma (STS) treated by ILP. Patients and methods Data about patient demographics, clinical und histopathological characteristics, tumor response, morbidity and oncological outcome of all patients with STS, who underwent an ILP at our institution in a 10-year period, were retrospectively detected and analyzed. Results The cohort comprised 30 patients. Two patients were treated with ILP for palliative tumor control, 13 patients because of a local recurrent soft tissue sarcoma (rSTS) and 15 patients because of primarily unresectable soft tissue sarcoma (puSTS). 25 of the 28 patients with curative intention received surgery after ILP (11 pts with rSTS and 14 pts with puSTS). Histopathologically we observed complete response in 6 patients (24%) and partial responses in 19 patients (76%) with a significant better remission in patients with puSTS (p = 0,043). Limb salvage rate was 75%. Mean follow-up was 69 months [range 13–142 months]. Seven (7/11; 64%) patients with rSTS and one (1/14; 7%) patient with puSTS developed local recurrence after ILP and surgery, whereas eight (8/13; 62%) rSTS patients and seven (7/15; 47%) puSTS patients developed distant metastasis. During follow-up, eight patients (28.5%) died of disease (5/13; 38%) rSTS and 3/15 (20%) puSTS. ILP in the group of previously irradiated sarcoma patients (n = 13) resulted in a limb salvage rate of 69% and was not associated in an increased risk for adverse events. Discussion ILP for advanced extremity STS is a treatment option for both puSTS and rSTS resulting in good local control and should be considered in multimodal management. ILP is also a good option for patients after radiation history.

Background: Neuroma of the biliary tree is extremely rare with no more than 100 cases reported so far. They mostly present with obstructive jaundice and have been commonly described after surgery or abdominal trauma. Although involvement of the extrahepatic bile duct is far more common, occurrence in the intrahepatic ducts has not so far been reported. Case Report: We describe 3 cases of diffuse biliary tree neuroma affecting 3 females aged 53–68 years. None had a history of neurofibromatosis type1. All presented with progressive obstructive jaundice with no evidence of gallstones. A history of previous surgery was noted in 2 patients. Initial impression on clinical and imaging examination was highly suspicious for bile duct carcinoma in 2 patients. Histology showed diffuse neuromatous proliferation replacing and thickening the bile duct walls. The third patient had concurrent neuroma and recurrent cholangiocarcinoma causing great clinical confusion as initial biopsy showed only benign neuroma, but CA 19-9 was steadily increasing, necessitating a second biopsy which then confirmed recurrent carcinoma. Conclusion: This uncommon cause of long-distance bile duct stenosis and progressive jaundice should be included in the differential diagnosis of bile duct neoplasms, in particular when there is a previous surgical history in this abdominal region.

Objective: The objective of this study was to investigate the prognosis of patients with metastatic soft tissue sarcomas (STS) and to define prognostic indicators for overall survival (OS). Methods: All patients who were treated at the Sarcoma Unit at the Mannheim University Medical Center between 2010 and 2016 and who developed metastatic disease deriving from a STS were included in this retrospective analysis. OS was investigated using data from clinical records and German registry offices. Clinical and pathological characteristics were recorded and analyzed. Results: A total number of 212 patients developed metastatic disease from STS during that period. Median OS after first documentation of metastatic disease was 24 months (95% CI 21–33). 1-, 2-, and 5-year OS rates were 70.0% (95% CI 64–77), 49.9% (95% CI 43–58), and 24.8% (95% CI 19–33), respectively. In multivariate analysis, significant predictors for mortality appeared to be gender, age, location and size of the primary tumor, histology, and disease-free interval. Conclusion: Being treated in a high-volume STS reference center in Germany, patients with metastatic disease could demonstrate an increased OS compared to former analyses. These data can be used as a benchmark for upcoming studies and highlight that further research on treatment strategies in this rare disease is urgently needed.

Background Caroli Disease (CD) and Caroli Syndrome (CS) are rare disorders presenting with dilation of the intrahepatic bile ducts. CD/CS are associated with cholangiocarcinoma (CCA). However, the true incidence of CCA is still unclear, although it may serve as an indication for surgery. In this paper, we analyzed (I) the incidence of CCA in German centers, (II) reviewed our single center population together with its clinical presentation and (III) performed a thorough literature review. Methods 17 large HPB-centers across Germany were contacted and their patients after surgical treatment due to CD/CS with histopathology were included. Medline search for all studies published in English or German literature was performed. Patients who underwent surgery at our department between 2012 and 2020 due to CD or CS were analyzed. Results In the multicenter study, 79 patients suffered from CD and 119 patients from CS, with a total number of 198 patients. In 14 patients, CCA was found (Overall: 7,1%; CD: 6,3%, CS 7,6%). Between 2012 and 2020, 1661 liver resections were performed at our department. 14 patients underwent surgery due to CD or CS. Histological examination showed synchronous cholangiocarcinoma in one patient. The literature review revealed a CCA-rate of 7,3% in large series, whereas in case reports a rate of 6,8% was found. Conclusion There is risk of malignant transformation and patients with CD might also benefit from resection due to improvement of symptoms. Therefore, resection is strongly advised. As certain patients with CS require transplantation, treatment should not be guided by the relatively low rate of CCA but by the concomitant diseases that come along with hepatic failure.

