Improved Metastatic-Free Survival after Systematic Re-Excision Following Complete Macroscopic Unplanned Excision of Limb or Trunk Soft Tissue Sarcoma.

BACKGROUND: Whether re-excision (RE) of a soft tissue sarcoma (STS) of limb or trunk should be systematized as adjuvant care and if it would improve metastatic free survival (MFS) are still debated. The impact of resection margins after unplanned macroscopically complete excision (UE) performed out of a NETSARC reference center or after second resection was further investigated. METHODS: This large nationwide series used data from patients having experienced UE outside of a reference center from 2010 to 2019, collected in a French nationwide exhaustive prospective cohort NETSARC. Patient characteristics and survival distributions in patients reexcised (RE) or not (No-RE) are reported. Multivariate Cox proportional hazard model was conducted to adjust for classical prognosis factors. Subgroup analysis were performed to identify which patients may benefit from RE. RESULTS: Out of 2371 patients with UE for STS performed outside NETSARC reference centers, 1692 patients were not reviewed by multidisciplinary board before treatment decision and had a second operation documented. Among them, 913 patients experienced re-excision, and 779 were not re-excised. Characteristics were significantly different regarding patient age, tumor site, size, depth, grade and histotype in patients re-excised (RE) or not (No-RE). In univariate analysis, final R0 margins are associated with a better MFS, patients with R1 margins documented at first surgery had a better MFS as compared to patients with first R0 resection. The study identified RE as an independent favorable factor for MFS (HR 0.7, 95% CI 0.53-0.93; p = 0.013). All subgroups except older patients (>70 years) and patients with large tumors (>10 cm) had superior MFS with RE. CONCLUSIONS: RE might be considered in patients with STS of limb or trunk, with UE with macroscopic complete resection performed out of a reference center, and also in originally defined R0 margin resections, to improve LRFS and MFS. Systematic RE should not be advocated for patients older than 70 years, or with tumors greater than 10 cm.

Mazabraud Syndrome Associated with McCune-Albright Syndrome: A Case Report and Literature Review.

The Mazabraud syndrome is a rare form of bone fibrous dysplasia associated with intramuscular myxomas. The McCune-Albright syndrome is characterized by the association of dysplasia fibrous bone to one or more extra-osseous manifestations, including café-au-lait skin spots and endocrine disturbances. Here, we report on a new case of a 52-year-old man with sacroiliac polyostotic bone fibrous dysplasia associated with intramuscular myxomas of the left buttock and thigh and a café-au-lait skin spot. Biopsy analysis of one muscular lesion on the left thigh analysis revealed a spindle cell tumor with myxoid stroma with GNAS gene mutation, confirming the diagnosis of intramuscular myxoma. Given the absence of radiological sign of malignancy at the bone level and a few pains relieved by simple analgesics, no specific treatment was applied. In March 2022, after 18 months of follow-up, the magnetic resonance imaging and the PET-CT scan showed a stable disease. To our knowledge, this case is the fourth one reporting association of Mazabraud syndrome and McCune-Albright syndrome in a man. The association of intramuscular tumors and bone tumors in the same anatomical region and without any continuity, especially in lower limbs, must evoke the diagnosis of Mazabraud syndrome.

Impact of fiducial markers placement on the delineation of target volumes in radiation therapy for oesophageal cancer: FIDUCOR study.