Advanced osteosarcomas (OSs) and Ewing sarcomas (ESs) tend to have poor prognosis with limited therapeutic options beyond first-line therapy. Aberrant angiogenesis and MET signaling play an important role in preclinical models. The anti-angiogenic drug cabozantinib was tested in a phase 2 trial of advanced OS and ES and was associated with clinical benefits. We retrospectively analyzed the off-label use of cabozantinib in adult patients with advanced OS and ES/primitive neuroectodermal tumors (PNETs) in three centers of the Hellenic Group of Sarcoma and Rare Cancers (HGSRC). Between April 2019 and January 2022, 16 patients started taking 60 mg of cabozantinib for advanced bone sarcoma or PNET. Median age at cabozantinib initiation was 31 years (17–83). All patients had received peri-operative chemotherapy for primary sarcoma and between 0 and 4 lines of treatment (median; 2.5) for advanced disease. The most common adverse effects included fatigue, anorexia, hypertransaminasemia, weight loss, and diarrhea. One toxic death was noted (cerebral hemorrhage). Dose reduction to 40 mg was required in 31.3% of the patients. No objective response was noted, and 9/16 patients exhibited stable disease outcomes. Progression-free survival varied from 1 to 8 (median; 5) months. Our study demonstrates that cabozantinib has antitumor activity in this population. In the real-life setting, we observed similar adverse events as in the CABONE study and in other neoplasms.

Purpose In a post hoc analysis of the MAGIC trial, patients with curatively resected gastric cancer (GC) and mismatch repair (MMR) deficiency (MMRd) had better median overall survival (OS) when treated with surgery alone but worse median OS when treated with additional chemotherapy. Further data are required to corroborate these findings. Methods Between April 2013 and December 2018, 458 patients with curatively resected GC, including cancers of the esophagogastric junction Siewert type II and III, were identified in the German centers of the staR consortium. Tumor sections were assessed for expression of MLH1, MSH2, MSH6 and PMS2 by immunohistochemistry. The association between MMR status and survival was assessed. Similar studies published up to January 2021 were then identified in a MEDLINE search for a meta-analysis. Results MMR-status and survival data were available for 223 patients (median age 66 years, 62.8% male), 23 patients were MMRd (10.3%). After matching for baseline clinical characteristics, median OS was not reached in any subgroup. Compared to perioperative chemotherapy, patients receiving surgery alone with MMRd and MMRp had a HR of 0.67 (95% CI 0.13–3.37, P = 0.63) and 1.44 (95% CI 0.66–3.13, P = 0.36), respectively. The meta-analysis included pooled data from 385 patients. Compared to perioperative chemotherapy, patients receiving surgery alone with MMRd had an improved OS with a HR of 0.36 (95% CI 0.14–0.91, P = 0.03), whereas those with MMRp had a HR of 1.18 (95% CI 0.89–1.58, P = 0.26). Conclusion Our data support a positive prognostic effect for MMRd in GC patients treated with surgery only and a differentially negative prognostic effect in patients treated with perioperative chemotherapy. MMR status determined by preoperative biopsies may be used as a predictive biomarker to select patients for perioperative chemotherapy in curatively resectable GC.

Brain metastases are rare events in patients with sarcoma and the available information is relatively limited. We retrospectively reviewed medical records of patients with sarcoma who developed brain metastases between April 2010 and April 2020 in six centers. Thirty-four adult patients were included with a median age at brain metastases diagnosis of 55.5 years (range, 18–75). The primary sarcomas originated either from soft tissue (n = 27) or bone (n = 7) and the most common subtypes were leiomyosarcoma (n = 8), Ewing sarcoma/peripheral neuroectodermal tumor (PNET) (n = 7) and osteosarcoma (n = 3). Most primary tumors were of high grade and located mainly in the extremities (n = 18). The vast majority of patients at the time of brain metastasis diagnosis already had extracranial metastatic disease (n = 26). The median time from sarcoma diagnosis to cerebral metastasis diagnosis was 16 months (range, 1–136). Treatment modalities for brain metastatic disease included whole-brain radiation therapy (WBRT) (n = 22), chemotherapy (n = 17), exclusive palliative care (n = 5), surgery (n = 9), targeted therapy (n = 6) or stereotactic radiosurgery (n = 2). Most patients experienced a progression of brain metastases (n = 11). The median overall survival from brain metastasis diagnosis was 3 months (range, 0–80). OS was significantly influenced by time-to-brain metastases (p = 0.041), WBRT (p = 0.018), surgery (p = 0.002) and chemotherapy (p = 0.006). In a multivariate analysis, only the localization of the primary (p = 0.047) and WBRT (p = 0.038) were associated with survival with statistical significance. Patients with sarcoma brain metastases have a particularly poor prognosis and an appropriate therapeutic approach is yet to be defined.

Background and Aims: Symptoms of patients with gastric cancer (GC) are often unspecific and differences in symptoms between patients with cardia and non-cardia GC have been poorly investigated. We aimed to characterize symptoms of patients with cardia and non-cardia GC. Methods: Patients with cardia (Siewert type II and III) and non-cardia GC were recruited in the German multicenter cohort of the Gastric Cancer Research (staR) study between 2013 and 2017. Alarm, dyspeptic and reflux symptoms at the time of presentation were documented using a self-administered questionnaire. Results: A completed self-administered questionnaire was available for 568/759 recruited patients (132 cardia GC, 436 non-cardia GC, male 61%, mean age 64 years). Dyspeptic symptoms were more common in patients with non-cardia GC (69.0 vs. 54.5%, p=0.0024). Cardia GC patients reported more frequently alarm symptoms (69.7 vs. 44.7%, p