Purpose: This prospective monocentric phase II study (FIDUCOR-study, NCT02526134) aimed to assess the impact of fiducial markers (FMs) implantation on conformal chemo-radiation therapy (CRT) planning in oesophageal carcinoma (EC) patients. Methods/materials: Fifteen EC patients were enrolled in the study. Each patient underwent two simulation CT-scans before (CT1) and after (CT2) FMs implantation, in the same position. FMs (3 mm length gold markers, preloaded in a 22G needle) were implanted after sedation, under endoscopic ultrasound (EUS) and X-Ray guidance, and were placed at the tumor's extremities, and in the visible lymph nodes. Target delineation and treatment plan were both performed first on CT1 with the assistance of diagnosis CT, gastroscopy and EUS details, and second on CT2 using FMs and CT-data. The value of FMs implantation was assessed by the difference of growth-tumor-volume (GTV) and clinical-target-volume (CTV) between CT1-based and CT2-based delineation. A significant difference was defined as a ≥5 mm-difference on axial(x) or coronal(y) slices, a ≥10mm-difference on sagittal slices, or a ≥20%-difference in GTV. The impact on dose distribution in organs at risk (OAR) (lung, heart, liver) was also studied. Results: Between 09/2014 and 12/2015, 15 patients could achieve fiducial procedures, without any complication. One FM migration occurred. We observed a significant modification of the GTV-dimension in 100% of the cases (15/15, 95%CI: [78.2;100.0]), mainly due to a difference in sagittal dimension with a mean variation of 11.2 mm and a difference> 10 mm for 8/15 patients (53.3%). One patient had a significant isocenter displacement as high as 20 mm. The oesophagus tumor was not seen on the CT-scan in one patient due to its small size. One patient had a distant lymph node metastasis not visible on CT-scan. We observed no significant impact on OAR distribution. Conclusion: In our study, FMs-implantation under EUS had a positive impact on accurate volume definition in EC-patients (modification of GTV in 15/15 patients). Close cooperation between gastroenterologist and radiation oncologist has the potential to improve local treatment of oesophageal carcinoma.

Overall survival in patients with re-excision of positive microscopic margins of limb and trunk wall soft tissue sarcoma operated outside of a reference center: a nationwide cohort analysis.

BACKGROUND: This French nationwide NETSARC exhaustive prospective cohort aims to explore the impact of systematic re-excision (RE) as adjuvant care on overall survival (OS), local recurrence free survival (LRFS), and local and distant control (RFS) in patients with soft tissue sarcoma (STS) with positive microscopic margins (R1) after initial resection performed outside of a reference center. METHODS: Eligible patients had experienced STS surgery outside a reference center from 2010 to 2017, and had R1 margins after initial surgery. Characteristics and treatment comparisons used chi-square for categorical variables and Kruskall-Wallis test for continuous data. Survival distributions were compared in patients reexcised (RE) or not (No-RE) using a log-rank test. A Cox proportional hazard model was used for subgroup analysis. RESULTS: A total of 1,284 patients had experienced initial STS surgery outside NETSARC with R1 margins, including 1,029 patients with second operation documented. Among the latter, 698 patients experienced re-excision, and 331 were not re-excised. Characteristics were significantly different regarding patient age, tumor site, tumor size, tumor depth, and histotype in the population of patients re-excised (RE) or not (No-RE). The study identified RE as an independent favorable factor for OS (HR 0.36, 95%CI 0.23-0.56, p<0.0001), for LRFS (HR 0.45, 95%CI 0.36-0.56, p<0.0001), and for RFS (HR 0.35, 95%CI 0.26-0.46, p<0.0001). CONCLUSION: This large nationwide series shows that RE improved overall survival in patients with STS of extremities and trunk wall, with prior R1 resection performed outside of a reference center. RE as part of adjuvant care should be systematically considered.

Correction: Efficiency of an endoscopic resection strategy in the management of submucosal tumors less than 20 mm in size and located in the upper gastrointestina.

[This corrects the article DOI: 10.1055/a-1783-8675.].

Efficiency of an endoscopic resection strategy for management of submucosal tumors < 20 mm in the upper gastrointestinal tract.