Background: Neoadjuvant treatment modalities in soft tissue sarcoma (STS) of the extremities have become more popular in recent years, but because of the rarity and heterogeneity of STS, there are yet few studies on the long-term impact of neoadjuvant treatment modalities, especially in terms of neoadjuvant radiochemotherapy. Methods: The study enrolled 136 patients with primary STS of the extremities who underwent surgery with curative intent or neoadjuvant therapy, followed by surgery in a 15-year period. Neoadjuvant treatment consisted of radiotherapy (RT) with 60 Gy and in most cases simultaneous chemotherapy (CTx) with ifosfamide (1.5 g/m2/d, d1–5, q28) and doxorubicine (50 mg/m2/d, d3, q28). We investigated the clinical, (post)-operative and histopathological data and the oncological follow-up as well. The median follow-up period was 82 months (range 6–202). Results: A total of 136 patients (M:F = 73:63) with a mean age of 62 years (range, 21–93) was observed. Seventy-four patients (54.4%) received neoadjuvant therapy (NT), 62 patients (45.6%) received primary surgery (PS). When receiving NT, patients with high-risk STS had a lower risk to develop distant metastasis (p = 0.025). Age, histological type, tumor size and surgical margins (R0 vs. R1) had no influence on any survival rates. There was an association between NT and the occurrence of postoperative complications (p = 0.001). The 5-year local recurrence free survival (LRFS), metastasis free survival (MFS), disease free survival (DFS) and overall survival (OS) rate of the whole cohort was 89.9%, 77.0%, 70.6% and 72.6%, whereas the 5-year LRFS, MFS, DFS and OS rate was 90.5%, 67.2%, 64.1% and 62.8% for the NT group and 89.5%, 88.3%. 78.4% and 83.8% for the PS group. Conclusion: Multimodal treatment strategies in patients with STS of extremities lead to excellent oncological outcomes. Patients with high-risk STS had a significantly better MFS when receiving NT than patients with low-risk STS. NT was associated with a higher probability of postoperative but well-manageable complications.

Simple Summary In patients with unclear soft tissue lesions, treatment planning largely depends on histology. Core needle biopsy is the diagnostic standard in these cases. The aim of this retrospective study was to investigate accuracy and safety of ultrasound guided core needle biopsy at a tertiary referral center. We show that ultrasound guided biopsy was feasible as a one stop shop procedure in an outpatient setting in 87.6% of the cases. The rate of conclusive biopsies was 88.5%. After surgical resection, the dignity, tumor type and histopathological grading of the biopsy matched one of the resection specimens in 97.2%, 92.7% and 92.5% of the cases, respectively. Major complications occurred in 0.8% of the cases. With this study, we confirm that ultrasound guided core needle biopsy is safe, effective and can be performed at the first outpatient presentation to speed up the diagnostic and therapeutic cascade in sarcoma patients. Abstract Background: The aim of this study was to investigate diagnostic accuracy, safety and histologic results of ultrasound guided core needle biopsy (CNB) in patients with soft tissue lesions (STL) at a tertiary referral center. Methods: A retrospective analysis of all consecutive patients undergoing ultrasound guided CNB for STL at our sarcoma outpatient service between January 2015 and August 2020 was performed. Results: A total of 392 patients were identified. Main histologic entities were sarcomas, lipomas and desmoid tumors. Biopsy was performed in an outpatient setting in 87.6% of the cases. Conclusive biopsies were obtained in 88.5% of the cases. In patients who underwent surgical resection after CNB, the concordance of dignity, tumor entity and histopathological grading between biopsy and resection specimen were 97.2%, 92.7% and 92.5% respectively. The risk of inconclusive CNB was highest in intraabdominal or retroperitoneal tumors (19.5%) and lowest in lesions at the lower extremity (4.4%). Major complications after CNB occurred in three cases (0.8%). No case of biopsy tract seeding was observed during the study period. Conclusions: Ultrasound guided CNB for STL at first presentation in a dedicated surgical outpatient setting is a safe procedure and yields a high diagnostic accuracy.

Background: Duodenal gastrointestinal stromal tumors (GIST) are a rare subset of GIST. Their surgical management in this anatomically complex region consists of varied approaches, and the administration of imatinib mesylate (IM) has not been clarified. Methods: We retrospectively reviewed patients with duodenal GIST treated during a 10-year-period. We analysed the clinicopathological characteristics and survival factors and evaluated the perioperative and long-term outcomes based on the extent of resection ((ocal-resection (LR) versus pancreaticoduodenectomy (PD)) and the IM-administration. The median follow-up period was 60 months (range, 12–140). Results: A total of thirteen patients (M:F = 7:6) with median age of 64 years (range, 42–77) underwent resection of duodenal GIST. Median tumor size was 5.2 cm (range, 1.5–13.3). Eight patients (61.5%) underwent LR and five patients (38.5%) PD. R0-resection was achieved in 92.5%. Neoadjuvant IM-therapy was administered in five patients leading to tumor downsizing and in 40% to less-extended resection. The PD group consisted of larger tumors with higher mitotic count, mostly located in D2 (p = 0.031). The PD group had longer operative time (p = 0.026), longer hospital stay (p = 0.016), and higher rate of postoperative complications (p = 0.128). The actuarial 1-, 3-, and 5-year overall survival were 92.5%, 84%, and 73.5%, respectively, whereas the disease-free survival rates at 1, 3, and 5 years were 91.5%, 83%, and 72%, respectively. A tendency towards increased risk of disease recurrence was demonstrated for patients with tumor &gt, 5 cm and high-risk potential. There was not statistic survival benefit for one or the other surgical approach. Conclusion: The type of resection depends on duodenal site of origin and tumor size. LR can be the treatment of choice for duodenal GIST whenever technically feasible. Recurrence of duodenal GIST is dependent on tumor biology rather than surgical approach. Administration of IM in neaodjuvant setting should be considered in cases with high-risk GIST scheduled for PD since it might facilitate less-extended resection.

Objectives Patients with autoimmune gastritis (AIG) are reported to have an increased risk of developing gastric cancer (GC). In this study, we assess the characteristics and outcomes of GC patients with AIG in a multicenter case-control study. Methods Between April 2013 and May 2017, patients with GC, including cancers of the esophagogastric junction (EGJ) Siewert type II and III, were recruited. Patients with histological characteristics of AIG were identified and matched in a 1:2 fashion for age and gender to GC patients with no AIG. Presenting symptoms were documented using a self-administered questionnaire. Results Histological assessment of gastric mucosa was available for 572/759 GC patients. Overall, 28 (4.9%) of GC patients had AIG (67 ± 9 years, female-to-male ratio 1.3:1). In patients with AIG, GC was more likely to be localized in the proximal (i.e. EGJ, fundus, corpus) stomach (odds ratio (OR) 2.7, 95% confidence interval (CI) 1.0–7.1). In GC patients with AIG, pernicious anemia was the leading clinical sign (OR 22.0, 95% CI 2.6–187.2), and the most common indication for esophagogastroduodenoscopy (OR 29.0, 95% CI 7.2–116.4). GC patients with AIG were more likely to present without distant metastases (OR 6.2, 95% CI 1.3–28.8) and to be treated with curative intention (OR 3.0, 95% CI 1.0–9.0). The five-year survival rates with 95% CI in GC patients with and with no AIG were 84.7% (83.8–85.6) and 53.5% (50.9–56.1), respectively (OR 0.25, 95% CI 0.08–0.75, p = 0.001). Conclusions Pernicious anemia leads to earlier diagnosis of GC in AIG patients and contributes significantly to a better clinical outcome.

Background: Angiogenesis is a hallmark of breast cancer (BC) and is mediated by the vascular endothelial growth factor (VEGF) signaling axis. It is regulated by different proangiogenic factors, including platelet-derived growth factor-CC (PDGF-CC) and heparin-binding EGF-like growth factor (HB-EGF), as well as co-receptors, such as neuropilin-1, which could have prognostic implications in BC patients. Patients and methods: We assessed the serum levels of VEGF, HB-EGF, PDGF-CC and neuropilin-1 in 205 patients with early BC (invasive, n = 187; in situ, n = 18) and in 31 healthy donors (HD) and investigated the potential associations with clinical and histopathological parameters. Results: VEGF serum levels were significantly higher in patients with invasive versus ductal carcinomas in situ. PDGF-CC serum concentrations varied among BC molecular subtypes. Furthermore, we observed a differential expression of most biomarkers between overweight/obese (body mass index (BMI) ≥ 25 kg/m2) and non-obese patients among the BC molecular subtypes. Finally, the classification of subjects according to menopausal status revealed a significant difference in specific biomarker levels between patients and HD. Conclusion: The serum concentrations of angiogenic molecules differ among breast cancer molecular subtypes and are affected by the BMI and menopausal status, which could have possible clinical or prognostic implications.

Purpose Incisional hernia (IH) is not uncommon after liver transplantation (LT). We investigated the long-term outcome of mesh-based hernia repair using an inlay-onlay technique. Methods Our analysis was based on a prospective collected database of all LT recipients from our hospital over a period of 15 years. We analyzed clinical data including the period between LT and hernia development, the size and localization of the hernia, the length of in-hospital stay, immunosuppression, and postoperative morbidity, as well as follow-up data. The median follow-up period was 120 (range 12–200) months. Results Among a total of 220 patients who underwent a collective 239 LTs, 29 (13%) were found to have an IH after a median period of 27.5 months (range 3–96 months). There were 12 (41%) men and 17 (59%) women, with a median age of 51 years. The median size of the IH was 13 cm (range 2–30 cm) and the median in-hospital stay was 6 days. Mild postoperative complications developed in seven patients, including two onlay mesh infections. One patient (3.4%) suffered recurrence. Conclusion Mesh-based hernia repair using the inlay/onlay technique represents an effective and safe method for patients with an IH after LT, without additional risk from continuous immunosuppression.

The undifferentiated pleomorphic sarcoma (UPS) is a part of the soft tissue sarcoma group and represents almost 10% of all soft tissue sarcomas. The case of a 49-year-old patient is presented who was diagnosed with a primary UPS in the left gluteus maximus muscle, which was treated with compartmental resection with adjuvant radiotherapy (60 Gy). During tumor follow-up (3 years later) a locoregional metastasis at an unusual location in the quadratus femoris muscle was detected, which was treated by in toto resection with intraoperative radiotherapy (10 Gy). The intra and postoperative outcome was without complications and without neurological deficits. In the last follow-up, 6 months postoperatively, the patient was free of tumors and symptoms.Das undifferenzierte pleomorphe Sarkom („undifferentiated pleomorphic sarcoma“ [UPS]) gehört zur Gruppe der Weichteilsarkome und macht fast 10 % aller Weichteilsarkome aus. Der Fall eines 49-jährigen Patienten wird vorgestellt, bei dem die kompartmentorientierte Resektion eines primären UPS im linken Musculus gluteus maximus mit adjuvanter Radiotherapie (60 Gy) durchgeführt wurde. Im Rahmen der Tumornachsorge (3 Jahre später) wurde eine lokoregionäre Metastase an einer ungewöhnlichen Lokalisation im M. quadratus femoris festgestellt, welche mittels einer In-toto-Resektion mit intraoperativer Radiotherapie (10 Gy) behandelt wurde. Der intra- und postoperative Verlauf gestalten sich komplikationslos ohne neurologische Defizite. Im Rahmen der Nachtuntersuchung 6 Monate postoperativ war der Patient tumor- und beschwerdefrei.