Background and study aims The histologic diagnosis of submucosal tumors (SMTs) < 20 mm is challenging. Monitoring is the main option offered, but compliance is debatable. Endoscopic resection (ER) of malignant SMTs or those with an uncertain diagnosis is an alternative that has already been reported about and proposed in our center. The aims of this study were to confirm the safety of this resection strategy and to perform long-term follow-up of malignant SMTs after resection. Patients and methods All patients who underwent ER for SMTs < 2 cm in a single center between 2007 and 2019 were included retrospectively. Patients were classified into two groups according to the need for postresection follow-up: benign SMTs (B-SMTs) and follow-up SMTs (FU-SMTs). Results One hundred and one patients were included. The mean tumor size was 16.7 mm. In total, 92 of 101 SMTs had an uncertain diagnosis. Macroscopic resection was completed for 95 SMTs (93.1 %), with en bloc resection in 94 (92.1%). The morbidity rate was 3 %, with no mortality. A total of 84 of 101 SMTs (84 %) were B-SMTs and did not need monitoring, and 17 SMTs (19.7 %) were FU-SMTs (8 gastrointestinal stromal tumors, 6 neuroendocrine tumors, and 3 others). No relapse was reported in the FU-SMT group, with a median follow-up duration of 33 months [4-127] (61 months [17-127] for the gastrointestinal stroma tumor group). Conclusions The study results suggest ER is a potentially reliable and effective strategy for upper gastrointestinal tract SMTs < 20 mm. Although the strategy needs further validation in advanced care units, it could eliminate the need for long-term monitoring, therefore targeting such follow-up efforts to patients with FU-SMTs.

Transhiatal esophagectomy as a treatment for locally advanced adenocarcinoma of the gastroesophageal junction: postoperative and oncologic results of a single-center cohort THE for locally advanced GEJC.

BACKGROUND AND PURPOSE: To report the postoperative and oncological outcomes of transhiatal esophagectomy for locally advanced cancer of the gastroesophageal junction. METHODS: Medical records of 120 consecutive patients who underwent transhiatal esophagectomy for locally advanced cancer of the gastroesophageal junction with curative intent after neoadjuvant treatment between February 2006 and December 2018 at our center were reviewed. RESULTS: All patients received either chemotherapy (46.7%) or chemoradiation (53.3%). The 90-day mortality and overall morbidity rates were 0.8% and 56.7%, respectively. Respiratory complications were the most common (30.8%). Anastomotic leakage occurred in 19 patients (15.8%), who were treated by local wound care (n = 13) or surgical drainage (n = 6). Recurrent laryngeal nerve injury occurred in 12 patients (9.9%). The median length of hospital stay was 15.5 days. The rate of R0 resection was 95.8%, and the median number of nodes removed was 17.5. Over a median follow-up of 77 months, the rate of recurrence was 40.8%, and the overall survival rates at 1, 3, and 5 years were 91%, 75%, and 65%, respectively. The median survival time was not reached. In multivariate analysis, disease stage was the only independent significant prognostic factor. CONCLUSIONS: Transhiatal esophagectomy is a safe and effective procedure with good long-term oncological outcomes for locally advanced tumors after neo-adjuvant treatment. It can be recommended for all patients with cancer of the gastroesophageal junction, regardless of the Siewert classification, tumor stage, and comorbidities.

Preoperative EUS evaluation of the response to neoadjuvant therapy for gastric and esophagogastric junction cancer is correlated with survival: A single retrospective study of 97 patients.

BACKGROUND AND OBJECTIVES: The European Society for Medical Oncology suggests performing EUS staging for esophagogastric junction and gastric cancers to further assess the T and N stages. The use of EUS after neoadjuvant therapy (NT) is still under debate. We aimed to evaluate the contribution of EUS after NT to staging, therapeutic choices, and prognosis prediction. SUBJECTS AND METHODS: In 97 patients with esophagogastric junction and gastric cancers who received NT (chemotherapy or radiochemotherapy) followed by carcinologic surgery, EUS was performed before (uT, uN) and after (yuT, yuN) NT. We compared the results of EUS staging after NT (yuT and yuN) and final histology (ypT and ypN). We analyzed the correlation between overall survival (OS), disease-free survival (DFS), and the objective and subjective responses to NT evaluated by EUS (comparison of uT and yuT and uN and yuN with OS and DFS). RESULTS: EUS staging detected metastasis that went undetected by computed tomography in 16% of metastatic patients. The accuracy between EUS after NT and postoperative pathological findings was 44.4% (34.2%; 54.7%) for T stage and 49.3% (37.5%; 61.1%) for N stage. On multivariate analysis, OS had significantly correlated with the objective response to NT. In the case of a response to NT, the median OS was 64.77 months, and in the case of stable disease, the median OS was 22.9 months (P = 0.01). CONCLUSION: EUS after NT can be used for staging. Despite its moderate accuracy, the evaluation of the response to NT by EUS seems to be correlated with patient prognosis.