Background: Neoadjuvant imatinib mesylate (IM) for advanced, non-metastatic gastrointestinal stromal tumors (GIST) of stomach is recommended to downsize the tumor prompting less-extensive operations and preservation of organ function. Methods: We analyzed the clinical-histopathological profile and oncological outcome of 55 patients (median age 58.2 years, range, 30–86 years) with biopsy-proven, cM0, gastric GIST who underwent IM therapy followed by surgery with a median follow-up of 82 months. Results: Initial median tumor size was 113 mm (range, 65–330 mm) and 10 patients started with acute upper GI bleeding. After a median 10 months (range, 2–21 months) of treatment, tumor size had shrunk to 62 mm (range, 22–200 mm). According to Response Evaluation Criteria In Solid Tumors version 1.0 and version 1.1 (RECIST 1.1), 39 (75%) patients had partial response and 14 patients had stable disease, with no progressive disease. At plateau response, 50 patients underwent surgery with an R0 resection rate of 94% and pathological complete response in 24%. In 12 cases (24%), downstaging allowed laparoscopic resection. The mean recurrence-free survival (RFS) was 123 months (95%CI, 99–147) and the estimated 5-year RFS was 84%. Conclusions: Neoadjuvant IM allowed stomach preservation in 96% of our patients with excellent long-term RFS, even when starting treatment during an episode of upper GI bleeding. Preservation of the stomach provides the physiological basis for the use of oral IM in the adjuvant or metastatic setting.

Background: Soft tissue sarcoma (STS) treatment is an interdisciplinary challenge. Along with radio(chemo)therapy, surgery plays the central role in STS treatment. Little is known about the impact of reconstructive surgery on STS, particularly whether reconstructive surgery enhances STS resection success with the usage of flaps. Here, we analyzed the 10-year experience at a university hospital&rsquo, s Comprehensive Cancer Center, focusing on the role of reconstructive surgery. Methods: We performed a retrospective analysis of STS-patients over 10 years. We investigated patient demographics, diagnosis, surgical management, tissue/function reconstruction, complication rates, resection status, local recurrence and survival. Results: Analysis of 290 patients showed an association between clear surgical margin (R0) resections and higher-grade sarcoma in patients with free flaps. Major complications were lower with primary wound closure than with flaps. Comparison of reconstruction techniques showed no significant differences in complication rates. Wound healing was impaired in STS recurrence. The local recurrence risk was over two times higher with primary wound closure than with flaps. Conclusion: Defect reconstructions in STS are reliable and safe. Plastic surgeons should have a permanent place in interdisciplinary surgical STS treatment, with the full armamentarium of reconstruction methods.

Background. This study aimed to investigate changes in treatment strategy and outcome for patients with primary retroperitoneal sarcoma (RPS) undergoing resection at referral centers during a recent period.Methods. The study enrolled consecutive adult patients with primary non-metastatic RPS who underwent resection with curative intent between 2002 and 2017 at 10 referral centers. The patients were grouped into three periods according to date of surgery: t1 (2002-2006), t2 (2007-2011), and t3 (2012-2017). Five-year overall survival (OS), disease-specific survival (DSS), and crude cumulative incidence (CCI) of local recurrence (LR) and distant metastasis (DM) were calculated. Multivariable analyses for OS and DSS were performed.Results. The study included 1942 patients. The median follow-up period after resection varied from 130 months (interquartile range [IQR], 124-141 months) in t1 to 37 months (IQR, 35-39 months) in t3. The 5-year OS was 61.2% (95% confidence interval [CI], 56.4-66.3%) in t1, 67.0% (95 CI, 63.2-71.0%) in t2, and 71.9% (95% CI, 67.7-76.1%) in t3. The rate of macroscopically incomplete resection (R2) was 7.1% in t1 versus 4.7% in t3 (p = 0.066). The median number of resected organs increased over time (p < 0.001). In the multivariable analysis resection during t3 was associated with better OS and DSS. The 90-day postoperative mortality improved over time (4.3% in t1 to 2.3% in t3; p = 0.031). The 5-year CCI of LR and DM did not change significantly over time.Conclusions. The long-term survival of patients who underwent resection for primary RPS has increased during the past 15 years. This increased survival is attributable to better patient selection for resection, quality of surgery, and perioperative patient management.

This study aimed to investigate changes in treatment strategy and outcome for patients with primary retroperitoneal sarcoma (RPS) undergoing resection at referral centers during a recent period.The study enrolled consecutive adult patients with primary non-metastatic RPS who underwent resection with curative intent between 2002 and 2017 at 10 referral centers. The patients were grouped into three periods according to date of surgery: t1 (2002-2006), t2 (2007-2011), and t3 (2012-2017). Five-year overall survival (OS), disease-specific survival (DSS), and crude cumulative incidence (CCI) of local recurrence (LR) and distant metastasis (DM) were calculated. Multivariable analyses for OS and DSS were performed.The study included 1942 patients. The median follow-up period after resection varied from 130 months (interquartile range [IQR], 124-141 months) in t1 to 37 months (IQR, 35-39 months) in t3. The 5-year OS was 61.2% (95% confidence interval [CI], 56.4-66.3%) in t1, 67.0% (95 CI, 63.2-71.0%) in t2, and 71.9% (95% CI, 67.7-76.1%) in t3. The rate of macroscopically incomplete resection (R2) was 7.1% in t1 versus 4.7% in t3 (p = 0.066). The median number of resected organs increased over time (p 0.001). In the multivariable analysis resection during t3 was associated with better OS and DSS. The 90-day postoperative mortality improved over time (4.3% in t1 to 2.3% in t3; p = 0.031). The 5-year CCI of LR and DM did not change significantly over time.The long-term survival of patients who underwent resection for primary RPS has increased during the past 15 years. This increased survival is attributable to better patient selection for resection, quality of surgery, and perioperative patient management.