Successful Imatinib Treatment of an Abdominal Compartment Syndrome due to Huge Gastrointestinal Stromal Tumour.

Gastrointestinal stromal tumours (GISTs) are the most common digestive mesenchymal tumours, whose prognosis has been revolutionised by targeted therapies such as oral imatinib. Abdomen compartment syndrome (ACS) is associated with mortality superior to 50% in adults. ACS has never been reported to date in patients with GIST. Specific anticancer treatment in critically ill patients in intensive care unit (ICU) remains a matter of debate given the high mortality rate. Here, we report the case of a 58-year-old woman with ACS related to a 40-cm huge GIST and multi-organ failure requiring mechanical ventilation, vasopressive support and haemodialysis. She was treated in emergency with imatinib via the naso-gastric tube (day 1), then at day 3 by decompressive laparotomy and "open abdomen" without any tumour removal. Imaging after 11 days imatinib showed objective tumour response. Because of improvement of multi-organ dysfunctions, the laparotomy was closed at day 14, and the resuscitation procedures were progressively stopped. After discharge from hospital, she survived nearly two years. This is the first case of successful treatment of cancer-associated ACS by targeted therapy and decompressive laparotomy. Imatinib in critically ill patients with GIST may be successful even in presence of multi-organ failure.

Giant Pedunculated Hepatic Hemangioma: A Case Report and Literature Review.

Hepatic hemangioma is the most common benign hepatic tumor, and most of them are small in size and asymptomatic. Giant hepatic hemangiomas are uncommon, but pedunculated giant hemangiomas are even rarer and often difficult to diagnose because of their exophytic development. We report here on a 48-year-old man with a symptomatic pedunculated giant hepatic hemangioma and hepatic angiomatosis, mimicking a gastric gastrointestinal stromal tumor with liver metastases. The preoperative diagnosis was suspected on imaging including CT scan and MRI. The patient was successfully operated (left hepatic lobectomy), without any complication, and the pathological analysis confirmed the diagnosis. We reviewed the English literature, and to our knowledge, our case represents the largest case reported so far when compared with the 19 other informative cases.

Metastatic Intimal Sarcoma of the Pulmonary Artery Sensitive to Carboplatin-Vinorelbine Chemotherapy: Case Report and Literature Review.

Pulmonary artery intimal sarcoma (PAIS) is a very rare tumour with a very poor prognosis. In advanced stages, chemotherapy and radiotherapy are poorly efficient, and no standard chemotherapy guideline is currently available. Here, we report on a 37-year-old woman with PAIS initially treated with surgical resection who developed metastatic relapse refractory to anthracycline-based chemotherapy, then trabectedin, then pazopanib. The patient was then given carboplatin-vinorelbine chemotherapy. The treatment was well tolerated, and, rapidly, a CT scan showed an objective response that lasted 8 months despite the 4th therapeutic line. We review the literature and show that our case is the second one that provides evidence of the efficacy of platinum-vinorelbine regimens in this aggressive tumour.

Gastrointestinal Stromal Tumour with Synchronous Bone Metastases: A Case Report and Literature Review.

Gastrointestinal stromal tumours (GISTs) are mesenchymal tumours of the digestive tract, derived from Cajal interstitial cells. Bone metastases are very rare, and there is no consensus regarding their treatment. Here, we present the unusual case of a 66-year-old man with a gastric GIST with synchronous bone and liver metastases, fully documented at the pathological and molecular levels with a KIT exon 11 mutation. After 9 months of imatinib, the scanner showed a 33% partial response of target lesions. We also review the literature and describe the characteristics, treatment, and outcome of all cases previously reported.

Neoplastic Barrett's oesophagus and long-term follow-up after endoscopic therapy: complete histological eradication of Barrett associated with high-grade dysplasia significantly decreases neoplasia relapse.

BACKGROUND AND AIMS: Endotherapy (ET) has replaced surgery as the first-line treatment of high-grade dysplasia (HGD)/superficial ECA (ECAs) from Barrett's oesophagus (BO). However, long-term follow-up and predictive factors of relapse are not so well studied. The aim of the following study was to evaluate the efficiency of ET for treatment of HGD/ECAs and to determine factors of long-term efficiency. METHODS: ET procedures were manually reported and registered in a hospital data base from March 2000 to July 2010. Inclusion criteria were HGD/ECA on pre-resection biopsies, complete histological and sufficient oncological resection of HGD/ECAs, and complete macroscopic resection of metaplastic BO. Sixty patients (53 men, mean age = 65 years) were included. RESULTS: Median follow-up was 66 months [range 42-80]. Complete eradication of residual histological metaplastic BO occurred in 29 patients (48 %). Relapse rate at 36 months was 16.6 % (n = 10) and was unchanged at 60 months of follow-up. There was only one relapse (3.4 %) in case of complete eradication of metaplastic BO and 9 (31 %) in case of incomplete eradication. In univaried and multi-varied analysis, complete eradication of metaplastic BO (p < 0.05) and BO length <5 cm (p < 0.05) were predictive of neoplastic BO non relapse. The length of BO remained a prognostic factor for disease-free survival (DFS). When these preponderant data were cancelled out in multi-varied analysis, complete eradication of BO was a prognostic factor for DFS (p < 0.05). CONCLUSION: Complete histological eradication of BO by ET significantly decreases the rate of neoplasia relapse.

Isolated Splenic Metastases of Her2+++ Gastroesophageal Junction Adenocarcinoma.

Isolated metastases from gastric adenocarcinoma to the spleen are very infrequent. Usually, there are multiple metastases from gastric cancer, and isolated splenic metastases are very rare [Lam and Tang: Arch Pathol Lab Med 2000;124:526-530] because of certain anatomical and physiological characteristics (e.g., angulation between the splenic artery and celiac trunk, paucity of afferent lymph flow toward the spleen, contractility of the spleen and major immune content). Here, we report 2 cases of isolated splenic metastases from an adenocarcinoma of the gastroesophageal junction, both with long-term survival outcome and overexpression of Her2.

Postoperative sepsis in cancer patients undergoing major elective digestive surgery is associated with increased long-term mortality.

BACKGROUND: Major postoperative events (acute respiratory failure, sepsis, and surgical complications) are frequent early after elective gastroesophageal and pancreatic surgery. It is unclear whether these complications impact equally on long-term outcome. METHODS: Prospective observational study including the patients admitted to the surgical intensive care unit between January 2009 and October 2011 after elective gastroesophageal and pancreatic surgery. Risk factors for 30-day major postoperative events and long-term outcome were evaluated. RESULTS: During the study period, 259 patients were consecutively included. Among them, 166 (64%), 54 (21%), and 39 (15%) patients underwent pancreatic surgery, gastric surgery, and esophageal surgery, respectively. Using the Clavien-Dindo classification, 117 patients (45%) developed at least 1 postoperative complication, including 60 (23%) patients with acute respiratory failure, 77 (30%) with sepsis, and 89 (34%) with surgical complications. The median follow-up from the time of intensive care unit admission was 34 months (95% confidence interval, 30-37 months). The 1-year survival was 95% (95% confidence interval, 92-98). Among the perioperative variables, postoperative sepsis and an American Society of Anesthesiologists score higher than 2 were independently associated with long-term mortality. In septic patients, death (n = 16) was significantly associated with cancer recurrence (n = 10; P < .0001). Independent factors associated with postoperative sepsis were a Sequential Organ Failure Assessment score on day 1, a systemic inflammatory response syndrome on day 3, positive intraoperative microbiological samples, Simplified Acute Physiology Score II and an American Society of Anesthesiologists score higher than 2 (P < .005). CONCLUSIONS: Postoperative sepsis was the only major postoperative event associated with long-term mortality. Postoperative sepsis may reflect a deep impairment of immune response, which is potentially associated with cancer recurrence and mortality.