Background Local recurrence (LR) of retroperitoneal soft tissue sarcoma (RPS) is a common and life-threatening event. The evaluation of the exact anatomical patterns of local recurrence might help to improve local treatment in RPS. Methods Of our local database we extracted ten patients with LR of RPS with axial MRI and/or CT datasets of the primary tumor (PT) and the LR. Using the Osirix DICOM viewer Version v.3.9.4 64-bit (Pixmeo, Geneva, Switzerland) we performed a three-step fusion algorithm consisting of: a) 3-point co-registration of the axial datasets depicting the PT and the LR using three abdominal landmarks b) re-orientation of the datasets and c) image fusion. We evaluated the feasibility of this technique with regard to categorizing the localization of LR as within or distant from the PT. Results Fusion imaging was feasible in seven out of ten patients. In the other three patients anatomical shifting of organs after surgery led to a relevant mismatch of anatomical landmarks and impeded interpretation of the fused images. In five of seven patients with successful fusion imaging, local recurrences were located within the anatomical borders of the primary tumor, in two out of seven patients local recurrences were distant to the primary. Conclusions Fusion imaging of primary tumors and local recurrences is feasible in most patients with RPS. Most local recurrences occurred within the anatomical localization of the primary tumor. For further investigations validation of the technique in larger patient cohorts is required.

Introduction Complete mesocolic excision (CME) is generally accepted as state of the art in colon cancer surgery. However, the long-term impact of CME has not been systematically examined. Therefore cohort studies might be a possible way to clarify any differences between conventional resections and CME. Following bilateral cooperation between the Department of Surgery/University Hospital of Erlangen and the 1st Surgical Department of the General Hospital of Nikaia/Piraeus, including teaching activities for introduction of CME, a cohort study was performed, considering surgical quality criteria and clinical outcome. Material and methods All patients with colon carcinomas (CME group, n = 31) referred to the 1st Surgical Department of General Hospital, Nikaia/Piraeus, Greece for surgery from January 2012 to December 2013 were prospectively analyzed and compared with patients who underwent conventional surgery for colon cancer between January 2008 and December 2011 (non-CME group, n = 35). Patients' follow-up was at least 48 months. Results There were significantly better results in terms of lymph node yield (CME group: 29.6 vs. non-CME group: 17.85; p < 0.001) and lymph node ratio (LNR) (CME group: 0.12 vs. non-CME group: 0.24; p < 0.001) and recurrence-free survival in favor of the CME group (CME group: n = 0 vs. non-CME group: n = 5) without any increase in surgical morbidity (CME group: n = 6 vs. non-CME group: n = 11; p = 0.10). Conclusions Complete mesocolic excision appears to offer a superior oncological result without any increase of postoperative morbidity and mortality. Furthermore, CME represents a surgical technique which can be established in a surgical department after previous teaching without increasing the postoperative complication rate.

Genetic associations between variants on chromosome 5p13 and 8q24 and gastric cancer (GC) have been previously reported in the Asian population. We aimed to replicate these findings and to characterize the associations at the genome and transcriptome level. We performed a fine-mapping association study in 1926 GC patients and 2012 controls of European descent using high dense SNP marker sets on both chromosomal regions. Next, we performed expression quantitative trait locus (eQTL) analyses using gastric transcriptome data from 143 individuals focusing on the GC associated variants. On chromosome 5p13 the strongest association was observed at rs6872282 (P = 2.53 x 10(-04)) and on chromosome 8q24 at rs2585176 (P = 1.09 x 10(-09)). On chromosome 5p13 we found cis-eQTL effects with an up-regulation of PTGER4 expression in GC risk allele carrier (P = 9.27 x 10(-11)). On chromosome 8q24 we observed cis-eQTL effects with an upregulation of PSCA expression in GC risk allele carrier (P = 2.17 x 10(-47)). In addition, we found trans-eQTL effects for the same variants on 8q24 with a downregulation of MBOAT7 expression in GC risk allele carrier (P = 3.11 x 10(-09)). In summary, we confirmed and refined the previously reported GC associations at both chromosomal regions. Our data point to shared etiological factors between Asians and Europeans. Furthermore, our data imply an upregulated expression of PTGER4 and PSCA as well as a downregulated expression of MBOAT7 in gastric tissue as risk-conferring GC pathomechanisms.

Health and quality of life outcomes 17, 170 (2019). doi:10.1186/s12955-019-1236-4, Published by BioMed Central, London

Introduction Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade malignant mesenchymal tumor of the soft tissue, characterized by slow infiltrative growth and common local recurrence, with rare distant metastases. Patients and methods We present a retrospective study of nineteen patients who were diagnosed with DFSP and operated at our institution in > 10-year period. We examined the clinicopathological parameters with special emphasis on the margin status regarding the clinical outcome and the follow-up. Results A total of eight cases underwent re-excision at our institution following primary excision or incisional biopsy performed at a different institution. Seven cases received excision after incisional biopsy at our institution. Four patients developed recurrent disease following primary excision with histological R0 margins at other institutions and received re-excision at our institution. All excisions at our institution resulted in R0 margins with no recurrence recorded at last follow-up (6 to 175; mean 84 months). The mean margin for those who received resection at our institution was 1.67 cm. Negative margins upon primary excision were achieved using a mean margin width of 2.04 cm. Most common tumor localization was the trunk (10 cases). Conclusion Awareness of this rare entity is important for a prompt diagnosis and a proper management of the disease. The greatest clinical challenge in the management of DFSP is achieving local control. Complete excision of the tumor with surgical margin widths of at least 2 cm is recommended.

INTRODUCTION The aim of the study was to assess the diagnostic accuracy of procalcitonin (PCT) as a marker for complications and as a prognostic factor for mortality after liver transplantation. MATERIAL AND METHODS Liver transplant patients between January 2007 and April 2011 were prospectively included in the study. Procalcitonin serum concentration was recorded before, 6 h after reperfusion and then daily. Postoperative clinical course was prospectively analyzed from admission to discharge. Main surgical data such as operating procedure, type of reperfusion, operating and ischemic times, high urgency (HU) status and MELD score at the time of transplantation were also recorded. RESULTS Sixteen patients with initial PCT > 5 ng/ml suffered ≥ 1 complication (p = 0.03). However, there was no association between the level of the 1(st) peak PCT and the further postoperative course or the occurrence of complications. Patients in whom a 2(nd) PCT peak occurred had a significantly higher risk for a complicated course, for a complicated sepsis course and for mortality (p < 0.0001). Warm ischemic time over 58 min, operating time over 389 min and HU status were significant independent factors for a complicated postoperative course (p < 0.001, p < 0.001 and p = 0.03 respectively). CONCLUSIONS Based on our results, we believe that PCT course and the occurrence of a 2(nd) peak seem to possess important diagnostic and prognostic power in the post-transplant setting after liver transplantation.

Castleman disease (CD) is a rare lymphoproliferative disorder of unknown etiology and one of the uncommon causes of nonneoplastic lymphadenopathy. Earlier synonyms of CD included angiofollicular lymph node hyperplasia, giant lymph node hyperplasia, lymphoid hamartoma, benign lymphoma and follicular lymphoreticuloma. This disease was first reported by Castleman et al. in 1954 [1] and later defined by Castleman et al. in 1956 in a series of 13 patients with unicentric hyaline vascular CD presenting with large thymoma-like masses in the anterior mediastinum [2]. The condition was later found to be occasionally associated with hypochromic anemia, hypergammaglobulinemia and bone marrow plasmacytosis [3, 4]. By the late 1960s and the early 1970s, the histological features of the different forms of the disease and the classification in current use had been established [5, 6]. The disease features three main histopathological presentations: hyaline vascular, plasma cell-rich, and mixed variants [6, 7]. The exact incidence of CD is unknown. Association of certain forms of the disease with the human immunodeficiency virus (HIV) and human herpes virus-8 (HHV-8) infections has been long recognized. In 1985, Lachant et al. reported on 2 patients with the acquired immunodeficiency syndrome (AIDS) who developed multicentric CD followed by Kaposi sarcoma [8]. In 1995, Soulier et al. reported frequent coincidence of HHV-8 in 31 patients with multicentric CD [9].

Andrian van der Spigel was the first to describe the semilunar line, in 1645 [1]. The Spigelian aponeurosis is formed by the fusion of the aponeurosis of the internal oblique muscle and the aponeurosis of the transversus abdominis muscle. The linea semilunaris forms the lateral margin and the lateral edge of the rectus muscle the medial margin of the Spigelian aponeurosis. It extends from the costal cartilage of the 8th rib to the symphysis pubis [1–3]. In the upper abdominal wall, the Spigelian aponeurosis is posterior to the rectus muscle, making it difficult even for the experienced surgeon to locate a fascial defect during the physical examination. Superior to the umbilical region, the aponeurosis of the internal oblique crosses over the transverses, creating a strong abdominal wall barrier. Beneath the umbilical region the aponeurosis forms a weak barrier that is susceptible to protruding peritoneal sacs or extraperitoneal fat [1, 3–5]. Spigelian hernia was first reported by Klinklosch in 1764. More than half of all Spigelian hernias are located in a 6 cm wide region beneath the umbilicus and superior to the inferior epigastric vessels. This region is referred as “the Spigelian hernia belt” [1, 2]. The most common symptom of Spigelian hernia is pain (60%) due to contraction of the abdominal musculature. Another common symptom is a palpable abdominal mass in 35% of cases [6–17]. Predisposing factors were collagen disorders, age, obesity, rapid weight loss, multiple pregnancies, chronic pulmonary disease, trauma, iatrogenic causes and congenital disorders [3, 10, 18]. Once Spigelian hernia is diagnosed, there is a need for surgical treatment because of the high risk for serious complications. Emergency surgery is estimated to be performed in 21–33% of cases, due to incarceration and strangulation [2, 9–11, 15–22]. Here, we present a unique case of Spigelian hernia with incarceration and obstruction of the terminal ileum, appendix, cecum and right hemicolon that was treated with a major surgical procedure on an emergency basis. A 70-year-old white female patient (body mass index: 34.2 kg/m2) referred to the Surgical Department of the Western Attica Hospital with abdominal pain in the right lower quadrant (RLQ), mild abdominal distention and progressive constipation. Her past medical history was significant for constipation symptoms, nausea and recurrent fever. Her past surgical history was not remarkable, and there was not a triggering effect for bowel entrapment. The physical examination revealed a 18 × 10 × 12 cm palpable mass in the RLQ. The overlying skin was irritated with signs of an extended inflammatory reaction (Figure 1). Laboratory examinations revealed leukocytosis (16 700 white blood cells/µl) and a major electrolyte disorder (hypokalemia (3.0 mmol/l) and hyponatremia (129 mmol/l)). The plain abdominal X-ray examination revealed gas-fluid levels indicating large bowel obstruction (Figure 2). Due to these findings an immediate surgical intervention under general anesthesia was performed. The surgical exploration was carried out via a right pararectal anterior approach over the mass. Laparotomy confirmed a great mass protruding through the neck of a Spigelian hernia of 15 × 8 × 10 cm size (Figure 3 A). After opening the hernia sac, strangulation of the cecum, terminal ileum and ascending colon with extended necrosis of the intestinal wall was found (Figure 3 B, C). A right hemicolectomy was performed (Figure 3 D), and the defect in the abdominal wall was repaired by Prolene Hernia System (20 × 15 cm) according to our technique [23]. The histopathological examination showed an extended necrosis, without any signs of malignancy. The patient was discharged on the 9th postoperative day after an unremarkable hospital course. The patient underwent a colonoscopy 12 weeks after surgical treatment in order to exclude a malignancy of the colon, and after 5 years of follow-up the patient is doing well without any evidence of hernia recurrence. Figure 1 Huge palpable mass in the right lower quadrant of the abdomen Figure 2 Plain abdominal X-ray examination with gas-fluid levels indicating large bowel obstruction Figure 3 A – Inflammatory mass in subcutaneous tissue. B – Neck of Spigelian hernia. C – Necrosis of intestinal wall. D – Surgical specimen after right hemicolectomy The most common symptoms of Spigelian hernia are pain (60%) and a palpable abdominal mass in 35% of cases [6]. Whether a Spigelian hernia should be diagnosed through physical examination remains a controversial matter: In more than 50% of cases the physical examination alone is not enough to establish the diagnosis [7]. However, according to Larson, patients’ history and physical examination alone establish the diagnosis of Spigelian hernia in 74% of cases [8]. Many authors have reported that many Spigelian hernias may remain unclear until laparotomy is performed. Weiss et al. reported in a series of 178 patients that the preoperative diagnosis was correct in 92 (51.7%) cases [9]. The diameter of the neck is usually about 0.5–2 cm, and due to this fact the risk of obstruction and incarceration is high (21%) [10, 11]. The hernia sac usually contains preperitoneal fat or greater omentum, but there are cases in which the sac contains parts of the small bowel or other solid organs [2]. Colonic obstruction is, nevertheless, a very rare condition. To our knowledge, only 10 cases have been reported in the literature (Table I) [12–17]. Abdominal X-ray examination may show signs of ileus in case of incarceration but is not specific enough to reveal the cause of the obstruction. Ultrasonography and computed tomography can clearly define the defect of the abdominal wall as well as the contents of the hernia sac [19, 20]. Once Spigelian hernia is diagnosed, there is a need for surgical treatment because of the high risk of further serious complications. Emergency surgery is estimated to be performed in 21–33% of cases, due to incarceration and strangulation [2, 9–11, 15]. The surgical procedures performed for repair of Spigelian hernia are generally classified as follows: open (primary suture repair, mesh-repair preperitoneal or intraperitoneal) and laparoscopic (intra- or preperitoneal). Open procedures are still widely used, because of the preoperative uncertainty of diagnosis and the high rate of emergency surgery, but the laparoscopic approach is gaining increased importance mainly in uncomplicated cases [11, 19, 21, 22]. Table I Reported cases with colonic obstruction secondary to a Spigelian hernia