High-grade soft tissue sarcoma arising in a desmoid tumor: case report and review of the literature.

Desmoid tumors are rare benign monoclonal fibroblastic tumors. Their aggressiveness is local with no potential for metastasis or dedifferentiation. Here we report on a 61-year-old patient who presented a locally advanced breast desmoid tumor diagnosed 20 years after post-operative radiotherapy for breast carcinoma. After 2 years of medical treatment, a high-grade undifferentiated pleomorphic soft tissue sarcoma arose within the desmoid tumor. Despite extensive surgery removing both tumors, the patient showed locoregional relapse by the sarcoma, followed by multimetastatic progression, then death 25 months after the surgery. The arising of a soft tissue sarcoma in a desmoid tumor is an exceptional event since our case is the fourth one reported so far in literature. It reinforces the need for timely and accurate diagnosis when a new mass develops in the region of a preexisting desmoid tumor, and more generally when a desmoid tumor modifies its clinical or radiological aspect.

Primary synovial sarcoma of the thyroid gland: case report and review of the literature.

Synovial sarcoma (SVS) of the thyroid gland is exceedingly rare. We report the case of a 55-year-old man with a rapidly growing 7-cm neck mass. Because of suspicion of anaplastic thyroid carcinoma, a total thyroidectomy was planned, without preoperative cytology. During surgery, the tumor ruptured, leading to fragmented and incomplete resection. The morphological and immunohistochemical aspects suggested thyroid SVS, which was confirmed by fluorescent in situ hybridization (SYT gene rearrangement). The patient experienced immediate local relapse in close contact with large vessels and the thyroid cartilage and was referred to our institution. Doxorubicin-ifosfamide chemotherapy led to a minor response that authorized secondary conservative surgery. Because of microscopically incomplete resection, adjuvant radiotherapy was chosen and is ongoing 10 months after initial surgery. The prognosis of thyroid SVS is associated with a high risk for local and metastatic relapses. Pretreatment diagnosis is fundamental and may benefit from molecular analysis. Margin-free monobloc surgical excision is the best chance for cure, but adjuvant chemotherapy and radiotherapy deserve to be discussed.

Can we resect EGC with Signet ring cells in Europe?

PURPOSE: The proposed guideline for performing endoscopic resection of early gastric carcinoma (EGC) in Paris classification is a well-differentiated carcinoma with maximum involvement Sm1. Signet ring cell carcinomas (SRC) are excluded from this recommendation. Authors from Eastern countries have proposed extending this resection to include selected undifferentiated EGC. Via an analysis of a series of cases of signet ring carcinoma, we will discuss whether it is possible to resect EGC with signet ring cells in Europe. METHODS: We retrospectively included patients with histological classification pT1 of EGC showing SRC. Data was extracted from the hospital gastrectomy register. Lymphadenomectomy D1.5 was performed on all patients. Histology results were retrospectively obtained from the electronic patient file. RESULTS: Twelve patients (mean age = 55.4, four women, eight men) underwent surgery, without previous chemotherapy, between 2000 and 2012, for EGC with SRC. Mean size of the lesions was 20.2 mm (5-35 mm). Seven lesions were located in the antrum, five in the fundus. In the case of nine patients, histology showed no lymphovascular involvement. None of these nine patients presented lymph node metastases (LNM). Five patients had intramucosal carcinoma, four were classified as Sm1, one patient was Sm2, and one patient was Sm3. On surgery, the three patients with lymphovascular invasion showed LNM. CONCLUSION: Endoscopic resection of EGC with SRC does not systematically imply complementary treatment by surgery, although criteria for endoscopic resection are difficult to determine because of the lack of data in Europe.