Peritoneal metastases (PM) are common in advanced-stage colorectal cancer (CRC) patients representing the second most common metastatic site of CRC. In the past, this clinical situation was treated with palliative intent. The introduction of new chemotherapeutic and targeted biologic agents has improved the prognosis of patients with metastatic colorectal cancer. However, the inclusion of treatment options such as cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) as inherent components of a multidisciplinary treatment was evolving for the management of these patients. Notably, the surgical approach of CRS/HIPEC has been effective offering a prolonged survival with curative intent in patients with colorectal PM. This article reviews and highlights the recent evidence of CRS and HIPEC as well as current research going on in this form of locoregional treatment in the setting of peritoneal metastases of colorectal cancer.

BackgroundOver the past decade, several changes occurred in diagnostics, treatment and understanding of pathogenesis of gastrointestinal stromal tumours (GIST). However, their coexistence with other malignancies of different histogenetic origin remains a challenging issue.MethodsPatients diagnosed with GIST in a 10-years period were identified retrospectively and clinical history and findings thoroughly explored for the presence of associated other malignancies. Follow up data were obtained and analysed for prognostic impact of the concurrent malignancy and/or GIST.ResultsThirty seven (27 males, 10 females) of 86 GIST-patients (43%) had another malignancy. Mean age was 70 years. Associated malignancies were gastrointestinal (n = 29; 69%), renal-/urological (n = 5; 12%), haematological (n = 4; 9.5%), cutaneous (n = 3; 7%) and thyroid (n = 1; 2.5%) in origin. Majority of GISTs occurred in stomach (65%) and small intestine (30.6%) and most (78%) were asymptomatic incidental findings during diagnostic or therapeutic procedures for associated malignancies. GIST size ranged from 0.1 cm to 9 cm (mean, 2.2 cm) and all of them had a low (

Introduction There is continuing controversy over the significance of prognostic factors in sporadic desmoid-type fibromatosis (DF). Further, only limited data is available in the literature concerning second recurrence in patients with recurrent DF. The purpose of this study was to identify prognostic factors influencing recurrence-free survival (RFS) and to determine rates of local recurrence (LR) in patients with primary and recurrent DF. Methods Fifty-three consecutive patients with DF who underwent surgery at a single institution were identified. As a result of strict exclusion criteria, a homogeneous cohort of thirty-two patients who received macroscopically complete resection was included in this study and split into a primary (PG) and recurrent disease (RG) group. Clinicopathological parameters and immunohistochemical markers were retrospectively and independently analyzed in both groups. Results The local recurrence rate was 35% for the PG and 33% for the RG. The PG's calculated median time to relapse was 17 months, and the RG's was 29 months. The estimated 1-, 5-, and 8-year RFS rates for the PG were 75%, 64%, and 55% while the corresponding RFS rates for the RG were 69%, 69%, and 52%, respectively. In primary disease, extraabdominal tumor location was the only significant adverse prognostic factor associated with worse RFS (p = 0.008). Whereas in recurrent disease, age ≥40 yrs (p = 0.022) and R0 margin status (p = 0.049) correlated with a significantly better outcome. Conclusion Recurrence in primary and recurrent DF is characterized by different predictive factors and benefits from distinct therapeutic strategies. Immunohistochemical markers are valuable tools in the diagnosis of DF, although have limited potential in predicting the outcome.